Clinical Guidelines February 2011 to January 2012 1 TABLE OF CONTENTS TABLE OF CONTENTS ............................................................................................2 1 GENERAL ADMINISTRATION .......................................................................6 1.1 UHW Telephone Numbers ............................................................................6 1.1.1 Consultant Medical Staff and Speciality Doctor ...................................6 1.1.2 Secretarial Staff and Specialist Cardiac Nurses.....................................6 1.1.3 Junior Medical Staff/Trainees................................................................7 1.1.4 Technical and Support Staff...................................................................7 1.1.5 Useful E-Mail Addresses .......................................................................7 1.1.6 Useful Contact Numbers........................................................................8 1.2 Welsh Hospitals ...........................................................................................10 1.3 Specialist Cardiac Nurses ............................................................................11 1.3.1 Contact Numbers .................................................................................11 1.3.2 Roles and Responsibilities ...................................................................11 1.3.3 Nurse-Led Clinic..................................................................................11 1.4 Bristol Children’s Hospital ..........................................................................12 1.4.1 Medical Staff – Paeds ..........................................................................12 1.4.2 Medical Staff – GUCH (Bristol Heart Institute)..................................12 1.4.3 Other Useful Numbers .........................................................................12 1.4.4 Bristol E-Mail Addresses.....................................................................12 1.5 UK and Ireland Paediatric Cardiac Units.....................................................13 1.6 Annual and Study Leave..............................................................................14 1.7 On-Call Arrangements .................................................................................14 1.7.1 Consultant Staff ...................................................................................14 1.7.2 Junior Medical Staff.............................................................................14 1.8 Audit and Research ......................................................................................14 1.9 Computers and CardiobaseВ® ........................................................................15 1.10 Medical Notes and Correspondence Headings ............................................15 1.10.1 Categories/Definitions of Admissions and Reviews............................16 1.10.2 Correspondence Headings ...................................................................16 2 DAY TO DAY BUSINESS ................................................................................17 2.1 Daily Timetable ...........................................................................................17 2.2 UHW Clinics................................................................................................18 2.2.1 General Clinics............................................................................................18 2.2.2 Specialist Clinics.........................................................................................18 2.2.3 Echo Clinic..................................................................................................18 2.3 Outreach Clinics...........................................................................................18 2.4 Teaching Topics...........................................................................................19 2.5 Teaching Ward Round .................................................................................19 3 ADMISSIONS AND REFERRALS .................................................................20 3.1 Routine Admissions .....................................................................................20 3.2 Day Case Admissions ..................................................................................21 3.2.1 Echocardiogram under sedation...........................................................21 3.2.2 ACE inhibitor protocol ........................................................................22 3.2.3 Carvedilol protocol ..............................................................................23 3.2.4 Adrenaline/Epinephrine challenge for LQTS ......................................24 2 3.2.5 Brugada syndrome - Flecainide or Ajmaline Challenge......................27 3.3 Admissions for Transoesophageal Echocardiography.................................28 3.4 Admissions for MRI or CT scan..................................................................28 3.4.1 Non-General Anaesthetic...........................................................................28 3.4.2 General Anaesthetic ...................................................................................29 3.4.3 Adenosine Stress Test by MRI ..................................................................29 3.5 Non-Cardiac Admissions and Casual Ward Attenders ................................30 Emergency admissions and procedures .......................................................31 3.6 3.6.1 Patients known to the Department .......................................................31 3.6.2 New Referrals ......................................................................................31 3.6.3 Neonatal Admissions ...........................................................................31 3.6.4 Protocol for PGE infusion (NB risk of apnoea) ........................................32 3.7 Emergency Interventional Procedures .........................................................32 3.7.1 Balloon Atrial Septostomy ...................................................................33 3.7.2 DC Cardioversion (DCCV)..................................................................33 3.7.3 Pericardiocentesis for cardiac tamponade ............................................34 3.8 Post Surgical Transfers (in) .........................................................................36 Inpatient Referrals ........................................................................................36 3.9 4 DISCHARGE AND TRANSFER PROCEDURES.........................................37 General Principles ........................................................................................37 4.1 Transfers to Other Hospitals ........................................................................38 4.2 Discharges Following Cardiac Surgery .......................................................39 4.3 4.4 Discharge Checklist .....................................................................................39 4.5 Death of a Patient .........................................................................................39 5 INVESTIGATIONS...........................................................................................40 Drug Monitoring and/levels .........................................................................40 5.1 5.2 Electrocardiogram ........................................................................................40 5.2.1 Basic ECG Interpretation .....................................................................41 5.3 Echocardiogram ...........................................................................................42 5.3.1 Echocardiogram to rule out cardiac source of embolism.....................42 5.4 Exercise Test ................................................................................................43 5.4.1 Indications for exercise test .................................................................43 5.4.2 Bruce protocols ....................................................................................43 5.4.3 Indications for termination:..................................................................44 5.4.4 Difficulties in interpretation .................................................................44 5.4.5 Helpful tips...........................................................................................44 5.5 Ambulatory ECG Monitoring ......................................................................45 5.5.1 Holter monitoring.................................................................................45 5.5.2 Event recorders ....................................................................................45 5.6 Ambulatory BP Monitoring .........................................................................45 5.7 Tilt Test ........................................................................................................47 5.8 MRI or CT Scan...........................................................................................48 5.9 Isotope Scans ...............................................................................................48 5.9.1 Cardiac Nuclear Scanning....................................................................48 5.9.2 Lung perfusion scan .............................................................................48 6 CLINICAL PROBLEMS ..................................................................................49 6.1 Anti-platelet Therapy and Anticoagulation .................................................49 3 6.1.1 Aspirin and Anti-platelet Therapy11 ...................................................49 6.1.2 Devices and Stents ...............................................................................49 6.1.3 Valve replacement ...............................................................................50 6.1.4 Cavopulmonary shunt / Fontan ............................................................50 6.1.5 Other indications ..................................................................................50 6.1.6 Commencing anticoagulation (heparin and warfarin) .........................51 6.1.7 INR Sampling ......................................................................................53 6.1.8 INR Protocol ........................................................................................53 6.1.9 Warfarin dosage table (doses shown in mg) ........................................55 6.1.10 Cessation of warfarin for surgical or invasive procedure ....................56 6.1.11 Factors that influence the efficacy of warfarin ....................................56 6.2 Asplenia and Immunodeficiency .................................................................57 6.2.1 Asplenia ...............................................................................................57 6.2.2 DiGeorge syndrome and chromosome 22 microdeletion ....................58 Cardiac Failure .............................................................................................59 6.3 6.4 Cardiac Tamponade - See section 3.7.2 .......................................................63 6.5 Cardiomyopathy ...........................................................................................63 6.6 Chest Pain in Children .................................................................................68 6.7 Endocarditis and Endocarditis Prophylaxis .................................................70 6.7.1 Infective Endocarditis ..........................................................................70 6.7.2 Endocarditis prophylaxis .....................................................................71 6.8 Exercise in Paediatric Cardiac Patients........................................................72 6.8.1 Myocardial Abnormalities .........................................................................75 6.8.2 Coronary Abnormalities.............................................................................75 6.8.3 Congenital Lesions and Conditions ...........................................................76 6.8.4 Recommendations in Post-Operative Patients ...........................................79 6.8.5 Recommendations for Athletes with Arrhythmias ....................................81 6.9 Fits, Faints and Funny Turns .......................................................................82 6.9.1 Reflex Syncope .........................................................................................83 6.10 Hypercyanotic Spells ...................................................................................85 6.11 Kawasaki Disease ........................................................................................86 6.12 MRSA Infection...........................................................................................88 6.13 Nutrition in Cardiac Patients........................................................................89 6.13.1 Faltering Growth..................................................................................89 6.13.2 Gastro-Oesophageal Reflux (GOR) .........................................................89 6.14 Oxygen Therapy in Cardiac Patients ...........................................................90 6.15 Premature Beats in Newborn Babies ...........................................................91 6.16 Prescribing Drugs Safely .............................................................................92 6.16.1 Medication Errors ....................................................................................92 6.16.2 Quick Calculations of Drug Concentrations for Infusions ......................93 6.17 Propranolol for the Treatment of Capillary Haemangiomas........................94 6.18 Protein-losing enteropathy (PLE) / Plastic bronchitis (PB) after Fontan ....96 6.19 Pulmonary Hypertension in Childhood .......................................................97 6.19.1 Definition, Classification and WHO Functional Status.......................97 6.19.2 Pathophysiology ...................................................................................98 6.19.3 Treatment of Pulmonary Hypertension................................................99 6.19.4 Persistent Pulmonary Hypertension of the Newborn (PPHN)...........101 6.20 Rheumatic Fever ........................................................................................105 6.20.1 Diagnostic criteria ..............................................................................105 6.20.2 Secondary Prevention of Rheumatic Fever (Recurrent Attacks) .......106 4 6.20.3 Duration of Secondary Rheumatic Fever Prophylaxis.......................106 6.21 RSV Infection in Cardiac Patients .............................................................106 6.22 Screening for Cardiac Disease (genetic, familial, etc) ...............................107 6.22.1 Heart muscle disease..........................................................................107 6.22.2 Heart Rhythm.....................................................................................111 6.22.3 Heart Structure ...................................................................................112 6.23 Supraventricular Tachycardia ....................................................................114 6.23.1 SVT in the Fetus ................................................................................116 6.24 Transplantation ..........................................................................................116 INDEX ......................................................................................................................119 5 1 GENERAL ADMINISTRATION 1.1 UHW Telephone Numbers 1.1.1 Consultant Medical Staff and Speciality Doctor Clinician Secretary Office Radiopage Mobile Dr Victor Ofoe 4746 3869 07623 905 928 07815 510 833 Dr Obed Onuzo 4606 2908 07623 906 018 07815 902 866 Dr Orhan Uzun 4743 4745 07623 906 121 07967 337 319 Dr Dirk G Wilson 4749 5156 07623 905 734 07968 822 824 Dr Peter Groves 3533 2354 07623 905 821 07899 727 937 Dr Navroz Masani 4086 4569 07623 905 821 07710 272 928 Dr Helen Wallis Contact via Neath Port Talbot Hospital Switchboard or Secretary (01639 722049) Hospital page Speciality Doctor in Paediatric 4759 6343 Cardiology Dr Amos Wong п‚· п‚· п‚· п‚· п‚· 1.1.2 Home Via UHW Switchboard Via UHW Switchboard Via UHW Switchboard Via UHW Switchboard Via UHW Switchboard Via UHW Switchboard Preferred contact is via radiopager or mobile phones. Most hospital mobiles do not work in Welsh hospitals, so radiopage is often more reliable during working hours. If you cannot make contact successfully try the home phone number via Switchboard. If all efforts to contact the on-call consultant are unsuccessful, contact one of the other consultants. In a dire emergency, speak to the on-call Bristol consultant for advice. Secretarial Staff and Specialist Cardiac Nurses Name Extension Other information Sarah Wooller (Dr Onuzo) Angela Butters (Dr Uzun) 4606 4743 Suzanne Cornish (Dr Wilson) 4749 Amanda Doyle (Dr Ofoe) Sarah Grinter (Dr Masani) Karen McCarthy (Dr Groves) 4746 4086 2354 08:30 – 17:00 09:00 – 17:30 08:30 – 17:00 08:00 – 14:30 09:00 – 17:00 08:30 – 17:00 08:30 – 17:00 Mon-Fri Mon-Fri Mon-Wed Thu Mon-Wed, Fri Mon-Fri Mon-Fri Specialist Cardiac Nurse (Area) Office Mobile Claire Logan (Cardiff and the Vale + Rhondda Cynon Taff) 5184 07811 197 136 Wendy Williams (Bridgend and West) 4753 07813 922 441 6 Alison Pearce (Gwent, western Valleys and Merthyr) 5524 07966 461 421 Ann Jermyn (Transition Care 12-19 years) 8046 07980 635 177 1.1.3 Junior Medical Staff/Trainees Title Cardiac Registrar Paediatric Registrar SHO/ST2 Bleep 5391 or 07623 906359 5394 5334 Extension 4759 4759 4759 1.1.4 Technical and Support Staff Staff Viv Booker (Echo) Bethany Glasser (Dietician) Tony Bradley (Social Work) David “Wally” James (Audit, Data and IT Support) Bleep/Radiopage 5503 07623 905 607 Mobile via Switch Extension 3920 3190 2776 3889 1.1.5 Useful E-Mail Addresses Name E-mail address Booker, Viv [email protected] Butters, Angela [email protected] Cornish, Suzanne [email protected] Doyle, Amanda [email protected] James, Wally [email protected] Jermyn, Ann [email protected] Logan, Claire [email protected] Ofoe, Victor [email protected] Onuzo, Obed [email protected] Pearce, Alison [email protected] Uzun, Orhan [email protected] Wallis, Helen [email protected] Wilson, Dirk [email protected] Wooller, Sarah [email protected] Wong, Amos [email protected] 7 1.1.6 Useful Contact Numbers Department/Individual Adult Cardiac ICU Anaesthetic Department B1 (Adult Cardiology) Bacteriology Biochemistry (Main Dept) Biochemistry (Emergency) Blood Bank CARDIAC ARREST Cardiac Day Case Unit Catheter Laboratory Clinical Investigation Unit Children’s Assessment Unit Coronary Care Unit Coagulation Coroner’s Office Delivery Suite Dental Surgeon Paeds Drug Information ECG (Main Dept, Pacing) ECG (Inpatient requests) EEG/Neurophysiology Exercise/Tilt Test Fetal Medicine General ICU Genetics Haematology Hand over room General Hand over room Specialty Heulwen Ward Histology Holter (24 hour tape) Immunology IT help desk Medical Illustration MRI MRI/CT CD Copying Extension 3265 3107 3382/4603 2044 2805/2637 5278 2157/2158 2222 4414 4642/3329/ 4607 3765 5441 2110 2214 20 222 111 2679 2260 2251 3325 6396 3194 3465 2279/3341 5319 4028 2805 8930 8820 4755/5375 2714 6396 5814 5073 3305/3307 3063 6631 8 Bleep Other Information 5388 Bleep for out-of-hours samples Call Lab for urgent processing 5268 5270 Outside line required Suite 11 OPD (Holters also) 5269 Bleep for out-of-hours samples Main Desk Department/Individual Neonatal Unit Reception NICU (ICU Room 1) OPD (Adult Cardiac) OPD (Paediatric) OPD (Room 4) Oncology Sky Ward Pacing Clinic Pacing Lab Paediatric HDU (Heulwen) Paediatric ICU Paeds Land Ward Paeds Ocean Ward Paeds South Pathology Personnel (Human resources) Pharmacy Porters Postnatal Ward Public Health SALT team Sophie Pearson Room Switchboard Teenage Cancer Trust Theatres (Reception) Toxicology Ultrasound X-ray Extension 2680/2684 6873 3266 3364 2242 8801/8802 4600 3081 4751 3282/4622 3274/3276 3359/3370 3277/2650 2710 3887 2988 2667 3343 2236 3736 6355 100 6784/6915 2993 72 6894 4834 3027 Bleep Other Information NNU HDU 7847 Adult Congenital Clinic Emergency 2222 Dr’s Office 2973 Drug levels 5299 9 Out of hours – call extn 8084 1.2 Welsh Hospitals District General Hospital WHTN External Number Aberdare General Hospital 01753 01685 872 411 Brecon War Memorial Hospital 01762 01874 622 443 Bronglais General Hospital(Aberyswyth) 01822 01970 623 131 Caerphilly Miner’s Hospital 01755 029 2085 1811 01776 (From UHW Llandough Hospital dial 72 then 029 2071 1711 extension) Morriston Hospital (Swansea) 01789 01792 702 222 Neath Port Talbot Hospital 01881 01639 862 000 Nevill Hall Hospital (Abergavenny) 01736 01873 732 732 Prince Charles Hospital (Merthyr) 01854 01685 721 721 Princess of Wales Hospital (Bridgend) 01855 01656 752 752 Prince Phillip Hospital (Llanelli) 01824 01554 756 567 Royal Glamorgan Hospital 01751 01443 443 443 Royal Gwent Hospital (Newport) 01738 01633 234 234 Saint David’s Hospital 01771 029 2053 6666 Singleton Hospital (Swansea) 01883 01792 205 666 West Wales General Hospital (Carmarthen) 01827 01267 235 151 Withybush Hospital (Haverfordwest) 01720 01437 764 545 Ysbyty Glan Clwyd (Rhyl) 01815 01745 583 910 Ysbyty Gwynedd (Bangor) 01746 01248 384 384 Add “100” to the WHTN number for Switchboard Operator or direct-dial if you know the extension 10 1.3 Specialist Cardiac Nurses 1.3.1 Contact Numbers There are 3.4 whole-time-equivalent paediatric cardiac liaison nurses: Claire Logan, Wendy Williams, Ann Jermyn and Alison Pearce (Alison is part time). All are funded or “adopted” by the British Heart Foundation. Nurse (Area) Claire Logan (Cardiff and the Vale + Rhondda Cynon Taff) Wendy Williams (Bridgend and West) Alison Pearce - Part time Th/Fr (Gwent, western Valleys, Merthyr) Ann Jermyn (Transition care, age 1219) Office Radiopage Mobile 5184 07623 905 758 07811 197 136 4753 07623 906 121 07813 922 441 5524 07623 906 297 07966 461 421 8046 07980 635 177 1.3.2 Roles and Responsibilities п‚· Nurse-led clinic (see 1.3.2 below) п‚· Primary contact in INR service (section 6.1) п‚· Link between clinicians and parents п‚· Link between Cardiff and Bristol – participation in weekly Planning Meeting п‚· Parent education and advice The cardiac liaison nurses should be contacted about: п‚· Newly diagnosed children п‚· Cardiac admissions to the ward п‚· Cardiac catheter or operation preparation п‚· Any decision to transfer a patient (so they can keep track of transfers out) п‚· Distressed or anxious children or parents п‚· Ward discharges 1.3.3 Nurse-Led Clinic п‚· Nurse-Led Clinic runs on Mondays and Fridays 1:30 – 4:00 pm in the KRUF Unit (extension 6782). п‚· The clinic is supported by the cardiac dietician and echo technician; junior doctors may be asked to review patients attending the clinic. п‚· Aspects of care provided include o Post-operative review o Nutritional review п‚· Patients are booked by an appointment system allowing 15 minutes per patient. п‚· A diary for these appointments is kept in the Liaison Nurses’ Office. п‚· The person booking the appointment is responsible for informing the patient/parents of the date, time and location of the clinic. 11 1.4 Bristol Children’s Hospital 1.4.1 Medical Staff – Paeds Consultant Dr Alison Hayes Dr Rob Martin Dr Gareth Morgan Dr Graham Stuart Dr Andrew Tometzki Dr Bev Tsai-Goodman Dr Rob Tulloh Mr Massimo Caputo Mr Andrew Parry Mr Serban Stoica 1.4.2 Radiopage/Mobile/Home 8859 8858 8923 8176 Via BRI Switchboard 01179 215 411 or 01179 276 998 0117 342 8854 (Christine McFadden) 0117 342 5967 Via BRI Switchboard 01179 215 411 or 01179 276 998 0117 342 6576 0117 342 6575 0478 Other Useful Numbers Switchboard BHI Coronary Care Unit Paediatric Cardiac Ward (32) Catheter Laboratory Echocardiography Laboratory GUCH Liaison Nurse (Sheena) PCLN (Cathy + Debbie) Paediatric ICU Paeds OPD SCBU (St Michael’s) 1.4.4 Office 8848 8849 Medical Staff – GUCH (Bristol Heart Institute) Dr Stephanie Curtis Dr Rob Martin Dr Gareth Morgan Dr Graham Stuart Dr Mark Turner 1.4.3 Secretary 0117 342 8856 0117 342 8855 0117 342 8852 0117 342 8852 0117 342 8853 0117 342 8856 0117 342 8856 01179 215 411 or 01179 276 998 0117 342 2278 0117 342 8332 / 8679 0117 342 8282 / 8456 0117 342 8722 0117 342 0463 0117 342 8286 0117 342 8377 or 8437 0117 342 8401 or 8402 0117 342 5275 or 5275 Bristol E-Mail Addresses Dr Stephanie Curtis Dr Alison Hayes Dr Rob Martin Dr Graham Stuart Dr Gareth Morgan Dr Andrew Tometzki Dr Beverly Tsai-Goodman Dr Rob Tulloh Dr Mark Turner [email protected] [email protected] [email protected] [email protected] [email protected] [email protected] [email protected] [email protected] [email protected] Cont’d overleaf 12 Mr Massimo Caputo Mr Andrew Parry Mr Serban Stoica 1.5 [email protected] [email protected] [email protected] UK and Ireland Paediatric Cardiac Units Centre Switchboard Alder Hey Children’s Hospital (Liverpool) 0151 228 4811 Birmingham Children’s Hospital 0121 333 9999 01179 230 000 Bristol Royal Hospital for Children 01179 276 998 01173 428 460 Freeman Hospital (Newcastle-upon-Tyne) 0191 233 6161 Glenfield Hospital (Leicester) 0116 287 1471 Great Ormond Street, The Hospital for Sick Children 0207 405 9200 Guys Hospital (Evelina Children’s Hospital, London) 0207 188 7188 Harefield Hospital (London) 01895 823 737 John Radcliffe Hospital (Oxford) 01865 741 166 Leeds General Infirmary 0113 243 2799 Our Lady’s Hospital for Sick Children (Dublin) 00 353 1409 6100 Royal Belfast Hospital for Sick Children 02890 240 503 Royal Brompton & Harefield NHS Trust (London) 0207 352 8121 Royal Hospital for Sick Children (Edinburgh) 0131 536 0000 Royal Hospital for Sick Children (Glasgow) 0141 201 0000 Royal Manchester Children's Hospital 0161 794 4696 Wessex Cardiothoracic Unit (Southampton) 02380 777 222 13 1.6 п‚· п‚· п‚· п‚· 1.7 Annual and Study Leave Full leave entitlement should be taken Book leave 6 weeks in advance using approved Hospital and Deanery forms (including study leave passport) Inform junior doctor rota coordinator, enter leave in Junior Doctors’ Leave Diary, which is held by Suzanne (DGW’s secretary) Unless there are exceptional circumstances (such as examinations or job interviews) two junior doctors must be present to cover the unit during normal working hours. On-Call Arrangements 1.7.1 Consultant Staff п‚· The consultant staff work a 1:4 rota, one week at a time, with a hand-over taking place on Monday mornings in the Planning Meeting. п‚· An on-call rota is circulated well in advance. п‚· All junior staff should ensure they know which consultant is on call. п‚· If you are unable to contact the on-call consultant in an emergency, contact one of the other consultants (or, in a dire emergency, the consultant covering the Bristol unit). 1.7.2 Junior Medical Staff п‚· The SHO/ST2 participates in the paediatric on-call rota. Cross-cover arrangements are in place with other speciality teams. п‚· The rotating general paediatrics registrar attached to the cardiac unit participates in the paediatric specialities or NNU middle grade rota. п‚· The cardiology registrar is on-call 1 night in 5 (non-resident) and does not participate in the general paeds rota; he or she may be asked to provide crosscover support during an emergency. п‚· The resident on-call specialities SHO/ST2 and specialities registrar provide out-of-hours cross cover for cardiac patients, including those on HDU. Clear hand-overs between clinical staff are essential. Hand-over rounds take place daily (Mon-Fri) at 08:30 and 16:30. The team-member covering the cardiac patients should attend the relevant hand-over round to pick up and convey any relevant information about the cardiac patients. Written hand-overs should be provided to the paediatric team when they are cross-covering the cardiac patients. 1.8 Audit and Research Effective medical audit can improve patient care. The broad principles behind medical audit are the setting of an accepted standard, comparison of current practice to that standard, making alterations and completing the audit by re-assessing the standard of care. This Department is actively involved in medical audit, both with the Cardiology service and General Paediatrics. Rotating paediatric junior staff should attend the paediatric audit meetings. Each junior doctor will be expected to undertake an audit project during his or her post. 14 Participation in research projects is expected from all junior staff and opportunities will exist for presentations at audit meetings or to bodies such as the Welsh Paediatric Society and the British Congenital Cardiac Association. The consultants will provide help and advice on projects. 1.9 Computers and CardiobaseВ® Protecting Data п‚· All medical records held on computer are subject to the Data Protection Act. п‚· You will be provided with a password for accessing the hospital network. п‚· Dr Uzun bears responsibility with the IT Department for use of the network by the junior staff. п‚· Any abuse will be dealt with harshly. п‚· Do not divulge your password to non-unit staff. п‚· Remember to log-off after use, and use screensavers and other security measures. п‚· Never take non-encrypted electronic patient identifiable information away from the hospital setting. п‚· It is essential that care be taken to avoid the introduction of computer viruses to the network in order to protect the integrity of the patient database. o Sources of “infection" are unauthorised software, cds, dvds, floppy discs and memory sticks. o Before such software or storage media are used they must be scanned for viruses. п‚· Disciplinary action may ensue if these rules are not observed. CardiobaseВ® - see Section 3.6.1 for out-of-hours login instructions п‚· The Unit maintains a comprehensive patient database (Cardiobase) which includes details of each patient, the diagnosis, previous surgery, and events such as outpatient consultations and echocardiograms. п‚· Cardiobase is available to all staff for the extraction of information. п‚· Data input is reserved for trained individuals. п‚· Check with Wally James, who oversees Information Technology/Audit, before entering any data. 1.10 Medical Notes and Correspondence Headings Medical notes should always п‚· Be written legibly in black ink, be accurate and relate to the correct patient п‚· Have the patient’s name printed on each page п‚· Have entries which are dated, timed and legibly signed by the appropriate doctor or nurse relating to each patient contact – many signatures are illegible, so print your name and ideally your GMC number under the signature п‚· Record verbal advice given to patient or relatives п‚· Record explanation of risks/benefits of proposed treatment explained to patient or relative п‚· Contain results of investigations and record action taken on abnormal results п‚· Be contemporaneous - not written days later (and if they are written “after the fact” this should be stated) п‚· Be capable of being read out in court by the patient’s barrister 15 п‚· At discharge contain a list of all diagnoses including co-morbidities and procedures (get a senior member of staff to confirm the entries), avoiding the use of ambiguous abbreviations 1.10.1 Categories/Definitions of Admissions and Reviews Category Admission Day Case Admission Casual Ward Attender Outpatient Visit Inpatient Referral Explanation Patient is admitted to a bed. Nursing resource is used. The patient stays overnight at least one night. Patient is admitted to a bed for an investigation or procedure (e.g. Captopril challenge, sedated echo). Nursing resource is used. The patient is discharged the same day. The patient attends the ward without being admitted to a bed (e.g. blood test). A patient seen in a booked outpatient clinic (including Nurse-Led Clinic). A patient is referred by another team for review whilst they are an inpatient. This may be at UHW or another hospital. 1.10.2 Correspondence Headings Medical Discharge Summaries Date of Admission Date of Discharge Cardiologist Diagnosis Procedure History/Examination Investigations Management Status at time of discharge Weight at discharge Discharge medication Follow-up Risk of endocarditis Y/N Comment Dictate a “Discharge Summary” on Cardiobase Dictate a brief “Discharge Summary” on Cardiobase – mark it as a Day Case Dictate a “General Letter” on Cardiobase if treatment is changed or if the outcome of the visit needs to be communicated to the GP Dictate and “Outpatient Letter” or “Nurse-Led Clinic” letter on Cardiobase Dictate and “Inpatient Report” on Cardiobase Inpatient Reports (see Section 3.9) Date of consultation Referring Doctor Cardiologist Ward Reason for referral Pertinent history/examination Investigations Final diagnosis Advice given Follow-up Copies to __________ Top copy of form to patient notes Bottom copy for Copies to __________ dictation/secretaries 16 The service doctor of the day bears the responsibility of dictating the correspondence, which should be dispatched to the clinician/GP by 5 working days. Outpatient Letters Date of clinic / dictation Date of typing Cardiac diagnoses Other diagnoses Medication (+ any changes) Findings, including height and weight Investigations Communication with parents Follow-up Risk of endocarditis Y/N Copies to __________ Parents are sent a copy of clinic letters. Ensure that the summary at the end of the letter will be understandable to the parents and contains terms a layperson would understand. If the parents are separated/divorced take care not to divulge sensitive, confidential details of the estranged family. A pro forma Conference Reports is incorporated into the appropriate section on Cardiobase. 2 DAY TO DAY BUSINESS 2.1 Daily Timetable Monday 08:15 Planning Meeting Tuesday Sophie Pearson Room 09:00 OU and OCO Clinic Paeds OPD 12:30 CVS Science Meeting WHRI 12:30 Microbiology and Paeds Seminar Room or X-Ray Meeting (Paeds) KRUF Seminar Room 13:30 Nurse-Led Clinic KRUF Unit 09:00 DGW and VDO Clinic Paeds OPD Adult ASD closures/ MRI list Cath Lab / Radiology (non-GA) Wednesday 13:30 Speciality Doctor Echo Clinic POPD 13:30 BRHC Weekly M&M Meeting Meeting Room 6 14:00 BRHC Surgical Conference Meeting Room 6 08:00 Echo Meeting C3 Seminar Room 09:00 Academic Session Sophie Pearson Room 17 Thursday Friday 2.2 10:00 Teaching Ward Round Sophie Pearson Room 13:30 Adult Congenital Clinic Cardiology OPD 14:00 Paediatric Grand Rounds Academic Seminar Rm 09:00 Fetal Clinic (OU) Antenatal 13:00 SHO In-House Teaching KRUF or Paeds SemRm 14:00 CVS Imaging MDT Meeting 4th Thursdays, SP Room 09:30 Speciality Doctor Echo Clinic POPD 13:00 Paediatric Case Presentations Paeds Seminar Room 13:00 Cardiology Academic Meeting WHRI 13:30 Nurse-Led Clinic KRUF Unit UHW Clinics 2.2.1 General Clinics The outpatient setting will provide good experience in listening to murmurs and dealing with some of the long-term management issues in paediatric cardiac patients. Make use of these clinics by attending regularly. 2.2.2 Specialist Clinics Experience is also offered in the specialist clinics, including Marfan, Pacemaker (held monthly on 3rd Mondays), Adult Congenital and Fetal clinics. 2.2.3 Echo Clinic The paediatric cardiac speciality doctor undertakes an “Echo Clinic” on Tuesday PM and Friday AM. Referrals to the clinic are made via the consultant medical staff. The on call paediatric cardiologist provides consultant support for this clinic. The following types of referral are seen in this clinic: п‚· Oncology patients having routine inter-treatment scans where the question is ?LV function п‚· Nephrology patients where the question is ?LV function, ?LV hypertrophy or those pre- or post- renal transplant needing “routine” echocardiography п‚· Patients seen or discussed with a consultant general paediatrician with a murmur where the clinical assessment is that it is innocent and confirmation by echo is desired (patients where pathology is suspected should be referred to a formal paediatric cardiology clinic) The Echo Clinic is not intended as a rapid access clinic for GP referrals. 2.3 Outreach Clinics UHW consultants undertake >200 peripheral clinic sessions each year. If you make the effort to attend some of these clinics you will be rewarded with a wealth of training opportunities. Post-discharge patients should be slotted into one of these local clinics, where appropriate – check with the consultant. 18 Bi- monthly Neath Port Talbot Hospital Nevill Hall Hospital (Abergavenny) Monthly Prince Charles Hospital (Merthyr) Monthly Princess of Wales Clinic (Bridgend) Royal Glamorgan Hospital Monthly Bi-monthly 1.5 / month Royal Gwent Hospital 3.5 / month Singleton Hospital (Swansea) Weekly West Wales General Hospital (Carmarthen) Bi-monthly Withybush Hospital (Haverfordwest) Bi-monthly Dr Ofoe (+ Quarterly GUCH clinic Dr Wilson) Dr Onuzo Dr Ofoe (+ Dr Wilson GUCH 2x/yr) Dr Uzun Dr Ofoe Drs Wilson/Onuzo Drs Wilson / Onuzo / Uzun Drs Uzun / Onuzo / Ofoe (+ Dr Wilson Marfan Clinic 2x/yr, monthly GUCH clinic) Dr Ofoe (Dr Wilson 2 extra clinics per year) Dr Ofoe (Dr Wilson 2 extra clinics per year) 2.4 Teaching Topics Each rotation will have core teaching topics, including: п‚· Acyanotic heart disease п‚· Cyanotic heart disease п‚· Cardiac emergencies п‚· Basic ECG interpretation п‚· Arrhythmias п‚· Fundamentals of echocardiography п‚· Genetic influence on cardiac disease п‚· Case presentations п‚· Infective endocarditis п‚· Pacemakers п‚· Cardiac transplantation п‚· Surgical treatment of congenital heart disease Other more specialised topics can be taught on request. 2.5 Teaching Ward Round The main round is on Wednesday commencing at 10 am after the teaching session. Each patient is discussed prior to being seen. When presenting the patient’s details the following points should be covered: п‚· Name п‚· Age п‚· Diagnosis п‚· Date of admission п‚· Reason for admission 19 п‚· п‚· п‚· п‚· п‚· п‚· Pertinent social history Developmental and immunisation history Clinical findings Results of investigations Latest CXR Current management plan. All patients must have an up-to-date problem list and growth chart, including head circumference in infants. Whilst attached to the Department of Paediatric Cardiology, you should take the opportunity to learn the basics of echocardiography (e.g. recognise an effusion). SHOs undertaking MRCPCH examinations need to focus their attention on exam preparation and additional coaching will be provided. A variety of books and journals on congenital heart disease may be borrowed on request, but you must inform the relevant consultant. They must not be removed from the hospital and should be returned promptly. 3 ADMISSIONS AND REFERRALS 3.1 Routine Admissions All admissions receive a pack on arrival in Heulwen Ward that includes general information about the Unit, hospital procedures, layout and social service entitlements. A routine paediatric clerking is required for all admissions. Developmental status is important, as neurological problems noted post-operatively may have been present pre-operatively! New patients with dysmorphic features or complex disease should have chromosome analysis, renal and cranial ultrasound and a genetics referral. You must document: п‚· Diagnosis п‚· Previous cardiac catheters and operations п‚· Social, developmental, vaccination, coagulation (excessive bleeding/bruising) history п‚· Clinical status and exercise ability п‚· Height, weight and head circumference (plot these on a centile chart) п‚· 4-limb blood pressure (all new patients) п‚· Oxygen saturation п‚· ECG - arrange if one has not been performed in the past three months п‚· Chest X-ray - consider the need п‚· Echocardiogram findings п‚· Action plan and problem list п‚· Results of any investigations undertaken 20 3.2 Day Case Admissions 3.2.1 Echocardiogram under sedation It is common for infants to be admitted for echo under sedation. Admissions are arranged in advance through the medical secretaries. The degree of sedation required for successful echocardiography is “moderate” (refer to NICE guideline). Before sedation: Ascertain the following: п‚· Patient weight and baseline observations п‚· Fitness of the patient for sedation o Patients who do not have significant respiratory disease or and airway problem should be NBM for п‚і2 hours pre-sedation o In patients with significant respiratory disease or symptoms of airway obstruction the “2, 4, 6” rule should be applied (NBM 2o for clear fluid, 4o for breast milk and 6o for formula milk or solids) п‚· Possible role of play therapist for toddlers (using distraction as an alternative to sedation) п‚· Possible need for and timing of ECG and CXR п‚· Availability of echo machine п‚· Availability of consultant to perform the echo п‚· Obtain informed consent using a standard hospital consent form п‚· Document all clinical findings Exposure: Ensure the top half of the patient is adequately exposed before the patient is asleep. Monitoring: Saturation and ECG monitoring is required during and after sedation, until the patient is fully awake. Sedation: When you have ensured the above, ascertain the consultant’s preference, then EITHER give : п‚· Chloral hydrate 75 mg/kg po or pr (maximum dose 1000 mg). NB in some circumstances it is reasonable to give chloral with a milk feed – this can be done on consultant instruction. OR п‚· Midazolam 0.1 mg/kg into each nostril (total dose 0.2 mg/kg). Older patients do not tolerate the intranasal route – an oral preparation is available (oral dose 0.5 mg/kg). With intranasal midazolam, the patient may not fall asleep, but should become cooperative within 5-10 minutes. When chloral is used, if the patient is not adequately sedated by 20 minutes, contact the consultant and check whether topup sedation should be given (e.g. intranasal or oral midazolam). Inform the echocardiographer as soon as the patient is sedated, as the effects may only last a few minutes. Discharge: The nurses will work to a pro forma that indicates the patient is fit for discharge. The discharging doctor should be satisfied that: п‚· Vital signs have returned to normal and that the airway, breathing and haemodynamic state have returned to baseline п‚· The patient is easily roused п‚· The patient has taken a feed 21 Document any problems in the patient record, e.g. difficulties with sedation, and any complications. When the patient is discharged, ensure the handwritten GP note is given to the patient and a very brief summary is dictated. Reference: www.nice.org.uk/guidance/CG112 3.2.2 ACE inhibitor protocol ACE inhibitors are indicated in: п‚· Large Lп‚®R shunt п‚· Severe aortic regurgitation п‚· Severe mitral regurgitation п‚· Ventricular dysfunction п‚· Hypertension (e.g. following coarctation repair) Important recognised side effects of ACE inhibitors include: п‚· First dose hypotension } particularly in young infants п‚· Renal impairment } on diuretics п‚· Blood dyscrasias ACE inhibitors must be used with great caution in premature babies and infants < 1 month of age. When therapy is first instituted a test dose of 10 microgram/kg (0.01 mg/kg) is used with careful monitoring. The target dose is 50 microgram/kg (0.05 mg/kg). Protocol for institution of ACE inhibitors in older children: 1 Ascertain that the patient is physically fit, including baseline BP 2 Check renal function BEFORE the dose is given 3 Give test dose of CAPTOPRIL (0.1 mg/kg) 4 Arrange for nursing staff to check BP every 15 minutes for 90-120 minutes If there is no marked hypotensive response, prescribe a maintenance dose 5 of ACE inhibitor (check consultant preference – captopril may be continued [up to 0.5 mg/kg/dose tds; doses of up to 1 mg/kg tds may be used in older children], or it may be preferable to give a longer-acting ACE inhibitor such as enalapril or lisinopril) Arrange for repeat U+E/creatinine 1 week after discharge 6 7 Remember the GP note and brief typed discharge summary 8 Ensure appropriate OPD follow-up Young infants with a lean systemic output due to a large Lп‚®R shunt may tolerate captopril poorly. It is therefore usually instituted in inpatients and it is not uncommon to increase the dose very slowly over 3-5 days. If doses over 0.3 to 0.5 mg/kg/dose tds are used, potassium-sparing diuretics may need to be discontinued. Renal function should be checked on a regular basis as the dose is increasing. If a patient is intolerant to captopril or other ACE inhibitor because of a “captopril cough” an angiotensin II antagonist should be used instead. There is most experience with losartan. The initial dose is 0.5 mg/kg once daily, increasing to 1-2 mg once daily according to response. If an ACE inhibitor is being substituted, a straight swap of losartan in place of the ACE inhibitor can be made without needing a test dose. 22 Alternatives to losartan in older patients are valsartan or candesartan (indications: hypertension and/or heart failure). 3.2.3 Carvedilol protocol Some patients with dilated cardiomyopathy, or with other causes of heart failure may be commenced on a пЃў-blocker – usually carvedilol. In older children, use of bisoprolol or metoprolol can be considered. The recommended carvedilol protocol for introduction of therapy is as follows: Event Dosage of carvedilol Test dose 0.05 mg/kg First increment (1 week after test dose) 0.1 mg/kg bd Second increment (1 week later) 0.25 mg/kg bd Third increment (1 week later, if clinically indicated) 0.5 mg/kg bd Comment Day case admission (see note below). If tolerated discharge on 0.05 mg/kg bd. Outpatient or ward review: check clinical status, including weight and BP. Before increase: repeat ECG looking at HR and intervals. Consider need for echo. Ward review: check clinical status including weight and BP. Before increase: repeat ECG and echo – discuss findings with consultant Outpatient or ward review: check clinical status including weight and BP. Before increase: repeat ECG and echo – discuss with consultant and increase dose if clinically indicated. Consider need for repeat U&E/creat/LFT. Day Case admission for Test Dose п‚· Ascertain patient’s clinical status; if there is a history of asthma, discuss with the consultant п‚· Check baseline weight and observations, including blood pressure п‚· Obtain baseline ECG (?bradycardia/heart block) and echo including LV dimensions and fractional shortening п‚· Check blood for baseline U&E/creat/LFTs п‚· Give commencing dose of carvedilol 90-120 minutes either side of other medications that could cause hypotenstion (e.g. captopril and furosemide) п‚· Prescribe/give a dose of 0.05 mg/kg orally п‚· Check BP every 30 min for 2 hours post administration п‚· If tolerated well with no significant drop in BP, discharge on a dose of 0.05 mg/kg/dose twice daily – unless hypotension was a problem, the timing of doses can coincide with other vasodilators such as captopril п‚· Arrange for review in 1 week for reassessment and ?increase in dose (see Table above). 23 NB – patients with DCM who are under observation on the ward may have an accelerated up-titration of the carvedilol dose over the period of a few days, depending on clinical response. 3.2.4 Adrenaline/Epinephrine challenge for LQTS This may be of value in assessing catecholamine-sensitive forms of LQTS, specifically LQTS 1, LQTS 2 and LQTS 3 (to a lesser extent). LQTS 1 – raw QT prolongs rather than shortens LQTS 2 – T-wave morphology may change (becoming notched) LQTS 3 – pronounced shortening of the QT interval. Indications: п‚· Suspected LQTS (e.g. suspicious ECG, history of recurrent collapse) п‚· Screening for LQTS (family history of LQTS, but no genetic mutation information available) In Advance: п‚· An HDU bed should be booked 2 weeks beforehand п‚· Book the procedure with ECG (extn 3325) п‚· Pre-test U&E/creat (?K+ normal) and bone profile (?Ca2+ normal) – preferably prior to admission п‚· Patients already on пЃў-blockers should discontinue treatment 3 days beforehand Mixing up the infusion: 1. Prepare a solution of adrenaline/epinephrine 1 mcg/mL (by adding 1 mL of adrenaline 1:1000 to 1000mL saline) Dilution: Adrenaline 1 in 1000 = 1 mg/mL = 1000 mcg/mL Add 1mL Adrenaline (1 in 1000) into 1000 mL normal saline = 1 mcg/mL 2. Calculate total volume (of this dilution) required for test Total volume required for test: (Body weight in kg x 0.025) mL x 80 3. Prescribed infusion rate in mL/hour as per the table below Infusion rate (mL/min) = Wt x dose in mcg/kg/min Infusion rate (mL/hr) = Wt x dose in mcg/kg/min x 60 Infusion Protocol: Duration Dose 10 min 0.025 mcg/kg/min 5 min 0.05 mcg/kg/min 5 min 0.1 mcg/kg/min 5 min 0.2 mcg/kg/min Volume Per Minute (ml) Volume Per Hour (ml) Volume to be given (ml) (Wt x 0.025) ml (Wt x 0.025 x 60) ml (Wt x 0.025 x 10) ml (Wt x 0.05) ml (Wt x 0.05 x 60) ml (Wt x 0.05 x 5) ml (Wt x 0.1) ml (Wt x 0.1 x 60) ml (Wt x 0.1 x 5) ml (Wt x 0.2) ml (Wt x 0.2 x 60) ml (Wt x 0.2 x 5) ml Total Volume to be given (Wt x 0.025 x 80) 24 п‚· п‚· п‚· п‚· п‚· п‚·   Patient should be in a monitored HDU bed, but if this is not available, the supervising consultant may allow the test to be done on a monitored non-HDU bed on Heulwen. For this to take place there must be the requisite nursing and junior doctor support and the resuscitation trolley must be immediately to hand. Confirm K+ and Ca2+ results were normal Check availability of resuscitation equipment + IV Esmolol Consent for procedure (small risk of torsades or VT, very small risk of VF) Pre-test ECG (25 mm/sec and 50 mm/sec) and observations (HR, BP, RR, sats) See above for instructions on how to mix up the infusion. Start adrenaline infusion at 0.025 пЃg/kg/min, continue for 10 minutes, then increase infusion stepwise to 0.05, 0.1 and 0.2 пЃg/kg/min every 5 minutes (by doubling the infusion rate) Continuous ECG monitoring + 12-lead ECG (25 mm/sec and 50 mm/sec) at baseline, 10 min, 15 min, 20 min, 25 min, 30 min and 35 min Continue monitoring for 30 min afterwards (risk of torsades de pointes in susceptible individuals – if this occurs give IV Esmolol 0.5 mg/kg over 1 min followed by 0.05 mg/kg/min for 4 min). 25 Interpretation: Comparison of the change in absolute QT intervals (О”QT) among the genotypes. LQTS 1: >30 msec prolongation of absolute (i.e. non-corrected) QT interval recorded from an average of 4 measurements taken from lead II and lead V5 В± induction of torsades. (Sensitivity 92%, Specificity 86%, Positive Predictive Value 75%, Negative Predictive Value 96%). LQTS 2: No paradoxical QT prolongation, but low-dose adrenaline elicits G1 or G2 notching in greater than 50% on patients with LQT2 who have non-diagnostic resting ECG (see figure below). LQTS 3: No paradoxical QT prolongation, but accentuated shortening of QT intervals (this finding is suggestive, but not diagnostic) Reference and Figures: H.Vyas et al; Epinephrine QT stress testing in congenital long QT intervals; Journal of Electrocardiology, Vol 39 (2006), S107-S113 26 3.2.5 Brugada syndrome - Flecainide or Ajmaline Challenge http://pmj.bmj.com/content/80/950/723/F2.large.jpg п‚· п‚· Brugada syndrome is an autosomal-dominantly inherited cause of sudden cardiac death and is generally due to mutations of the SCN5A gene on chromosome 3. The hallmark ECG features of RBBB pattern with ST elevation in leads V1V3 are not always obvious in the resting ECG, but may be invoked or accentuated by blocking myocyte sodium channels using Class I antiarrhythmic agents, e.g. flecainide or ajmaline. Indications: п‚· Suspected Brugada syndrome (abnormal ECG with symptoms of palpitation or syncope) п‚· Screening of first degree relative with a confirmed diagnosis of Brugada syndrome (usually with symptoms or suspicious ECG). An HDU bed must be booked in advance, ideally 2 weeks before, but if this is not available, the supervising consultant may allow the test to be done on a monitored non-HDU bed on Heulwen. For this to take place there must be the requisite nursing and junior doctor support and the resuscitation trolley must be immediately to hand. Inform ECG department well in advance of the procedure. Drug administration: 1. Ensure drug available from pharmacy BEFORE the planned admission 2. Inform ECG Dept in advance (location and timing) – extn 3325 3. Ensure monitored bed 4. Obtain consent (1:200 risk of VF requiring DC cardioversion) 5. Patient to wear gown (open to the front to enable access to the precordium) 6. Baseline physical examination and observations 7. IV access 8. Continuous cardiac monitoring 9. Baseline 12 lead ECG, continuous rhythm recording with 12-lead ECG every minute pre- and post- drug infusion 10. Drug infusion: Ajmaline 1 mg/kg given iv over 5 mins (usual solution is 5 mg/mL, so volume of ajmaline to be infused is body weight divided by 5 – NB check 27 calculation!). Record ECG every minute for 5 minutes during infusion, then every minute for 5 mins after infusion, and at any time ST elevation is noted. OR Flecainide 2 mg/kg of neat solution iv over 10 minutes. Record ECG every minute for 10 minutes during infusion, then every minute for 10 minutes after infusion, and at any time ST elevation is noted. 11. If the test is positive – continue with 12-lead ECG every 2 minutes until ECG normalises. Continue to monitor patient for at least 30 minutes if the test is negative and for 60 minutes if it is positive or equivocal. 12. Inform consultant of results and enter results of study in “Exercise Test” section on Cardiobase. Positive Test: 2 mm ST elevation in one or more RV leads (V1-3). The ST segment is measured 0.08 sec (2 small squares) after the J point (junction of QRS complex with ST segment). In all positive individuals a VStim study should be considered to assess inducibility of ventricular arrhythmias and to measure the conduction intervals (the H-V interval is frequently prolonged in Brugada syndrome. Reference: Brugada.org 3.3 Admissions for Transoesophageal Echocardiography Admission: These patients are normally admitted the day before the procedure. This permits full cardiac evaluation and anaesthetic review. Straightforward, older TOE patients may be admitted as day cases with prior anaesthetic approval. Consent: This is obtained by the person undertaking the procedure. Discharge: Patients who have undergone TOE only may be discharged when they are drinking normally and have managed something to eat. 3.4 Admissions for MRI or CT scan All patients for MRI must have renal function checked prior to admission – the result should be made available to the MRI coordinator in Radiology. 3.4.1 Non-General Anaesthetic Admission: Children having cross-sectional imaging without GA should attend the paediatric day case unit 2 hours prior to the procedure. IV Access: Venous access should be established as early as possible to enable a period of heart rate recovery (use Emla or Amitop). Play Therapy: If the play therapist is to be involved, this should also be scheduled within this 2-hour window. пЃў-Blocker: Patients having CT should be prescribed propranolol 0.5 mg/kg 1 hour pre-procedure to attain a HR <80 bpm. (Ensure no contra-indication) Consent: Obtained by clerking doctor State that iv contrast medium is likely to be administered [small risk of contrast reaction]; if for CT – dose of radiation carries a very small risk of tumour development (~1:2500 or less). Discharge: Patients who have undergone CT without GA may be discharged immediately after the procedure if there is no evidence of an anaphylactic reaction to the iv contrast medium. 28 3.4.2 General Anaesthetic Admission: Children admitted for cross-sectional imaging under GA are routinely admitted the afternoon before the procedure to allow anaesthetic assessment. IV access: Not necessary for MRI scan (line placed by anaesthetist) For CT angiogram, there is a requirement for a heart rate < 80 bpm – this may be achieved by anaesthesia alone, but check with the consultant paediatric cardiologist / radiologist / anaesthetist whether propranolol should be prescribed (ensure no contraindication). If so, a dose of 0.5 mg/kg should be given 1-2 hours before the procedure. In infants, place an IV line and prescribe maintenance dextrose/saline for 4 hours pre-procedure to prevent hypoglycaemic reaction. If a пЃў-blocker is not to be given pre-procedure, there is no need for line placement. Consent: Obtained by clerking doctor (state that iv contrast medium is likely to be administered – small risk of contrast reaction); if for CT – dose of radiation carries a very small risk of tumour development (~1:2500 or less). Discharge: Patients who have undergone CT under GA may be discharged when they are drinking normally and have managed something to eat. NB – infants under 60 wks post conceptual age (i.e 20 wks/5 months corrected age) must be kept in for observation overnight (small risk of apnoea). 3.4.3 Adenosine Stress Test by MRI Aim To evaluate myocardial perfusion reserve Protocol Baseline MRI scan (with or without GA) Adenosine dose: Mix 4 amps of Adenosine (25mg/5ml) into 80ml normal saline (total 100 mL) This gives a concentration of 1mg/mL (Alternatively pre-order from pharmacy a pre-mix 120ml Adenosine bag of 1mg/mL) Give Adenosine at 140 пЃg/kg/min for 6 minutes using an infusion pump. (i.e 140 пЃg X weight = ………..пЃg of adenosine per minute for a total of 6 minutes) Inject contrast 3 minutes into Adenosine infusion Monitor heart rate, BP, ECG at rest, 1 minute intervals during adenosine infusion and continue for 5 minutes post termination of infusion Practical considerations Need 2 IV access lines – 1 for the adenosine, 1 for anaesthetic drugs Infusion pump for Adenosine - ensure availability of Adenosine and saline Resuscitation equipment Aminophylline as antidote for Adenosine-induced bronchospasm Ensure availability of anaesthetist / radiologist 29 Contraindications Patients with active bronchospasm / reactive airway disease Patients with more than first-degree heart block Patients with low BP Patients on dipyridamole or methylxanthines Early Termination Severe hypotension Symptomatic Mobitz-I second-degree heart block Mobitz-II or third-degree heart block Bronchospasm Severe chest pain associated with ECG changes Adverse effects Systemic effects include dizziness, headache, symptomatic hypotension, dyspnoea, and flushing. The main GI side effect is nausea. Cardiac effects include chest pain and ST-segment changes. Monitoring Chart for Adenosine Stress Test (with or without GA) Heart Rate Blood Pressure ECG changes Remarks Baseline 1st minute (infusion) 2nd minute (infusion) 3rd minute (infusion) 4th minute (infusion) 5th minute (infusion) 6th minute (infusion) 1st minute (recovery) 2nd minute (recovery) 3rd minute (recovery) 4th minute (recovery) 5th minute (recovery) NB-Print out the above chart for documentation when overseeing this test (Highlight desired text, then “print selection”) 3.5 Non-Cardiac Admissions and Casual Ward Attenders Occasionally children with congenital heart disease are admitted to the ward for dental treatment, plastic surgery or ENT procedures. The main reason is to provide cardiac supervision during the admission and allow anaesthesia in main theatres with full cardiac back up. The new NICE guideline for preventing infective endocarditis 30 should apply – unless specified by the consultant (e.g. very high risk cases) no antibiotic cover is given. Patients attend the ward for a number of reasons, including post-transplant supervision, INR estimation, post-operative review, parental concern, and so on. Some of these patients can be reviewed in the Nurse-Led Clinic to avoid burdening the ward nurses. The patient's diagnosis, active problems, weight, and current progress should be noted. If there are any concerns then the registrar or consultant should be informed. Any significant changes in therapy should be communicated with the patient's GP. 3.6 Emergency admissions and procedures 3.6.1 Patients known to the Department The Unit operates an "open door" policy for all known patients. If parents contact you about their child, judge whether the patient should be seen urgently on the ward (cardiac problem) or by their GP and referred to the Children’s Assessment Unit (e.g. for general paediatric problems). Following a full clerking, the consultant on-call should be informed of the admission whatever the time day or night. If telephone advice is given, please use the “Telephone Advice” pro forma kept on Heulwen ward. Access to Cardiobase out of hours: Patient information is held on Cardiobase. The database is accessible out of hours from Heulwen Ward PCs. Log-in to any of the ward PCs, double-click on the Cardiobase icon, log in as Doctor (username) Junior (password). This is read-only access – do not attempt to alter the patient record. 3.6.2 New Referrals New patients will be referred to the Unit from a variety of sources (e.g. clinic, GPs, paediatricians etc.). The consultant may prefer to see neonatal referrals at the cotside in the referring hospital, so check before arranging transfer to UHW. Many neonates, once seen by the UHW cardiologist, are referred directly from the referring hospital to the surgical centre. п‚· п‚· п‚· Ascertain the basic history and clinical status of the patient from the referring doctor (e.g. age of child, oxygen saturation, presence of cardiac failure, ventilation status, and overall condition). The name and telephone number of the referring doctor should be noted and then the cardiologist contacted. Remember to contact the referring hospital when the cardiac condition is established and update them on the diagnosis and treatment plan. 3.6.3 Neonatal Admissions Ensure the birth weight and gestational age are documented in the medical records. Special considerations apply to the management of neonates with suspected congenital heart disease as many will have duct dependent lesions and some single 31 ventricle physiology. The history and examination are important in distinguishing cardiac from respiratory causes. Investigations should include the O 2 saturation, 4-limb BP, chest X-ray, ECG, FBC, U&E and capillary blood gas (if unwell). Many will have duct dependent lesions and there should be a low threshold for starting prostaglandin. Even despite this mixing may be inadequate and urgent balloon atrial septostomy may be necessary. Those with single ventricle physiology (eg HLHS) will require a different ventilation strategy and babies with dysmorphic features (e.g. DiGeorge Syndrome) irradiated, CMV negative blood products. Indications for prostaglandin E therapy: Physiology Diagnosis Critical PS Low pulmonary blood flow Pulmonary atresia Tricuspid atresia Critical AS Severe CoA Low systemic blood flow HLHS Interrupted aortic arch Inadequate mixing Transposition of the great arteries Presentation Profound cyanosis п‚± Murmur (Cardiogenic shock) Cardiac failure Cardiogenic shock Poor or unequal pulses Cyanosis Tachypnoea Collapse 3.6.4 Protocol for PGE infusion (NB risk of apnoea) (a) Wt x 30 = number of microgram of PGE added to 50 mL of 5% or 10% dextrose 1 mL/hr п‚є 10 nanogram/kg/min OR (b) 50 microgram in 50 mL of PGE added to 50 mL of 5% or 10% dextrose 0.3 mL/kg/hour is equivalent to 5 nanogram/kg/min Protocol (a) is recommended, as this is the one used by BRHC. Starting dose is 5-10 nanogram/kg/min In unwell and/or ventilated patients doses of up to 50 nanogram/kg/min can be used, and titrated downwards depending on response. Caution: high initial doses of PGE can precipitate apnoea in unventilated patients. There is also an association between the use of high-dose PGE and necrotising enterocolitis. 3.7 Emergency Interventional Procedures 32 3.7.1 Balloon Atrial Septostomy BAS may be required to improve mixing between the pulmonary and systemic circulations in conditions such as: п‚· Transposition of the great arteries п‚· Tricuspid atresia п‚· Pulmonary atresia with intact septum. In most cases BAS may be performed semi-electively in the surgical centre, but in many cases it is a life-saving procedure in a collapsed neonate. In most circumstances BAS is performed with sedation and local anaesthesia п‚± muscle relaxant in a ventilated neonate. In experienced hands BAS is usually a very safe procedure, but there is a risk of serious complications including: п‚· Bleeding п‚· Pericardial tamponade п‚· IVC avulsion п‚· Mitral valve damage п‚· Stroke п‚· Arrhythmia. The risk is higher in certain situations (e.g. in the context of juxtaposed atrial appendages, atrial septal aneurysm, or interruption of the IVC). The risks of the procedure must be weighed against the risk of delaying life-saving intervention. Except in life-threatening situations, high risk BAS should be delayed until transfer to a surgical centre. The on-call Bristol interventional consultant may be asked to undertake emergency septostomy in a Welsh hospital. They will bring a septostomy kit with them. Prerequisites for BAS п‚· Skilled operator п‚· Support staff trained in safe airway management (this may be an intensivist, neonatal consultant or an experienced NICU registrar) п‚· Safe environment with full monitoring and resuscitation equipment (e.g. PICU or NICU) п‚· Equipment necessary for the procedure (brought by Bristol consultant) п‚· 2 units of cross-matched blood п‚· Informed consent from the parent/guardian 3.7.2 DC Cardioversion (DCCV) Patients with a shockable rhythm in cardiac arrest should receive DCCV as part of the resuscitation algorithm (this is undertaken wherever the arrest is being managed and does not require separate sedation or anaesthesia). DCCV outside the context of cardiac arrest may be needed in cases of shockable atrial or ventricular arrhythmia with cardiovascular compromise, e.g. rapid SVT not responsive to medication, VT with a pulse but with features of shock, or where semiurgent DC cardioversion is needed as “first-line” therapy, e.g. atrial fibrillation or flutter. Prior to carrying out DCCV, ensure the following: 33 п‚· п‚· п‚· п‚· п‚· The patient should be nil by mouth (the “2,4,6” rule applies except in dire emergency: NBM 2 hours for clear fluid, 4 hours for breast milk and 6 hours for formula milk or solids) Inform the on call paediatric anaesthetist Inform Main Theatres (3099) Inform PICU (in case admission is required afterwards) Obtain formal consent. The potential risks include: o Intractable cardiac arrest o Neurological deficit o Surface irritation or burns to skin o Risks of anaesthesia Procedure: п‚· Once the patient is anaesthetized and stable, set up the defibrillator as per APLS/PLS guidance. п‚· Ensure you are recording the process on paper. п‚· Mark events on the machine if this facility is available. п‚· Apply appropriate energy – usually 0.5 J/kg as the first shock. The energy is increased to 1, then 2 J/kg as necessary for future shocks. If an energy level of 2 J/kg is unsuccessful, consider changing pad position to anterior-posterior (apex and L subscapular), or reverse polarity (reverse position of apex and subclavicular paddles). After DCCV, admission to PICU should be considered if the patient remains haemodynamically unstable – have a low threshold if ventricular function is poor. Consider what prophylactic anti-arrhythmic medication is required. 3.7.3 Pericardiocentesis for cardiac tamponade Aetiology Cardiac tamponade may accompany a variety of illnesses, such as viral infections (e.g. coxsackie, mumps, adenovirus, and HIV), bacterial infection (e.g. TB), immune mediated diseases (e.g. rheumatic fever, Kawasaki disease), connective tissue diseases (e.g. JRA, SLE) and uraemia. Pericardial effusion is also a common post-operative complication. It usually resolves spontaneously or following treatment with diuretics п‚± anti-inflammatory drugs such as aspirin or ibuprofen. Often the effusion is a feature in the early post-operative period and patients are discharged from the surgical centre only when it is clear the collection is not increasing in size. In other cases the effusion may only become apparent many days or even weeks post-operatively and forms part of the postcardiotomy (Dressler’s) syndrome. Clinical features п‚· Prodromal illness п‚· Chest pain п‚· Disappearance of a previous pericardial rub п‚· Tachycardia 34 п‚· Gallop п‚· Tachypnoea п‚· Hepatomegaly and raised JVP (evidence of elevated venous pressures) п‚· GI upset п‚· Pulsus paradoxus п‚· Muffled heart sounds п‚· Cardiac arrhythmia ECG: low voltages, ST-T changes Management Post-operative pericardial effusions are monitored with careful clinical and echocardiographic examination. Patients with features of tamponade or impending tamponade require urgent pericardiocentesis. Faced with a patient with tamponade/impending tamponade, the clinician must judge whether it is safer to (a) perform a pericardial tap locally, or (b) transfer the patient to the surgical centre for pericardiocentesis. The potential risks of inadvertent myocardial or coronary damage must be weighed against that of cardiac arrest during transfer. As the worst-case scenario is the need to perform urgent pericardiocentesis during transfer, the patient should be accompanied by an individual capable of performing this procedure (e.g. APLS or PALS trained). Except in dire emergency, the patient should be nil by mouth (“2, 4, 6” rule applies). Technique The removal of even a small amount of fluid from the pericardial space can be life saving and attempted relief of tamponade is preferable to the consequences of nonintervention. In an emergency any large intravenous cannula can be used. Clean the xiphoid and subxiphoid areas. Echo guidance is recommended Use local anaesthetic if necessary Attach the syringe to the needle Puncture the skin 1-2 cm inferior to the left side of the xiphoid junction at a 45o angle Advance the needle towards the tip of the left scapula aspirating all the time When fluid is withdrawn advance the cannula over the needle and withdraw the needle Remove as much fluid as possible If the fluid is bloody then it can be difficult to decide if the needle is in the pericardial space or heart as the blood will pulsate out of the needle even if it is in the correct place. If the fluid is squirted onto a white swab it is often obvious if the blood is fresh or old. When echo is available a saline can be injected through the needle and will readily demonstrate if it is in the pericardial space or heart If the cannula is in the correct place the clinical condition of the patient will rapidly improve as fluid is withdrawn. Prerequisites for Pericardiocentesis п‚· Skilled operator 35 п‚· п‚· п‚· п‚· п‚· п‚· Anaesthetist or intensivist and appropriate support staff Safe environment with full monitoring and resuscitation equipment (e.g. operating theatre, PICU) Equipment necessary for the procedure Ability to admit/transfer to PICU should complications or the patient’s clinical condition dictate 2 units of cross-matched blood Informed consent from the parent/guardian Possible scenarios 1. Well child with increasing significant pericardial collection with no signs of response to treatment. Appropriate management – arrange for transfer to surgical centre for semi-urgent pericardiocentesis. 2. Child with significant pericardial collection and impending collapse due to tamponade. Appropriate management – transfer to Main Theatres or PICU for urgent pericardiocentesis (paediatric anaesthetist / intensivist support). 3. Collapsed child. Appropriate management – perform pericardiocentesis at bedside as part of resuscitation (monitored bed). Transfer to PICU for post-arrest management. 3.8 Post Surgical Transfers (in) Following cardiac surgery some cases are transferred back to Cardiff prior to final discharge home. This is usually because their postoperative course is slow or complicated. They may have: п‚· Feeding problems п‚· Persisting haemodynamic problems п‚· Pericardial effusion п‚· Persisting respiratory problems п‚· Persisting neurological problems п‚· Ongoing infection See the relevant section in Clinical Problems or Surgical Complications. All patients transferred to Cardiff for post-operative management need an up-to-date ECG, CXR and echocardiogram. 3.9 п‚· п‚· п‚· п‚· п‚· Inpatient Referrals Inpatient referrals are frequently received from neonatologists, paediatric surgeons and general paediatricians. Before reviewing the patient, ensure the consultation has been requested by/discussed with the referring consultant. It is essential to clarify the clinical question being posed by the referring team. If the question is not clear, discuss this with the cardiac consultant on-call. These patients should have an outpatient-style consultation, a full examination and an ECG, echocardiogram, and chest x-ray (where appropriate). 36 п‚· п‚· п‚· п‚· The findings should be discussed with the on-call consultant, preferably well before the end of the normal working day – remember all echos are reviewed by a consultant. Details of the consultation should be documented in the case record notes, including information about the echocardiogram, and an inpatient report dictated. Ensure copies of this report are sent to the parents, the GP and the patient’s DGH (since the parents are receiving a copy, ensure the summary at the end is in plain English). Inpatient referrals should be reviewed as necessary and follow-up arrangements should be clearly stated. NB – There is almost no such thing as a referral for “echo only”. Exceptions are follow-up neonatal echos (check known PDA), or chemo patients having LV function assessment between courses (if there have been no previous problems). All other echo requests are regarded as a “referral for cardiology opinion”. Inpatient referral checklist: Was the referral generated by a consultant? Is the clinical question clear? Take a history and perform a clinical examination Arrange essential investigations (consider need for CXR) Are the findings adequately documented in the medical record? Has the case been discussed with the consultant? Has the clinical problem been addressed? Has the outcome been clearly communicated to the referring team/GP/DGH paediatrician? Has an inpatient report been dictated, checked, signed and dispatched? Are any outpatient arrangements in place? See Section 1.10 for Inpatient Report headings. 4 4.1 п‚· п‚· п‚· п‚· DISCHARGE AND TRANSFER PROCEDURES General Principles Before transferring or discharging a patient, imagine yourself as the doctor receiving the transfer, or the paediatrician in the DGH, the GP, or the consultant seeing the patient in clinic in a few weeks’ time. Also picture yourself as the parent taking their child home after a stressful time in a specialist centre. Have they received clear communication about the status of their child and the management plan? Do they know whom to contact if they are worried? Clear written (and often verbal) communication is paramount. On discharge, patients will be given the standard hand-written note to take to their GP. Sufficient drugs should be prescribed for up to one month (if needed that long). 37 п‚· Parents should be told to contact their GP and local pharmacy soon after discharge for a repeat prescription as many of the drugs commonly prescribed from hospital (e.g. furosemide suspension) need to be specially ordered and the chemist will require a few days to obtain them. It is the responsibility of the SHO or registrar on-call the day of the patient’s discharge to ensure a summary is dictated that day. See Section 1.10 for a guide to discharge summary headings. Summaries on patients with protracted or complicated admissions should be dictated or checked by one of the registrars. As a minimum standard, all discharge summaries should be dictated, typed and posted within five working days of discharge from the hospital. 4.2 Transfers to Other Hospitals Before transferring a child to the surgical centre consider what level of transfer should be undertaken. п‚· Well patients may be transferred safely in the family car or in a hospital taxi with a nurse escort. п‚· Patients on minimal support (e.g. intravenous drugs given by bolus, patient receiving low-flow oxygen) may be appropriately transferred by the nursing staff. п‚· Patients receiving a higher level of support (e.g. prostaglandin infusion) must be transferred with an experienced member of the medical staff with a paramedic crew. Intubated patients will normally be transferred under the supervision of NICU or PICU consultant staff. п‚· Wherever possible, a typed summary should be provided and this should detail all of the relevant medical details, including an up-to-date medication list. п‚· In some circumstances a photocopy of the patient’s medical notes should also be sent. If time constraints do not permit a typed summary, a clear hand-written note is require, but a summary should be dictated for typing later. This permits accurate audit of transfers out of the Unit. Transfer Checklist Is there a clear indication for transfer? Ensure patient is fit for transfer Communicate with medical staff in the receiving unit Ensure appropriate ambulance has been arranged Ascertain which members of the medical/nursing team should accompany the patient Written transfer letter (preferably typed) Document current medications and recent lab results Should any of the notes be copied? Ensure the proper equipment is available (transfer pack) Ensure safe level of monitoring equipment Telephone receiving hospital just prior to departure Ensure a discharge summary is dictated 38 4.3 п‚· п‚· п‚· Discharges Following Cardiac Surgery The standard hand-written GP note should be provided and the post-surgical handout for the parents should also be given. Where the post-operative course has been prolonged or complicated, the DGH consultant and GP should be informed of the discharge by telephone. A typewritten summary should be dispatched within five working days. Wound Care and Physical Activity: п‚· Normal bathing is permitted but the wound should be protected until the scabs have come off. п‚· Parents should know that if the child is unwell prior to the OPD appointment, the ward should be informed and a review will be arranged. п‚· Most children should not attend school until after their first review in NurseLed Clinic or OPD. As a general rule the child should avoid PE (or equivalent activity) for 6/52 and contact sports (or equivalent) for 3/12. Other diseaserelated activity restrictions may apply. п‚· Follow-up Arrangements: 2 week appointment Nurse-Led Clinic, 4 week appointment Cardiology OPD (at local hospital where possible) – ensure these arrangements are in place. Discharge Checklist Is the patient fit for discharge? Are the medical notes complete with full documentation of the patient’s status? Has there been adequate communication (with parents, nursing staff, GP, referring paediatrician? Is the GP letter/TTH complete? Are suitable follow-up arrangements in place? Has the discharge summary been dictated? 4.4 4.5 Death of a Patient Inform the on-call consultant immediately and the child’s consultant, even if not oncall, at the earliest opportunity. Consider the need for the coroner's office to be informed of the death. It is Unit policy to ask the family to consider a hospital PM (either full or limited) if the death is not a coroner’s case. Use the new hospital PM consent form. Contact: п‚· GP and Health Visitor п‚· DGH Consultant п‚· Birth Clerk/Community Paediatrics (to prevent routine appointments being sent to Cardiff patients) п‚· Tony Bradley (Social Worker, who is also trained in bereavement counselling) – NB Tony can only be directly involved with the family if they have given their consent – if appropriate, try to seek this consent during the initial bereavement discussions п‚· Cardiac Liaison Nurse п‚· Consultant Obstetrician (in cases of neonatal death) п‚· Consultant Cardiac Surgeon (in the case of post-operative death) 39 п‚· If the death was unexpected, consider the need to inform the Clinical Director and the Child Death Review team As the notes are usually “lost” in the pathology department after death a summary should be dictated at the time the death certificate is signed. Copies of this should be sent to everyone who has been involved in the patient's care, including previous hospitals. If not all the information is available for the summary at this time explain an additional report will follow. A leaflet is available on the ward that gives advice to relatives of the deceased about obtaining the death certificate and contacting funeral directors. The patient’s consultant will normally write to the family of the deceased child a short time after the event to see if they want to discuss the death of their child further. 5 INVESTIGATIONS 5.1 Drug Monitoring and/levels Drug Investigation Specimen Amiodarone TFT, LFT, CXR and consider Opthalmology (changes are reversible) Lithium heparin bottle Bosentan U&E, LFT (must specify ALT and AST); add FBC if Eisenmenger Lithium heparin bottle (plus EDTA if having FBC) Ciclosporine Drug level EDTA bottle Digoxin Drug level Plain/clotted sample Flecainide Drug level and ECG Plain/clotted sample Tacrolimus LFT, U&E, FBC and drug levels Specimen bottle for Tacrolimus EDTA Comments Check TFT and LFT at baseline, at 1 month and then every 6 months. Perform CXR every 12 months. Drug levels (+ metabolites) can be checked through Toxicology Lab, but rarely indicated. Transaminases may become elevated on bosentan. Bloods done monthly. Fax results to GOS 0207 905 2321 Desired range varies – check with transplant team. Fax results to GOS 0207 813 8440 Trough level is needed (6 hours post dose) Desired level = 1.0-2.0 пЃg/mL Check U&E at same time. Consider need for ECG if level abnormal. Trough level 6 hours after last dose. QRS duration on ECG should lengthen by <25% of baseline. Desired level = 0.15-0.9 Desired range varies – check with transplant team (bleep 0600 via GOS Switchboard for dosing advice). Fax results to GOS 0207 813 8440 5.2 Electrocardiogram During working hours these are requested via the ECG Department (6396). Fill in a yellow request form. ECGs may be performed out-of-hours using the machine kept on Heulwen Ward. Familiarise yourself with the use of this machine. 40 ` Patients admitted with a suspected arrhythmia should have a 12-lead ECG in the arrhythmia, during pharmacological or other cardioversion therapy, and once sinus rhythm has been restored. 5.2.1 Basic ECG Interpretation For more detailed information about quantitative ECG interpretation (e.g. age-related intervals), look on the “Measurements” section of the shared directory. When assessing and ECG you should determine the following: п‚· Correct patient demographics п‚· Heart rate п‚· Rhythm п‚· Electrical axis п‚· PQRST morphology п‚· Ventricular forces п‚· Intervals o PR o QRS duration o QT The ECG computer package will calculate the intervals for you, but errors in the QT measurement are common and you should ideally calculate the corrected QT interval (QTc) yourself. A QT interval >440-460 msec could signify the presence of long QT syndrome where the use of certain drugs is contraindicated (for a list see www.QTdrugs.com). The most commonly-used method for QTc calculation is the Bazett formula: R-R interval QT interval interval QTc = QT_______ пѓ–RR interval 41 The Bazett formula over-corrects for slow and under-corrects for fast heart rates. An alternative method, particularly for faster heart rates, is the Framingham formula: QTc (in msec) = QT (in msec) + (1000 – RR interval in msec / 6.5) 5.3 Echocardiogram For more detailed information about normal values (including Z-score graphs), look on “Measurements” section of the shared directory. During working hours, echos on cardiac patients are arranged via the Echo Technician (Ms Viv Booker – Extension 3920, Bleep 5503). Requests for echo should be communicated to her early in the day. She has clinic commitments Monday, Tuesday and Thursday AM, and Wednesday and PM. Echo referrals from other teams: п‚· There is almost no such thing as a referral for “echo only”. п‚· Exceptions are follow-up neonatal echos (check known PDA), or chemo patients having LV function assessment between courses (if there have been no previous problems). п‚· All other echo requests are regarded as a “referral for cardiology opinion”. п‚· Ensure the referral has been discussed by the child’s lead consultant. Ensure the formal referral request document is filled in with adequate information. п‚· Remember – all echo reports are confirmed by a consultant - ensure echos for review are brought to the attention of the relevant consultant well before the end of the normal working day. п‚· If there are referrals for echocardiography out of normal working hours, this must be discussed with the consultant on call. 5.3.1 Echocardiogram to rule out cardiac source of embolism Young patients with stroke should have a detailed echo to exclude a cardiac source of embolism. This includes assessment of right-to-left shunt across PFO with bubblecontrast echocardiography which requires an experienced echocardiographer and assistant. The recommended protocol is as follows: п‚· Intravenous cannulation is usually performed in the upper limb. п‚· The procedure for Valsalva with release is explained to both the subject and the parents. The subject is instructed to inhale deeply and exhale through the nose. Concurrently either the subject or the parent blocks the nasal passages externally with release on indication from the echocardiographer. п‚· Baseline transthoracic echocardiogram is performed. 5 ml of normal saline is drawn up into a 10 ml Luer-lock syringe and attached to a three-way tap with a further 10 ml Luer-lock syringe attached to another port. The final port is connected to the subject. п‚· The saline is agitated with air and the subject’s blood (5 ml 0.9% saline to 0.5 ml of air and 0.5 ml of patient’s own blood) by plunging it between the two syringes, ensuring that they are fully screwed on to prevent leakage of blood. When the saline mix is sufficiently agitated, initial injection with the patient breathing normally is performed to assess spontaneous passage of contrast into the left atrium. Sniffing can be requested at this time as this can sometimes open up a PFO and trigger a bubble shunt. A short sharp sniff is needed. 42 п‚· п‚· If no shunt is seen, the subject is instructed to exhale forcefully against the blocked nasal passages and mouth while a slow injection of agitated saline is performed. A slow injection is needed to ensure adequate contrast is present in the right atrium throughout the Valsalva. After an adequate Valsalva, where the left heart should become visibly smaller, and with contrast present in the right atrium, the echocardiographer indicates to release the Valsalva and the number of bubbles crossing to the left atrium is assessed. This is repeated on up to five subsequent occasions, ensuring an adequate study. Adequacy in this case means that a Valsalva caused a reduced size in the left heart and was released when bubbles were filling the right atrium. o Small shunt - <6 bubbles o Medium shunt 6–20 bubbles o Large shunt >20 bubbles o Massive shunt - there would be opacification of the left ventricle. False-positives: п‚· Pulmonary arteriovenous malformations (these always give a shunt without Valsalva, bubbles can be visualised coming down the pulmonary veins usually within 3 heart beats п‚· Use of colloid rather than saline to mix the contrast (in some patients, colloid contrast may pass through normal pulmonary capillaries allowing microbubbles to reach the left atrium when no PFO is present). Reference: Arch Dis Child 2008;93:255-259 5.4 Exercise Test Contact the ECG Department and fill in a request form. Most children over 4 years of age should be able to manage the exercise treadmill. 5.4.1 Indications for exercise test These include: п‚· Objective assessment of exercise tolerance п‚· Assessment of sinus node disease п‚· Provocation of exercise-induced arrhythmias п‚· Heart rate response in complete heart block п‚· Risk assessment in WPW п‚· Assessment of long QT syndrome (use modified Bruce protocol, regardless of age) п‚· Assessment of peak oxygen consumption (VO 2 peak) and aerobic threshold (cardio-pulmonary exercise test, CPET) п‚· Assessment of severity of asymptomatic severe aortic stenosis* п‚· Risk assessment in hypertrophic cardiomyopathy* *meticulous attention to BP required (see below) 5.4.2 Bruce protocols The method of assessment at UHW is the exercise treadmill. It provides information on the haemodynamic and electrocardiographic response to a staged, progressive increase in load over a short period of time. In relatively fit, older patients the standard Bruce protocol should be used (arrange with the ECG technician). In 43 younger patients, those with moderate or severe exercise limitation, and those being assess for long QT syndrome, the modified Bruce protocol should be employed. Other measurements employed on occasion: п‚· Saturation data (does the patient desaturate on exertion?) п‚· Peak flow rate pre- and post-exertion (is there exercise-induced asthma?) п‚· Echocardiography (how high does the aortic gradient go on exertion?) Exercise test protocol: п‚· Check resting ECG (technician) and BP (doctor) п‚· Checking standing ECG (technician) п‚· Patient commences exercise п‚· Check BP and 12-lead ECG every 3 minutes (prompted by machine) п‚· Watch for arrhythmias п‚· Continue until patient exhausted, there is a medical indication to stop, or the patient has reached the end of Stage 5 (standard Bruce) and the target HR is achieved п‚· Document reason for cessation п‚· Take report to consultant’s secretary (a report will be entered on Cardiobase by the consultant or one of the registrars) п‚· If the test was terminated early because of an unexpected abnormality, please discuss this with the patient’s consultant, or the service consultant before the patient is allowed home 5.4.3 Indications for termination: п‚· Genuine patient exhaustion п‚· Significant arrhythmia (SVT, increasing ventricular ectopy, VT, CHB) п‚· Hypotension or failure to increase BP with symptoms of dizziness or chest pain; hypertension with systolic BP >220-240 п‚· Chest pain with significant flat or downsloping ST depression or ST elevation (>2-2.5 mm in the absence of pre-existing bundle branch block) 5.4.4 п‚· п‚· п‚· п‚· п‚· п‚· Difficulties in interpretation Breathlessness due to other cause (e.g. asthma) Pre-existing BBB, especially LBBB LVH WPW Digoxin (affects ST segments – consider stopping drug prior to exercise test) пЃў-blocker therapy (blunts HR response and may mask ischaemia– consider stopping drug prior to test) 5.4.5 Helpful tips п‚· Continual gentle encouragement of the patient will maximise the information obtained in the study п‚· BP should be measured with the patient’s arm lifted off the equipment and supported by the operator – this minimises machine noise п‚· If there are difficulties auscultating the BP, use the palpation technique (radial or brachial artery) 44 п‚· п‚· a surrogate for peak BP Meticulous BP measurement is essential in patients being exercised with HOCM – a flat BP response (failure of systolic blood pressure to rise by more than 20–30 mm Hg from baseline), drop in BP during exertion, or precipitate drop in BP post-exertion are all poor prognostic signs (Circulation. 1997;96:2987-2991, J Am Coll Cardiol 2000;36:2212–8) The need for careful BP measurement also applies in AS – a flat BP response signifies important AS 5.5 Ambulatory ECG Monitoring This is requested through the ECG Department (6396). Reports should not be filed in the patient record until the result has been entered on to Cardiobase. 5.5.1 Holter monitoring Uses include: п‚· Investigation of palpitations, atypical seizures and syncope of unknown aetiology п‚· Assessment of severity of known arrhythmias п‚· Assessing cardiac rhythm in those at risk of arrhythmia (e.g. Tetralogy of Fallot, post Senning or Mustard procedure, Fontan circulation, pre-excitation on ECG, 1st or 2nd degree heart block) п‚· Risk assessment of cardiomyopathy, CHB and LQTS п‚· Assessment of pacemaker function п‚· Assessment of response to anti-arrhythmic medications Holter monitoring can be undertaken in any age group and can run from 24 hours up to one week (you need to specify the required duration on the request). 5.5.2 Event recorders Two types of device are provided: CardioCall and King of Hearts. They are issued for up to two weeks. Both devices can be set up as a loop recorder (attached via leads to the chest continuously; when the patient presses a button the device saves the latest loop) or placed on the chest for recording during symptoms (patient activated). Rarely, a recording device may be implanted subcutaneously (Reveal device), but this is reserved for severe, infrequent episodes. If an episode is experienced, the patient needs to attend the ECG department for the device to be interrogated. 5.6 Ambulatory BP Monitoring Blood pressure varies significantly over a 24 hour period and single, elevated measurements do not necessarily reflect the true situation. Ambulatory monitoring during routine daily activities provides several readings throughout the day and night, and offers a profile of blood pressure during rest as well as activities. It is especially important in differentiating spuriously high readings (white coat effect) from true sustained hypertension. It is helpful in: п‚· Assessing whether anti-hypertensive therapy should be commenced (e.g. after CoA repair) п‚· Assessing BP control in patients taking anti-hypertensives 45 п‚· п‚· Deciding on optimal time to take anti-hypertensive medications Evaluation of hypotensive symptoms in patients taking anti-hypertensives, diuretics and ACE inhibitors The cuff is permanently on the arm and the recorder placed on a belt or rucksack. The non-dominant arm is normally used, but in coarctation patients it is mandatory to check the right arm BP (there may be a BP gradient between the upper limbs). There are several ambulatory monitors available for use in paediatric patients. Most use the oscillometric technique and correlate the reading with simultaneous recording of the heart rate which is also plotted on the graph. These monitors print the actual readings and mean BP values during time periods throughout the 24 hours. Treatment decisions should be based on the mean values over the monitored period and take into account the shape of the profile. Normal 24 hour profiles show a fall in the blood pressure level during sleep - evidence suggests that patients with no fall (non-dippers) have an increased risk of end organ damage. Oscillometric mean ambulatory BP values in healthy children: Percentile for 24 hr period Height in cm (n) 50th Daytime percentile * 95th Nighttime percentile †50th 95th 50th 95th Boys 120 (33) 105/65 113/72 112/73 123/85 95/55 104/63 130 (62) 105/65 117/75 113/73 125/85 96/55 107/65 140 (102) 107/65 121/77 114/73 127/85 97/55 110/67 150 (108) 109/66 124/78 115/73 129/85 99/56 113/67 160 (115) 112/66 126/78 118/73 132/85 102/56 116/67 170 (83) 115/67 128/77 121/73 135/85 104/56 119/67 180 (69) 120/67 130/77 124/73 137/85 107/56 122/67 Girls 120 (40) 103/65 113/73 111/72 120/84 96/55 107/66 130 (58) 105/66 117/75 112/72 124/84 97/55 109/66 140 (70) 108/66 120/76 114/72 127/84 98/55 111/66 150 (111) 110/66 122/76 115/73 129/84 99/55 112/66 160 (156) 111/66 124/76 116/73 131/84 100/55 113/66 170 (109) 112/66 124/76 118/74 131/84 101/55 113/66 180 (25) 113/66 124/76 120/74 131/84 103/55 114/66 *Daytime: 8 am to 8 pm / †Nighttime: midnight to 6 am Reference: The Journal of Pediatrics Volume 130, Issue 2, February 1997, Pages 178-184 46 5.7 Tilt Test Indications: п‚· Investigation of unexplained collapse п‚· Suspicion of vasovagal syncope (a.k.a. neurocardiogenic syncope or neurally mediated syncope) п‚· Assessment of therapy Procedure п‚· The test is booked through the ECG department п‚· The test is overseen by a doctor with APLS or PALS certification п‚· Check resuscitation equipment and drugs prior to beginning п‚· Ensure you have a drug chart and a supply of GTN (400 microgram dose) п‚· Explain to the patient and parents what the procedure involves; mention that if GTN is used it may cause a headache п‚· No IV line is required for sublingual GTN Tilt Test but make sure that an immediate access to IV line insertion is available in any emergency situation. NB - Insert IV cannula before the test only if this is specified on the request form (possible reasons: (a) the history is very positive for collapse, or (b) isoprenaline is to be given [dose 0.02-0.05mcg/kg/min until baseline heart rate rises 25%-30%]); use Ametop or Emla as appropriate; allow 15-20 min recovery before commencing test п‚· The ECG technician will “wire up” the patient and secure the supporting straps on the tilt table п‚· Continuous heart rate and BP monitoring is required п‚· Allow the patient to lie supine for 10-15 mins п‚· Stage I: Drug Free State: o Tilt the table (head up!) to 70 degrees for 20 minutes, recording heart rate and BP continuously o Make a note of any significant symptoms (nausea, sweating, presyncope) and check BP immediately. o If the patient blacks out lay the table flat immediately and ensure the ECG is being recorded continuously o If the patient does not experience syncope within 20 minutes, proceed directly to Stage II without returning the patient to supine п‚· Stage II: Pharmacological challenge with sublingual GTN 400-500 microgram spray: o Administer 400 microgram of sublingual GTN and continue the tilt for a further 15-20 minutes. o Make a note of any significant symptoms (nausea, sweating, presyncope) and check BP immediately. o If the patient blacks out lay the table flat immediately and ensure the ECG and blood pressure are being recorded continuously o If no syncope occurs with GTN terminate the procedure and return patient to supine position. Allow patient to rest for 10-20 minutes until they feel ready to stand up. Provide a written assessment of the test on the print-out. Discuss the results with the requesting consultant and ensure a report is entered into Cardiobase п‚· 47 5.8 п‚· п‚· п‚· п‚· MRI or CT Scan Useful in assessing cardiac anatomy (particularly the great vessels), cardiac function, severity of valve regurgitation and other haemodynamic issues. Scans are organised through Dr Andrew Wood, Consultant Cardiovascular Radiologist. Scans under GA have to be booked well in advance (see section 3.4 for admission arrangements for inpatient scans). If the patient has pre-existing renal dysfunction there is a small risk of a rare but irreversible skin complication called nephrogenic fibrosing sclerosis. o To guard against this adverse effect it is now a requirement to provide the MRI coordinator with “up-to-date” U&E/creat (i.e. within 4 months of the scan). o Depending on the age and location of the patient this test can be done by the GP, the local DGH or at UHW prior to the investigation. o In practice it is best to have the results at least a week before the scan is due – try not to leave until day of admission for MRI. 5.9 Isotope Scans These use a small dose of radio-isotope, which is excreted in the urine. Ensure the child’s mother is not pregnant! Sedation may be required; intravenous access will be required – co-ordinate with the children’s Clinical Investigation Unit. 5.9.1 Cardiac Nuclear Scanning Main indications include assessing myocardial ischaemia and ventricular function. Requests need to be discussed with Dr Richard Wheeler (adult cardiologist). 5.9.2 Lung perfusion scan Organise through Dr John Rees, Consultant Radiologist. Indications are exclusion of pulmonary embolus (V/Q scan) and assessment of differential lung perfusion (e.g. in branch PS). 48 6 6.1 CLINICAL PROBLEMS Anti-platelet Therapy and Anticoagulation 6.1.1 Aspirin and Anti-platelet Therapy Indications for use of aspirin in children (plus see tables below): п‚· Anti-inflammatory action – treatment of o Acute pericarditis o Kawasaki disease (acute phase) o Acute rheumatic fever п‚· Anti-platelet therapy – treatment of o Kawasaki disease (convalescent phase) o Systemic-pulmonary shunt o Chronic cyanosis (e.g. cavopulmonary shunt, Eisenmenger syndrome – relative indication) o Prosthetic valve with history of embolism despite anticoagulation (added therapy) If aspirin cannot be used (e.g. allergy), consider the use of other agents such as dypridamole or clopidogrel (limited data in children). In the event of development of chicken pox, herpes, influenza, rubella, or other severe flu-like febrile illness, the clinician will determine whether the risks / benefits of continuing aspirin vs the small risk of Reye’s syndrome. Parents should be instructed to telephone to ask for advice in this situation. Patients on aspirin for a B-T shunt should not have therapy discontinued, even during a febrile illness; however patients with a weaker indication for aspirin (e.g. chronic cyanosis with a cavopulmonary shunt, Kawasaki disease) should discontinue aspirin temporarily during the feverish phase of an illness. The consultant may consider use of dypridamole or clopidogrel during this period (NB the data sheet for clopidogrel also advises discontinuation during chicken pox, etc). 6.1.2 Devices and Stents Indication Treatment Duration ASD device Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* Not indicated 6 months VSD device Aortic stent Pulmonary artery stent PDA device 49 6 months 6 months 6 months *NB – there may be individual clinical reasons to extend treatment, to supplement with other antiplatelet agents (e.g. clopidogrel), or to use formal anticoagulation. Aspirin dose of 150 mg or 300 mg may be used in older adolescents. See section 6.1.1 for advice regarding use of aspirin during intercurrent illness. 6.1.3 Valve replacement Indication Treatment Duration Tissue valve Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* 6 months (triscuspid, mitral, aortic) Tissue RV-PA conduit or pulmonary valve (any type, including transcatheter implant, Ross operation) 6 months Prosthetic valve Warfarin – target INR 3.0 Indefinite В± 0.5 (i.e. range 2.5-3.5)** *NB – there may be individual clinical reasons to extend treatment, to supplement with other antiplatelet agents (e.g. clopidogrel), or to use formal anticoagulation. Aspirin dose of 150 mg or 300 mg may be used in older adolescents. See section 6.1.1 for advice regarding use of aspirin during intercurrent illness. **NB – there may be individual clinical reasons to use a higher range, e.g. small valve, or increased risk of thrombosis – this needs to be clearly specified in the medical notes and in the INR booklet. Our unit experience tells us that an upper range of 4 has been very safe in the past. (any type including mitral or aortic) 6.1.4 Cavopulmonary shunt / Fontan Indication Treatment Duration Glenn / superior cavopulmonary shunt Aspirin 3-5 mg/kg once To continue until Fontan / daily, usual maximum 75 TCPC** mg once daily* Fontan / TCPC Warfarin – target INR = Indefinite 2.5 В± 0.5 (i.e. range 2-3) *NB – See section 6.1.1 for advice regarding use of aspirin during intercurrent illness. **NB – There may be individual clinical reasons to extend treatment, to supplement with other antiplatelet agents (e.g. clopidogrel), or to use formal anticoagulation. 6.1.5 Other indications Indication Treatment Duration Modified Blalock-Taussig Shunt Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily Kawasaki Disease Refer to AHA or UK guidelines EITHER Aspirin 3-5 mg/kg once daily, usual maximum 75 mg once daily* To continue until next definitive surgery; treatment to continue with febrile illness Refer to AHA or UK guidelines Indefinite Pulmonary Arterial Hypertension 50 OR Warfarin – target INR range 1.8-2.5 Post L heart Aspirin 3-5 mg/kg once 3 months electrophysiology ablation daily, usual maximum 75 mg once daily* *NB – there may be individual clinical reasons to extend treatment, to supplement with other antiplatelet agents (e.g. clopidogrel), or to use formal anticoagulation. Aspirin dose of 150 mg or 300 mg may be used in older adolescents. See section 6.1.1 for advice regarding use of aspirin during intercurrent illness. References: 1. 2. 3. Guideline on antiplatelet and anticoagulation management in cardiac surgery Eur J Cardiothorac Surg 2008;34:73-92 Valvular and Structural Heart Disease. Chest 2008; 133: 593S - 629S. Antithrombotic Therapy in Neonates and Children. Chest 2008;133;887S-968S 6.1.6 Commencing anticoagulation (heparin and warfarin) Most patients will be commenced on heparin first. Therapy with unfractionated (standard) heparin Loading dose: 50 – 100 U/kg Maintenance: 10 – 20 U/kg/hr (higher doses may be needed) Check APTT/KCCT 6 – 8 hours after starting therapy. Aim for ratio of 1.5 – 2.0. If APTT ratio is LOW – increase maintenance dose by 10-20% and recheck APTT ratio 6-8 hours later. If APTT ratio is >2 and ≤3, reduce the maintenance dose by 10% and recheck APTT ratio 6-8 hours later. If APTT ratio is >3, stop heparin for 1 hour, then restart at a reduced maintenance dose (reduce by 10-20%). NB – if maintenance doses of >35 U/kg/hour are required it may be appropriate to accept slightly low APTT ratios. Therapy with low molecular weight heparin Indications for use: п‚· Neonates needing anticoagulation п‚· Any patient requiring anticoagulation and deemed to be at risk of haemorrhage п‚· Patients in whom venous access for administration and monitoring of heparin is difficult. Baseline investigations: п‚· FBC п‚· PT п‚· APTT п‚· ?thrombophilia screen (if indicated) 51 Target anti-factor Xa levels: Treatment dose: 0.5-1.0 U/mL Prophylactic dose 0.1-0.3 U/mL Starting dose: Age < 2 mo Age 2 mo – 18 yr o 1 mg/kg/dose SC q 12o 1.5 mg/kg/dose SC q 12 Treatment dose 0.75 mg/kg/dose SC q 12o 0.5 mg/kg/dose q 12o Prophylactic dose The maximum dose is 2 mg/kg/dose q 12o, i.e. 4 mg/kg/day – see BNF for adult dose Effectiveness of therapy is assessed by monitoring Anti-Xa levels; the ideal dose is the one that reflects plasma anti-Xa concentrations of 0.5-1.0 U/mL 4 to 6 hours after injection. The lab routinely checks anti-factor Xa levels on Monday/Wednesday/Friday afternoons. Requests outside of this time must be discussed with the lab/haematologist. Adjusting LMW heparin in children (therapeutic dose): Anti-Xa Hold next Dose Repeat anti-Xa level dose? change? measurement (U/mL) Increase by <0.35 No 4 hours after next dose 25% Increase by 0.35 - 0.49 No 4 hours after next dose 10% Next day, then 1 week later, 0.5 - 1.0 No No and monthly thereafter (4 hours after morning dose) Decrease by 1.1 - 1.5 No Before next dose 20% Decrease by Before next dose then 4 hours 1.6 - 2.0 3 hours 30% after next dose Before next dose, then every Until anti-Xa 0.5 Decrease by >2.0 12 hours until anti-Xa level U/mL 40% <0.5 U/mL Long-term therapy – check bone densitometry every 6 months. Source: ABC of antithrombotic therapy. BMJ 2003;326:93-96 + BCH policy Loading with warfarin Remember to check baseline PT/INR. If PT is >1.3, the patient will need a lower loading dose. The recommended loading dose is 0.1 – 0.2 mg/kg. In practice the following guide may be applied: 52 Age group Infants Young children Older children Teenagers Loading dose 1 – 2 mg 3 – 5 mg 5 – 8 mg Day 1: 8 mg / Day 2: 8 mg Adults 10 / 10 / 5 mg over successive days Comment Check INR next day Check INR next day Check INR next day Check INR on the morning after second dose Check INR 2nd – 3rd day In patients on heparin who are being loaded on warfarin, it is essential to send a full coagulation screen. A high KCCT/APPT will lead to an erroneously high INR level. If only the INR is checked, the true reading off heparin may be much lower. 6.1.7 INR Sampling It is the responsibility of the person taking the blood sample for INR estimation to find out the result and prescribe the appropriate dose of anticoagulant (see INR protocol below). An INR machine is kept on the ward most of the time except for quality-control when it is in the Cardiac Liaison Office. Special strips are used with the machine (these are available on prescription but a supply is kept on the ward). Anyone who has not been trained to use the machine should not be operating the machine. Ask one of the Cardiac Liaison Nurses to show you how the machine is operated. Basic instructions are as follows: 1 Gather all the necessary equipment (INR machine, coagucheck strip, pen and lancet, cotton wool balls) 2 Turn machine on Check battery power (batteries need charging when showing only one bar) Check date and time are correct Ensure coagucheck strip code corresponds with machine – press the “M” button to confirm 3 The machine is ready when the “strip” symbol flashes on the display. Put in strip. Machine begins to count down from 180. 4 From the time the countdown starts, you have 180 seconds to place a FRESH (<15 sec old) drop of blood on to the hole/reservoir in the strip. Use as large a drop as possible. When the drop is applied the machine flashes a “clock” symbol on the display to show the INR is being processed. If the drop was too small error 5 flashes on the display. 5 The machine then displays the INR reading – record this in the INR books. If an INR reading is unexpectedly deranged, consider the cause (intercurrent illness, drug interaction, alcohol ingestion). 6.1.8 INR Protocol Use this protocol when prescribing warfarin. If unsure, check with consultant. (see Warfarin Dosage Table in section 6.1.9) 53 INR <1.8 NB with prosthetic mitral valve, if INR is under 2.2-2.4, discuss with consultant >1 below target range >0.5 – 1.0 below target range 0.2 – 0.5 below target range Target range <0.2 above target range 0.2 – 1.0 above target 1.01 – 1.5 above target range >1.5 above target range INR >6 or history of bleeding* *see Table below Action/Comment Discuss with consultant. Arrange admission for heparinisation in patients with a prosthetic valve. Patients with other indications for anticoagulation may need increased dose or limited re-load, but this can often be overseen as an outpatient. Increase dose by 3 increments Increase dose by 2 increments Increase dose by 1 increment No change in dose No change (depending on trend) Reduce dose by 1 decrement Reduce dose by 2 decrements Halve dose, discuss with consultant; repeat INR next day; restart with dose reduced by 1 decrement. Discuss with senior – confirm reading with lab sample. Very high INR levels will require hospital admission with consideration of FFP or vitamin K. Consider discussing case with Haematology team.* Recheck INR: п‚· Stable (and no worrying trend up or down) п‚· Dose changed 1 place up or down п‚· Dose changed >1 place up or down п‚· Half dose / reload 4-6 weeks 2 weeks (depending on trend) 1 week (depending on trend) 1-3 days Health Board Policy for high INR (Feb 2004) vs Unit experience Clinical Situation UHB Policy Unit Experience INR 6-7.9 with no bleeding: Stop warfarin; restart when INR <5 INR 8-10 with no or minor bleeding Stop warfarin; restart warfarin when INR<5; give vitamin K 0.51 mg iv (adult dose); recheck INR next day Stop warfarin; restart warfarin when INR<5; give vitamin K 0.51 mg iv (adult dose); daily INR; consider hospital admission Confirm reading with lab sample; reduce usual warfarin dose (at least halve), recheck daily Confirm reading with lab sample; omit warfarin, restart when INR <5; admit for daily INRs Confirm reading with lab sample; stop warfarin; restart when INR <5; consider vit K or prothrombin complex concentrate (discuss with Haematology); admit for daily INRs HDU or PICU admission; Haematology consultation INR >10 with no or minor bleeding Major bleeding with high INR Stop warfarin; involve Haematology; give prothrombin complex concentrate (PCC)*; take blood to measure pre- and post-concentrate INR and coagulation factors; give vit K *PCC dose: INR 2-3.9 [25 U/kg]; INR 4-5.9 [35 U/kg]; INR >6 [50 U/kg] 54 6.1.9 Warfarin dosage table (doses shown in mg) 10 9.5 / 10 / 10 9.5 / 10 9.5 / 9.5 / 10 9.5 9 / 9.5 / 9.5 9 / 9.5 9 / 9 / 9.5 9 8.5 / 9 / 9 8.5 / 9 8.5 / 8.5 / 9 8.5 8 / 8.5 / 8.5 8 / 8.5 8 / 8 / 8.5 8 7.5 / 8 / 8 7.5 / 8 7.5 / 7.5 / 8 7.5 7 / 7.5 / 7.5 7 / 7.5 7 / 7 / 7.5 7 6.5 / 7 / 7 6.5 / 7 6.5 / 6.5 / 7 6.5 6 / 6.5 / 6.5 6 / 6.5 6 / 6 / 6.5 6 5.5 / 6 / 6 5.5 / 6 5.5 / 5.5 / 6 5.5 5 / 5.5 / 5.5 5 / 5.5 5 / 5 / 5.5 5 4.5 / 5 / 5 4.5 / 5 4.5 / 4.5 / 5 4.5 4 / 4.5 / 4.5 4 / 4.5 4 / 4 / 4.5 4 3.5 / 4 / 4 3.5 / 4 3.5 / 3.5 / 4 3.5 3 / 3.5 / 3.5 3 / 3.5 3 / 3 / 3.5 3 2.5 / 3 / 3 2.5 / 3 2.5 / 2.5 / 3 2.5 2 / 2.5 / 2.5 2 / 2.5 2 / 2 / 2.5 2 1.5 / 2 / 2 1.5 / 2 1.5 / 1.5 / 2 1.5 1 / 1.5 / 1.5 1 / 1.5 1 / 1 / 1.5 1 0.5 / 1 / 1 0.5 / 1 0.5 0 / 0.5 / 0.5 0 / 0.5 0 / 0 / 0.5 NB 5 / 5 / 5.5 means give 5 mg Day 1, 5 mg Day 2 and 5.5 mg Day 3; then repeat the cycle 55 6.1.10 Cessation of warfarin for surgical or invasive procedure Generally speaking it is safe to undertake a “minor” procedure (cardiac catheterisation, dental extraction, minor non-cardiac surgery) with an INR of 2.2-2.4 or less. Where the type of surgery is associated with a high risk of bleeding (e.g. spinal surgery) an INR of <1.6 may be desirable. Patients with prosthetic heart valves* need careful peri-operative management. The following approach is recommended, although if the risk of operative bleeding is low it may be safe to proceed with the operation on a slightly reduced dose of warfarin: п‚· Check INR 3 days before planned procedure (the parents can do this if they have a home INR monitor) п‚· If the INR was in therapeutic range, give the normal dose 3 days pre-op (if not, discuss with consultant) п‚· Discontinue warfarin therapy 2 days before procedure and admit to hospital п‚· Repeat INR daily п‚· If INR falls below 2, start iv heparin therapy (including loading bolus) with the aim of maintaining APTT/KCCT ratio 1.5-2.0 п‚· Discontinue heparin therapy 6 hours before surgery п‚· Recommence heparin (with reloading bolus if surgery was lengthy) once bleeding is controlled; aim for APTT/KCCT ratio 1.5-2.0 п‚· Recommence warfarin (consider gentle loading dose) and maintain heparin until INR is therapeutic for 24-48 hours п‚· Remember – a full lab coagulation screen is needed for patients on heparin and warfarin NB: Clexane (fractionated heparin) may be used to replace or supplement continuous iv heparin in some circumstances – discuss with the adult haematology registrar. *Please note that patients who are anticoagulated for dilated cardiomyopathy or following a Fontan procedure generally do not require the above steps in preparation for invasive procedures – warfarin therapy can be stopped ~2 days prior to the procedure. It is advised to check the INR prior to the procedure ensuring the reading is <2.2-2.4, then normal therapy can be resumed afterwards. 6.1.11 Factors that influence the efficacy of warfarin Patient factors: п‚· Enhanced anticoagulation effect with o Weight loss o Intercurrent illness o Liver disease o Heart failure o Renal failure o Excess alcohol ingestion п‚· Reduced anticoagulant effect o weight gain o D+V o Asian or Afro-Caribbean background 56 Drug interactions with warfarin: п‚· Reduced protein binding o Aspirin o Chlorpromazine п‚· Inhibition of warfarin metabolism o Erythromycin o Sodium valproate o Cimetidine o Cranberry juice п‚· Enhanced metabolism of warfarin o Phenytoin o Carbamazepine o Phenobarbitone п‚· Reduced synthesis of coagulation factors II, VII, IX and X o Phenytoin o Salicylates п‚· Reduced absorption of vitamin K o Broad-spectrum antibiotics o Laxatives Enhanced risk of bleeding is seen with aspirin and other NSAIDs, corticosteroids, thrombolytics. Consider prescribing ranitidine to reduce the risk of GI bleeding. Monitor INR more closely if new medications are prescribed, or if the patient is acutely unwell. (Source: ABC of antithrombotic therapy. BMJ 2002;325:762-4) 6.2 Asplenia and Immunodeficiency It is not uncommon for cardiac patients to have specific immune problems. If there is any doubt in dealing with immunosupressed patients, consult with the Public Health (Virology) and Immunology Departments (Dr Stephen Jolles). 6.2.1 Asplenia This occurs in cardiac children with right atrial isomerism (known as “asplenia syndrome in the USA). Children with left atrial isomerism (“polysplenia syndrome”) may also have functional hyposplenism and this needs to be assessed carefully. Children with absent or dysfunctional spleen tissue are at risk of overwhelming infection particularly with encapsulated organisms such as Pneumococcus, Meningococcus and Haemophilus influenza. Other high risk infections in this group include malaria, E. coli, Babesiosis (tick borne disease) and infections transmitted by animal bites. 57 Asplenic children under 5 years have a particularly high infection risk of overwhelming sepsis (>10%). They should receive п‚· Lifelong prophylactic antibiotics – because cover against Haemophilus influenzae is needed in <5s, Amoxycillin is recommended o Child 1 month–5 years Amoxycillin 125 mg bd o Child 5–12 years Amoxycillin 250 mg bd Child 12–18 years Amoxycillin 500 mg bd In over 5s the risk of H influenzae is reduced and it is reasonable to give prophylactic phenoxymethylpenicillin (penicillin V) – but check with the local infection control team to confirm local sensitivities: o Child 6–12 years Penicillin V 250 mg bd o Child 12–18 years Penicillin V 500 mg bd Erythromycin is the alternative in patients allergic to penicillin. п‚· п‚· Standard vaccination schedule, including live vaccines Influenza vaccination each Autumn (reduces the risk of secondary bacterial infections) п‚· Pneumococcal vaccine o <24 months of age give 3 doses of conjugate vaccine at monthly intervals, followed by 1 dose of polysaccharide vaccine at 2 years of age o >24 months (if not immunized so far), give 2 doses of conjugate vaccine at monthly intervals, followed by a dose of polysaccharide vaccinee o Booster pneumovax doses should be given every 5 years п‚· Meningococcal A vaccine if travelling into an endemic area Advice for family: п‚· Parents should be made aware of the excess infection risk and the need for immediate treatment of suspected infection. п‚· They should have a reserve supply of antibiotics at therapeutic doses to take on holiday. п‚· They should be given the Asplenia Warning Card to carry. п‚· The excess risk from malaria should preclude unnecessary travel to endemic areas. п‚· Antibiotics should be given after animal bites (Augmentin) and despite prophylactic antibiotics a serious suspected infection should be given immediate treatment with cefotaxime or ceftriaxone. п‚· Isomerisms can be familial therefore other family members should be screened. References: 1 – Davies JM, Barnes R, Milligan D. Update of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. Clin Med. 2002 Sep-Oct; 2(5): 440-3. 2 – Finn A, Booy R, Moxon R, Sharland M, Heath P. Should the new pneumococcal vaccine be used in high-risk children? Arch Dis Child. 2002 Jul; 87(1): 18-21. Review. 6.2.2 DiGeorge syndrome and chromosome 22 microdeletion DiGeorge syndrome has a broad spectrum of clinical features, not all of which maybe present in any individual patient. Although many patients have an immune defect, it is 58 not always present and when present it may be of variable severity. The features include: п‚· Characteristic facial appearance п‚· Low serum calcium concentration п‚· Congenital abnormality of the heart or great vessels (particularly conotruncal abnormalities) п‚· Absent thymus gland with abnormal lymphocyte numbers of function п‚· Chromosome 22 q11 microdeletion (95% detected by FISH study with TUPLE probe) Although the index case may be a new mutation the inheritance is autosomal dominant thus parental chromosomes should also be assessed. The absence of a thymus gland, although suggestive of thymic aplasia, does not necessarily mean that its function is grossly deranged. The total number of lymphocytes and the numbers of T and B cells can be very helpful. Low numbers of T cells are suggestive but not diagnostic of the DiGeorge anomaly. Likewise abnormal lymphocyte function may show very variable results. In addition the immune systems of children with DiGeorge can develop in the absence of the thymus gland and these children’s immunity usually improves with time. Sometimes however the T cells can be normal at birth but can then decrease and so initially immuno-competent children can be become compromised later on. It is useful to assess the number of T & B cells at 5-8 months. If a blood transfusion is required then any cellular blood products should be irradiated in order to avoid a graft-vs-host immune reaction. Until tests of immunity show otherwise contact with other people who have coughs, colds or any other infections should be avoided. Give normal human immunoglobulin (NHIG) if in contact with measles and VZIG if in contact with chicken-pox or shingles (seek advice from the consultant virologist). Any infections must be treated very promptly. If the T cells are low in number or have abnormal function the child should receive prophylactic co-trimoxazole to prevent infection with Pneumocystis carinii. No live vaccines should be administered (Polio [Sabin], Measles, Mumps, Rubella, BCG). NB In the new immunisation schedule the killed polio vaccine (Salk) is given and this can be administered to patients with compromised T-cell function. Siblings and household contacts of should receive Salk vaccine, as live polio vaccine can be excreted for 6 weeks. Hypocalcaemia should be sought for and treated appropriately. Blood should be taken at the age of 5-8 months to check the presence of antibodies to the vaccines administered and re-assess the lymphocytes numbers. (Source: Clinical Immunology) 6.3 Cardiac Failure Aetiology: Congenital Anomalous origin of left coronary artery Cardiomyopathy Coarctation Acquired Cardiac tamponade Cor pulmonale (BPD, CF) HIV 59 Critical aortic stenosis Interrupted aortic arch Large Lп‚®R shunt Severe valve regurgitation TAPVD Truncus arteriosus Infective endocarditis Myocarditis Myopathies Severe anaemia Severe hypertension SVT Congestive heart failure is a maladaptive state that is triggered by initially beneficial mechanisms. It is best understood as a chronic inflammatory condition initiated by the adrenergic system in an effort to restore adequate tissue perfusion and oxygen supply. It is often triggered by a mismatch between the myocardium and its loading conditions. Heart failure should be distinguished from myocardial failure and circulatory failure. Myocardial failure can lead to heart failure but is not synonymous with it. Many children in heart failure have normal myocardial function e.g. large VSD. Conversely, some patients with impaired myocardial function are clinically not in heart failure. Similarly, although heart failure leads to circulatory failure, there are patients in circulatory failure who at least initially have normal cardiac function e.g. acute blood loss or severe diarrhoea. The management of heart failure is the same irrespective of the initial insult that caused it. This is because treatment is directed at the maladaptive mechanisms that have produced the clinical syndrome and not primarily at the initial insult. It is however also necessary to discern what the underlying triggering mechanism is, in order to provide a long-term solution. Pathophysiology The two basic adaptive mechanisms that the heart uses to respond to abnormal loading conditions are: 1. The Frank-Starling mechanism 2. Activation of neuro-humoral systems. The Frank-Starling mechanism enables the heart initially to generate increased force of contraction. This is however at the expense of increased wall stress and therefore disadvantageous to myocardial perfusion and fibrosis. After a few weeks this leads to impaired myocardial function. Neurohumoral mechanisms restore circulating blood volume and increase myocardial mass as well as rate and force of myocardial contraction. In time however these also cause myocardial failure from increased oxygen requirement, apoptosis, myocardial fibrosis and imbalance between myocardial size and tissue receptors and organelles. The main stay of modern treatment of heart failure is therefore focused on two of these systems namely the renin-angiotensin system and the adrenergic system. Signs and Symptoms of Heart Failure These will vary with age at presentation: 60 п‚· п‚· Infants – tachypnoea, sweating, poor feeding, vomiting and poor weight gain Older children – poor appetite, effort intolerance and failure to thrive Findings include: п‚· Tachycardia with or without gallop rhythm п‚· Tachypnoea, recession with or without basal crackles п‚· Hepatomegaly Investigation п‚· ECG п‚· Chest x-ray п‚· Echocardiogram п‚· Oximetry п‚· Capillary gas (if unwell) Treatment п‚· Oxygen to maintain saturation (beware that excessive use can make heart failure worse in left to right shunts) п‚· Consider sedation in the distressed child п‚· Treat fluid overload – fluid restriction and diuretics п‚· Prevent further salt and water retention – diuretics, angiotensin coverting enzyme (ACE) inhibitors, and angiotensin receptor blockers п‚· Tachyphylaxis – пЃў-Blockers and digoxin (maintain digoxin level at 0.51.0ng/ml) п‚· Increase myocardial contractility – Inotropes or inodilators (PDE inhibitors preferably) and digoxin Cardiac Output п‚µ Blood pressure and 1/п‚µ Vascular Resistance Blood pressure is commonly assumed to reflect cardiac output. This is incorrect. In the face of increased vascular resistance blood pressure may in fact be “normal” but the cardiac output is not. Equally there may be a low blood pressure in patients with an “adequate cardiac output” if there is a low systemic vascular resistance e.g. sepsis, or due to use of vasodilators. The adaptive mechanisms respond to oxygen delivery to the tissues. This in turn depends more on the sufficiency of the cardiac output for the loading conditions rather than the level of the blood pressure. An adequate cardiac output implies appropriate blood flow to the tissues and should allow the patient to produce urine and maintain normal acid-base balance. Cardiac output should therefore be assessed in other ways independent of blood pressure. Clinical assessment of cardiac output: There is no suitable objective method of measuring cardiac output in children and thus assessment of an adequate cardiac output relies in indirect methods using other validated objective criteria. These include vital signs and other reproducible clinical criteria. It should also be recognised that circumstances can change rapidly. 61 Parameter Heart rate Blood pressure Arterial waveform Pulses Peripheral perfusion Core-toe temperature gap Urine output Base deficit Mental status Adequate CO normal normal large area under curve; late dicrotic notch peripheral pulses easy to feel capillary refill < 2sec < 2oC >1 ml/kg/hr <2 mmol/L co-operative Inadequate CO elevated normal or low small area under curve; early dicrotic notch peripheral pulses weak central pulses palpable capillary refill >2sec > 2oC <1 ml/kg/hr >2mmol/L agitated, disorientated Pre-load affects cardiac output. If there is a low preload then the cardiac output is reduced. A low pre-load will be manifest as a low CVP and/or left atrial pressure. Often there is a large respiratory swing of the systemic blood pressure. The appropriate treatment is to give volume. Pre-load will also be decreased in tamponade as the heart cannot fill due to external compression. Diastolic ventricular relaxation also affects the ventricular filling and hence output. Relaxation is affected by a high sympathetic drive or muscle disease (e.g. hypertrophic cardiomyopathy). A high pre-load may overstretch the myocardial wall and compromise contractility. If the cardiac output is compromised then decrease the filling pressures by removing circulating volume (fluid restriction, diuretic therapy or peritoneal dialysis). пЃў1 effects increase stroke volume and heart rate and thus myocardial oxygen consumption. пЃў2 receptors reduce peripheral vascular tone. Alpha receptors increase vascular tone. The cardiovascular response to exogenous catecholamines is influenced by: п‚· п‚· п‚· п‚· Dose - alpha effects predominate at higher concentrations. Age – receptors are poorly developed and insensitive to inotropes in neonates and infants e.g. response to dopamine and dobutamine is diminished Treatment with пЃў-blockers increases the number of myocardial пЃў-receptors and sensitivity to beta-adrenergic stimulation is increased for 36-48 hours following discontinuation of пЃў-blockade. Decreased receptor sensitivity - catecholamines are less effective in patients with chronic heart failure. The dose, selection and combination of catecholamines are based on the underlying haemodynamic problem. The aim is to optimise (not necessarily normalise) myocardial contractility, heart rate and pulmonary and systemic vascular resistance to 62 achieve an adequate cardiac output. Hypovolaemia, tamponade and residual defects have to be excluded especially in patients requiring excessive inotropic support. On occasions the cardiac output remains unsatisfactory despite high doses of standard inotropic agents. This may be due to decreased sensitivity of пЃў receptors or to the deleterious effects of inotropes (vasoconstriction and tachycardia). Catecholamines table Drug Dopamine Dobutamine Isoprenaline Epinephrine Norepinephrine пЃў1 ++ +++ ++++ ++ ++ пЃў2 + ++++ ++ пЃЎ ++ + Dopaminergic +++ +++ ++++ Phosphodiesterase inhibitors: Milrinone amrinone, and enoximone are phosphodiesterase inhibitors that increase intracellular cyclic AMP. They increase peripheral vasodilation and improve ventricular relaxation allowing increased ventricular filling. Milrinone is the agent most commonly used in paediatric practice. It is given via continuous IV infusion – refer to BNFc for prescription advice. Catecholamines, PDE inhibitors and cardiac glycosides improve myocardial contractility at different levels of intracellular calcium transport. Their receptor actions are however not specific and they thus have multiple effects. After-load may become a critical factor determining myocardial function because the autonomic response to a poor cardiac output is an increase in systemic vascular resistance, and hence after-load, further compounding the problem. Reduction in after-load thus reduces myocardial work and oxygen requirement and improves cardiac output. Suitable drugs include glyceryl trinitrate, sodium nitroprusside, prostacyclin, phenoxybenzamine and ACE inhibitors. Too slow a rate will reduce the cardiac output. The rate can be increased with the use of isoprenaline or pacing. Ideally if pacing is used, sequential A-V pacing (atria then ventricles) should be selected. Sometimes the cardiac rate may be too high (> 200 bpm) to allow adequate ventricular filling. Digoxin or пЃў-blockers may be used to reduce the rate by slowing AV node conduction. 6.4 Cardiac Tamponade - See section 3.7.2 6.5 Cardiomyopathy In most children with dilated cardiomyopathy an underlying cause is not identified. However there are several rare metabolic disorders that may be associated with cardiomyopathy and, whilst certain conditions may be suggested by the clinical presentation, not uncommonly metabolic conditions may have no extra-cardiac 63 features at all. It is therefore very important that a carefully structured investigation protocol is followed to prevent these being overlooked. Aetiology п‚· Infective п‚· Metabolic & cellular п‚· Immunological п‚· Toxins (e.g. anthracylines, alcohol) п‚· Left heart obstruction (e.g. AS, CoA) п‚· Hypertension п‚· Coronary abnormalities (anomalous LCA) п‚· Chronic left-to-right Shunt п‚· Intractable arrhythmia п‚· Familial (~20%) п‚· Peripartum History п‚· Parental consanguinity п‚· Antenatal history - HELLP Syndrome, Fatty Liver of Pregnancy or Severe PET, consider fatty acid oxidation defect п‚· Neonatal death or SIDS in a sibling п‚· Symptoms of preceding viral infections п‚· Liver disease or multiple transfusions п‚· Myopathy, ataxia or autoimmune disease п‚· Abnormal nutrition history (e.g. long term parenteral nutrition) п‚· Delayed development п‚· Past medical history, malignancy, chemotherapy (cumulative dose of anthracyclines) п‚· Myocardial ischaemia may have an unusual presentation in children who are unable to localise pain Examination п‚· Dysmorphic features п‚· Myopathy п‚· Hepatosplenomegaly (storage disorder) п‚· Hepato-renal dysfunction п‚· Blood pressure - hypertensive cardiomyopathy may present with normal blood pressure if cardiac function is poor п‚· A detailed general examination and developmental assessment Cardiomyopathy - Investigations ECG - 12-lead and 24 hour tape required. Look for evidence of ischaemia, tachycardia, low voltage complexes and repolarisation abnormalities. Holter – rule out sustained heart rhythm problems Chest X-ray - Assess cardiomegaly and pulmonary vascularity Echocardiography - A full cross-sectional echocardiogram and Doppler study should be performed with particular attention paid to coronary artery morphology, systolic 64 adherent to LV wall). Laboratory investigations: Biochemical - Urea, electrolytes, creatinine, calcium, phosphorous and liver function tests, CRP, acid-base balance, anion gap, lactate, cholesterol, uric acid and glucose, serum ammonia, total and free carnitine, acyl carnitine profile, plasma amino acids, creatinine kinase, troponin, TFT, autoantibodies. Haematology - FBC, ESR, coagulation screen, ferritin and blood group, blood film to look for vacuolated lymphocytes. Virology - Viral titres (paired sera) for enteroviruses including Coxsackie together with standard virology assay, consider need for HIV test. Urine specimens - Organic acids and amino acids, ketones (dipstick), glucose and protein, mucopolysaccharide screen, VMA (special bottle may be required – liaise with Biochem). Further specific investigations may be required for certain conditions like mitochondrial disease. Certain children with isolated hypertrophic cardiomyopathy and suspected respiratory chain disorders may require skeletal muscle biopsy, endomyocardial biopsy, DNA studies and mitochondrial respiratory chain studies. Approximately 13mls of blood are needed (it includes ESR-4mls in EDTA) , one Guthrie card, capillary gas and BM capillary for Blood sugar. Requests : 1 Haematology form FBC plus blood film (d/w lab – looking for vacuolated wbc) – 1 mL EDTA ESR – 4 mL EDTA Clotting – 1mL paeds citrate sample nd 2 Haematology form – Ferritin 1 mL plain top 1 Immunology form – Autoantibodies 1 mL in plain top 1 Virology form – Standard virology assay – 1 mL in plain top Ring lab before sending coxsackie, enteroviruses (send with stool sample) Biochemistry 4 lithium heparin samples 2 Lithium heparin samples for standard biochemistry (see list below) Ammonia assay 0.5 ml Lithium heparin sample in ICE – send within 10 minutes 0.5mL Litimum heparin for Plasma amino acids Request nursing staff to send Urine tests (Don’t forget to give request forms) Further specific investigations may be required for certain conditions like mitochondrial disease. Certain children with isolated hypertrophic cardiomyopathy and suspected respiratory chain disorders may require skeletal muscle biopsy, endomyocardial biopsy, DNA studies and mitochondrial respiratory chain studies. Consider need for HIV test. 65 Cardiomyopathy Screen - Investigations Name: Unit Number: DOB: Biochemistry Lab: Test (units) Urea Creatinine Sodium Potassium Bicarbonate Chloride Total Protein Albumin ALP AST Bilirubin Calcium Phosphorus CRP CK Cholesterol Triglyceride Uric acid Troponin Ammonia Date Result (mmol/L) (mmol/L) (mmol/L) (mmol/L) (mmol/L) (mmol/L) (g/L) (g/L) (IU/L) (IU/L) (пЃmol/L) (mmol/L) (mmol/L) (mg/L) (IU/L) (mmol/L) (пЃg/L) (пЃmol/L) Carnitine Total and Free (пЃmol/L) (пЃmol/L) (пЃmol/L) Acyl Carnitine Profile Comments Lithium Heparin (LH) LH LH LH LH (to calculate anion gap) LH (to calculate anion gap) LH LH LH LH LH LH LH LH LH LH LH LH LH LH 0.5 ml (ice sample –send within 10 min) LH or white Guthrie card LH or white Guthrie card LH (0.5 ml) Capillary Plasma Amino acids Blood gas Anion gap (пЃmol/L) Glucose (mmol/L) BM Capillary or oxalate (yellow top) 0.5 mL (mmol/L) BM capillary or oxalate LH Lactate Thyroid Function tests Calculate make sure requesting HCO 3 and Chloride in Biochemistry sample TFT Free T4 Free T3 66 Immunology Auto antibodies Plain top 1 ml Haematology Lab WCC Hb Platelet Blood Film ESR APTT PT Fibrinogen Ferritin EDTA EDTA EDTA EDTA EDTA 4mls Citrate (Green top) Citrate (Green top) Citrate (Green top) Plain top 1 ml to Haematology Urine tests Urine organic acids Urine amino acids Urine Glucose Urine Protein Urine Ketones Urine-Mucopolysaccharide screen Urine-VMA Other Standard Virology assay Coxsackie *Viral titres and Enteroviruses** Plain top 1 ml *Plain top and discuss with virology for coxsackie ** for enterovirus send with stool sample ECG Extn 3325 Chest Radiograph Extn 3027 ECHO Viv – 3920 Print out table and put at front of patient record for ready reference Natural History 5 year transplant-free survival following diagnosis of “cardiomyopathy” (%) All causes Myocarditis Idiopathic DCM Unclassified Neuromuscular Metabolic 56 78 52 70 26 33 Reference: JACC 2010;55:1377-84 – results of 189 patients, excludes pts exposed to anthracycline (small numbers) Worse outcomes are seen: п‚· In patients presenting >2 years of age п‚· When presenting ejection fraction is <20% (FS ~<10%) п‚· In enterovirus myocarditis п‚· There is intracardiac thrombus at presentation 67 п‚· If severe LV dysfunction persists >3 months. More favourable outcomes tend to be seen: п‚· In patients <2 years at presentation п‚· In those presenting with acute myocarditis п‚· In patients where a treatable cause of DCM is seen, e.g. those presenting with a tachycardia-related cardiomyopathy where the rhythm can be controlled. If LV dysfunction persists, there will eventually be a progressive deterioration with worsening cardiac failure, arrhythmias and systemic and/or pulmonary emboli. Management п‚· Diuretics п‚· ?Digoxin п‚· ACE inhibitors п‚· пЃў-blockade – once features of acute heart failure have been stabilised (see section 3.2.3) п‚· Anti-platelet or anticoagulation therapy п‚· Cardiac transplantation Screening Both dilated and hypertrophic forms of cardiomyopathy may be familial. Undertaking screening of family members may have serious consequences for adults e.g. refusal of life assurance. Referral to Medical Genetics is recommended. See Section 6.22. 6.6 Chest Pain in Children Chest pain in children is usually benign and rarely due to a cardiac cause. The precordial catch (benign chest pain of unknown cause) is the commonest cause experienced by most of us at one time or another. It may occur at rest or during exercise, is located usually at the apex but may be found at the sternal edges. It is usually described as a sharp stabbing pain of short duration. It is particularly common in adolescents. Its frequency varies from once every now and again to several times a day. If very frequent it can be disabling. There are no other abnormal symptoms or signs of cardiac disease. It improves with sympathy, time and reassurance. Pointers to pathological causes are: п‚· Symptoms suggestive of angina п‚· Clear relationship with exertion п‚· Associated palpitation or pre-syncope п‚· Known underlying cardiac disease Musculoskeletal chest pain п‚· Sharp pain related movement, deep inspiration or previous trauma п‚· Usually well localized п‚· May be reproduced by pressure on the affected area or “springing” the ribs п‚· Usually acute and subsides over days. п‚· Tietze’s syndrome o Costo-chondritis usually of the 2nd to 5th costo-chondral junctions o Swelling of the junctions may occur 68 o Spontaneous resolution is invariable. Cardiac causes п‚· Arrhythmia: In SVT or VT the high cardiac oxygen requirement may outstrip the supply. The usual presenting complaint is however a noticeable fast heart rate with the chest pain a secondary phenomenon. п‚· Aortic valve disease: If severe this may present with chest pain on exercise. A 12 lead ECG even at rest is likely to show some ST-T segment changes which become marked on exercise. An echocardiogram is also likely to show LVH, a LVOT velocity greater than 4 m/s and/or severe aortic regurgitation. п‚· Pericarditis: This may presents in the context of a systemic illness with chest pain alleviated on sitting up; it also may occur post-operatively. An ECG may show ST elevation and echocardiogram an effusion. п‚· Hypertrophic cardiomyopathy: May rarely present with chest pain especially in childhood. If it occurs it is usually central chest pain on exercise. п‚· Kawasaki disease: May present with myocardial ischaemia if the coronary arteries are affected. This is very unusual in the acute phase tending to occur in those with coronary artery sequelae. Other vasculitides (e.g. Takayasu) may present in a similar fashion. п‚· Congenital abnormalities of the coronary arteries: These are rare, but may present with chest pain. In the infant this may be pallor and crying on feeding whilst exercise induced pain is more common in the older child. The ECG or exercise ECG will be abnormal and the echocardiogram should confirm the diagnosis. An MRI or CT angiogram may also be necessary. п‚· Pulmonary hypertension: Patients with Eisenmenger’s syndrome may develop chest pain with exercise. This may be due to RV ischaemia. They may also experience chest pain during pulmonary hypertensive crises. Vasodilators e.g. nitrates, but may exacerbate cyanosis by reducing SVR. Venesection can be considered if polycythaemia is severe. п‚· Aortic dissection: Always consider this in patients with aortic enlargement, e.g. patient with Marfan syndrome. The classical signs of the syndrome will be present. The chest pain is often in the back and described as tearing. Suspected cases should have an urgent CXR, TOE or CT/MRI scan and, if confirmed, should proceed to surgery immediately. п‚· Rheumatic fever may cause pericardial pain secondary to inflammation. Other arthritides (e.g. JRA) May cause pericarditis. п‚· Drug abuse e.g. cocaine, glue sniffing may cause coronary arterial spasm. п‚· Mitral valve prolapse – can be associated with non-specific chest pain (cause unknown). Pulmonary causes Classical pleuritic pain is usually easy to distinguish from cardiac pain due to its location and variation with respiration. Signs of respiratory disease are often also present (dyspnoea, tachypnoea, haemoptysis, fever and abnormal breath sounds). Pericardial pain may occasionally be difficult to distinguish. GI causes Oesophagitis due to reflux may occur but it is uncommon except in those with abnormal GI tracts. Peptic ulcers are probably under-diagnosed in childhood and may 69 present with epigastric or chest pain. Vomiting and other symptoms of reflux, haematemesis or melaena may point to the diagnosis. Psychosomatic chest pain This may be a learned phenomenon as chest pain in later adult life is often of a serious nature. Adult role models may thus initiate the symptoms that are then used as an excuse to avoid unwelcome activities. A careful history and examination with judicious investigation should allow appropriate management. Investigation and treatment Appropriate diagnosis of the cause of the chest pain and reassurance are usually all that is required. An ECG often helps reassure and exercise test may be helpful if the chest pain is associated with exercise or in the context of structural cardiac disease. 6.7 Endocarditis and Endocarditis Prophylaxis 6.7.1 Infective Endocarditis Aetiology п‚· Risk factors: endothelial damage + bacteraemia п‚· Increased risk with o All CHD except secundum ASD; includes most repaired defects o Localised infection (e.g. osteomyelitis, skin infection) o Poor dentition or following dental/surgical procedures o Prosthetic heart valves o Indwelling vascular lines o Immunodeficiency, including post-transplant п‚· 90% due to Strep viridans, enterococci or Staphyloccoal species (aureus or coagulase-negative) Features п‚· Insidious onset (usually): fatigue, fever, anorexia, pallor п‚· Murmur (especially changing), fever, splenomegaly, clubbing п‚· Petechiae, nail splinters, Osler’s nodes, Janeway lesions, embolic phenomena (PE, cerebral, haematuria, Roth spots) Investigations п‚· If the patient is not acutely unwell, at least three sets of blood cultures should be undertaken over the space of 12-24 hours; cultures during fever or rigor are more likely to yield positive results, but they can be taken even if the patient is afebrile п‚· If the patient is acutely unwell take at least two sets of cultures before starting immediate treatment п‚· Other investigations include o FBC, ESR, Coagulation profile o U+E/creatinine/LFT/CRP o Urinalysis o Serial echocardiography Treatment 70 The mainstay of treatment of endocarditis is antibiotic therapy. Patients with a subacute history should be treated with a combination of penicillin (4-6 weeks) and gentamicin (2 weeks). Treatment may be tailored according to the results of microbiological investigations. Patients with an acute history should additionally receive flucloxacillin to cover the possibility of staphylococcal infection. If a myocardial or paravalvar abscess is shown on echocardiography, or if a vegetation is large with a high risk of embolising, early surgical discussion is required. In staphylococcal endocarditis where vegetations are associated with prosthetic material within the heart, used of vancomycin/gentamicin may be advisable (refer to European Society of Cardiology [ESC] guidelines). Other management points Close consultation with the microbiologists is required. Progress of the disease is monitored by serial FBC, ESR, CRP and echocardiography. Echocardiography may demonstrate vegetations but a negative scan does not exclude the diagnosis of endocarditis. TOE is a more sensitive means of demonstrating vegetations. The scan should also establish a baseline of valvular and myocardial function – both of which may change during the course of the illness. A dental consultation should be undertaken – it may reveal the source of infection and reduce the risk of recurrence. Antibiotics should not be given until instructed by the consultant. 6.7.2 Endocarditis prophylaxis Most cases of endocarditis do not have an identifiable precipitant, but some cases can be traced to a dental or surgical episode. In 2008 NICE published guidelines covering the prevention of IE. It was recognised that patients with CHD, prosthetic valves and post-transplant are at increased risk of IE, but the committee was not persuaded that prophylactic antibiotic cover offered protection against infection. The NICE guideline calls for: п‚· Meticulous dental hygiene п‚· Regular dental visits п‚· The avoidance of body piercing and tattooing п‚· Prompt treatment with antibiotics of bacterial infections (e.g. abscesses or boils) п‚· Cessation of the practice of giving routine prophylactic antibiotics prior to certain surgical and dental procedures. The guideline hints that cover should be given for the first 6 months post intracardiac or intravascular device implant (but does not specify what type of cover). GI operations where there is a bowel abscess should be covered with antibiotics. Reference: http://guidance.nice.org.uk/CG64 The BCCA recommended acceptance and implementation of the NICE guideline, but did emphasise the fact that each clinician should give the advice that they think should be followed in the best interest of the patient. If a clinician feels that in individual cases the NICE guideline should not be applied, current AHA or ESC guidelines should be followed (the AHA wallet card is on the Shared Directory under “Info sheets”). 71 6.8 Exercise in Paediatric Cardiac Patients Exertional syncope, arrhythmias and sudden death are associated with certain types of congenital and acquired heart disease. In a study of 387 deaths during exercise the most common cardiac causes were: Cause of death on PM No % Hypertrophic cardiomyopathy 102 26.4 Commotio cordis 77 19.9 Coronary artery anomalies 53 13.7 Left ventricular hypertrophy of indeterminate causation 29 7.5 Myocarditis 20 5.2 Ruptured aortic aneurysm (Marfan syndrome) 12 3.1 Arrhythmogenic right ventricular cardiomyopathy 11 2.8 Tunneled (bridged) coronary artery 11 2.8 Aortic valve stenosis 10 2.6 Atherosclerotic coronary artery disease 10 2.6 Dilated cardiomyopathy 9 2.3 Myxomatous mitral valve degeneration 9 2.3 Other cardiovascular cause 4 1.0 Long QT syndrome 3 0.8 In many paediatric cardiac conditions, exercise is unrestricted, however in a number of conditions, intense exertion and competitive sports represent a risk to the health of the patient and should be restricted. The following meant to be a simple, practical guide with which clinicians can advise patients and their parents. General Principles Although hypertrophic cardiomyopathy (HCM) is the commonest cause of sudden death in young athletes, most deaths in HCM occur with a history of minimal or no exertion. In contrast, sudden death in aortic stenosis is almost exclusively associated with exertion. Most available data and therefore the clearest recommendations relate to athletes undertaking competitive sports rather than the type of activities that most children engage in. It is important, therefore, to have a sense of perspective in giving advice about sports participation. Incorrect or inappropriate advice, however, may lead to the premature death of a young person. 72 In general the following principles should apply: п‚· Patients with known HCM, AS, DCM, congenital coronary artery anomalies, enlarged aorta and LQTS should avoid high intensity/explosive sports. п‚· Patients with known HCM, AS, DCM, congenital coronary artery anomalies, LQTS and WPW should avoid unaccompanied crosscountry running and swimming. п‚· Patients with LQTS, particularly LQTS type 1, should avoid diving into cold water. п‚· Patients with a significantly enlarged aorta (e.g. Marfan syndrome, bicuspid aortic valve spectrum, some post-op CoA, arterial switch, tetralogy and Ross patients) should not engage in high-impact contact sports or those that involve intense straining, such as rugby scrummaging, weight-lifting and rowing. п‚· Patients on anticoagulants or anti-platelet agents should avoid highimpact contact sports. п‚· Patients with exercise-induced SVT should avoid the activities that precipitate symptoms, or should take prophylactic anti-arrhythmics prior to sports participation. п‚· Patients with VT should avoid sports. п‚· In many cases, with appropriate advice, the school PE Department may be able to modify activities to permit at least some sports participation for the vast majority of patients. п‚· Patients with a pacemaker or ICD device should not engage in contact sports (risk of damage to device or electrodes). Refer to lesion-specific recommendations related to the underlying pathology for more detailed advice. 73 Classification of Competitive Exercise Static Component A. Low B. Moderate C. High III. High Bobsledding/Luge*С„; Track & Field (throwing events Gymnastics*С„ Martial arts* Sailing Rock climbing Water skiing*С„ Weight lifting*С„ Windsurfing*С„ Archery Auto racing*С„ Diving (pool) *С„ Equestrianism*С„ Motorcycling*С„ Body building*С„ Downhill skiing*С„ Skateboarding*С„ Snowboarding*С„ Wrestling (GrecoRoman) Boxing* Canoeing Kayaking Cycling*С„ Decathlon Rowing Speed-skating*С„ Triathlon*С„ American football* Track & Field (jumping events) Figure skating* Rugby* Running (sprint) Surfing*С„ Synchronised swimmingС„ Baseball/softball* Fencing Table tennis Volleyball Basketball* Ice hockey* Cross-country skiing (skating technique) Lacrosse* Running (middle distance) Swimming II. Moderate Billiards, snooker, pool Badminton Bowls Cross-country skiing 10-pin bowling (classical technique) Curling Field hockey* Golf Orienteering Riflery Race walking Cricket (NB minimum Squash of helmet and body Running (long armour needed for distance) patients on Soccer* anticoagulants) Tennis Increasing Dynamic Componentв†’ * = Danger of bodily collision – avoid if on anticoagulants Р¤ = Risk is greater if likelihood or history of syncope th Based on 36 Bethesda Conference – Recommendations for competitive athletes with CVS abnormalities JACC 2005;1318 onwards I. Low Exercise advice by lesion NB – These recommendations are for competitive sports during participation. Higher levels of both static and dynamic components may be attained during training. The guidelines may or may not apply to non-competitive sports participation and this is where the discretion of the clinician is needed. Some of the recommendations have been adapted for the UK or paediatric context. 74 6.8.1 Myocardial Abnormalities Acute Myocarditis Athletes with probable or definite evidence of myocarditis should be withdrawn from all competitive sports and undergo a prudent convalescent period of about six months following the onset of clinical manifestations. Athletes may return to training and competition after this period of time if: (i) LV function, wall motion, and cardiac dimensions return to normal (based on echocardiographic and/or radionuclide studies at rest and with exercise). (ii) Clinically relevant arrhythmias such as frequent and/or complex repetitive forms of ventricular or supraventricular ectopic activity are absent on ambulatory Holter monitoring and graded exercise testing. (iii) Serum markers of inflammation and heart failure have normalized. (iv) The 12-lead ECG has normalised. Persistence of relatively minor ECG alterations such as some ST-T changes are not the basis for restriction from competition. Hypertrophic Cardiomyopathy (HCM) All patients with HCM undergo regular clinical and risk factor assessment (clinical evaluation, ECG, 48-hour Holter, exercise test, echo). (i) Patients with a pre-clinical genetic diagnosis and no abnormalities on the above tests do not need exercise limitation, but should be reassessed every 12-18 months (ii) Athletes with a probable or unequivocal clinical diagnosis of HCM should be excluded from most competitive sports, with the possible exception of those of low intensity (class IA). This recommendation is independent of age, gender, and phenotypic appearance, and does not differ for those athletes with or without symptoms, LV outflow obstruction, or prior treatment with drugs or major interventions with surgery, alcohol septal ablation, pacemaker, or implantable defibrillator. 6.8.2 Coronary Abnormalities Congenital coronary anomalies of wrong sinus origin are the second most common cardiovascular cause of sudden death in young athletes. Identification of these anomalies during life can be difficult because patients often do not experience warning symptoms, and rest and exercise ECGs are usually normal. Coronary anomalies should be considered in athletes with exertional syncope or symptomatic ventricular arrhythmia and should be investigated using appropriate studies such as echocardiography, cardiac MRI, or CT angiogram. Coronary arteriography is indicated if other studies are not diagnostic. Surgery is usually performed when the diagnosis is made. Recommendations: (i) Detection of coronary anomalies of wrong sinus origin in which a coronary artery passes between great arteries should result in exclusion from all participation in competitive sports. (ii) Participation in all sports three months after successful operation would be permitted for an athlete without ischemia, ventricular or tachyarrhythmia, or dysfunction during maximal exercise testing. 75 In patients with a history of Kawasaki Disease: (i) Patients with no coronary artery abnormalities or transient coronary artery ectasia resolving during the convalescent phase of the disease are permitted to participate in all sports after six to eight weeks. (ii) Patients with regressed aneurysms can participate in all competitive sports if they have no evidence of exercise-induced ischemia by stress testing with myocardial perfusion imaging. (iii) Patients with isolated small- to medium-sized aneurysms in one or more coronary arteries and judged to be at low risk for ischemic complications (normal left ventricular function, absence of exercise-induced ischemia or arrhythmia) may participate in low to moderate static and dynamic competitive sports (classes IA, IB, IIA, and IIB). Stress testing with evaluation of myocardial perfusion should be repeated at one- to two-year intervals to monitor ischemia and guide further recommendations about sports competition. (iv) Patients with one or more large coronary aneurysms or multiple (segmented) or complex aneurysms with or without obstruction to coronary flow may participate in class IA and IIA sports if they have no evidence of reversible ischemia on stress testing, normal LV function, and absence of exercise-induced arrhythmia. Stress testing with evaluation of myocardial perfusion should be repeated at one-year intervals to monitor ischemia and guide further recommendations about sports competition. (v) Athletes with recent MI or revascularization should avoid competitive sports until their recovery is complete—usually six to eight weeks. Those with normal LV ejection fraction, exercise tolerance, absence of reversible ischemia or myocardial perfusion testing, and absence of exercise-induced arrhythmias can participate in class IA and IB sports. Those with left ventricular ejection fraction less than 40%, exercise intolerance, or exercise-induced ventricular tachyarrhythmias should not participate in competitive sports. (vi) Patients with coronary lesions who are taking anticoagulants and/or antiplatelet drugs (aspirin, clopidogrel) should not participate in sports that pose a danger of high speed collision. 6.8.3 Congenital Lesions and Conditions Aortic regurgitation Evaluation should include clinical examination, ECG, Holter, echo, metabolic exercise test Definitions: Severity of AR Gauged by… Mild Absent or slight signs of AR; normal LV size (<60 mm in adults, taking into account athletic training Moderate Peripheral signs of AR with mild-moderate increase in LV size Severe Peripheral signs of AR with severe LV enlargement В± LV dysfunction 76 Recommendations: (i) Mild AR, aorta <97th percentile in diameter: no restrictions. Moderate AR with normal LV systolic function: class I and II sports (ii) permitted. NB Only Class IA sports if aorta ≥97th percentile. (iii) Severe AR or any degree of AR with symptoms, no competitive sports are permitted. Aortic stenosis and bicuspid aortic valve Grading severity of AS Severity of AS Catheter gradient Mean echo (peak-to-peak, Doppler gradient non-anaesthetised) (mmHg) (mmHg) Mild <30 <25 Moderate 30-50 25-40 Severe >50 >40 Peak echo Doppler gradient (peak CW) (mmHg) <40 40-70 >70 Mild AS – If ECG is normal and there are no symptoms, activities are unrestricted (NB exercise test recommended). (ii) Moderate AS – If there is no or mild LVH on echo, absence of LV strain on ECG, and a normal exercise test PLUS no symptoms, then exercise classes IA, IB and IIA are permitted. (iii) Severe or symptomatic AS – No competitive sports are permitted. (iv) Bicuspid aortic valve with no AS or AR and aorta not enlarged or only mildly enlarged (<40 mm in adult or equivalent percentile in children): no restrictions. (v) BAV with moderate aortic enlargement (40-45 mm in adults, equivalent percentile in children): Non-contact IA, IB, IIA, IIB permitted. (vi) After intervention (balloon or surgical valvotomy) with mild or better residual AS/AR – see recommendations above. If AR > mild, Class I and II sports are permitted (non-contact if aorta significantly enlarged). (vii) Patients with prosthetic valves, on anticoagulants and normal ventricular function can participate in non-contact IA, IB and IIA sports. (viii) Patients after Ross operation: if there are no symptoms, the exercise test is satisfactory, ventricular function is normal or only mildly impaired, AR is no worse than mild and RVOTO gradient is <40 and aortic size is <97th percentile, activities are unrestricted. If any of the above apply, see individual lesions. (i) Coarctation of the aorta (i) Athletes with mild CoA (systolic gradient <20 mmHg on 4-limb BP, absence of major collaterals, absence of significant aortic root enlargement [<97th percentile for body surface area] and peak systolic BP on exercise of ≤230 mmHg): activities are unrestricted. (ii) >Mild CoA (worse than above), class IA permitted (iii) Post repair (surgery, balloon or stent): ECG, echo, CXR, MRI or CTa, 4limb BP and exercise test needed to ascertain residual problems. If gradient < 20 mmHg (4-limb), aorta <97th percentile and BP is normal at 77 rest and on exercise, then ≥3 mo post-repair, all sports are permitted except IIIA, IIIB or IIIC. If aorta ≥97th percentile, or an aortic aneurysm is present, then only classes IA and IB (but non-contact) are permitted. Congenitally corrected transposition of the great arteries (double discordant TGA) A metabolic exercise test should be undertaken. Provided VO 2 max and Holter recordings are normal and ventricular dysfunction is no worse than mild, class IA and IB competitive sports are permitted. Class III sports are not recommended. Cyanotic CHD Exercise class IA is permitted (to competitive level) if saturations remain over 80% and ventricular function is good. Non-competitive exercise should be limited by symptoms. Ebstein’s malformation (i) Sports participation is unlimited if the valve lesion is mild, there are no significant arrhythmias, the patient is not cyanosed and RV size is normal or near-normal. If TR is moderate and there are no arrhythmias, class IA sports are (ii) permitted. (iii) Severe Ebstein’s: no competitive sports are permitted (iv) Post repair: if TR is no worse than mild, arrhythmias are absent on Holter and exercise testing, and heart size is not significantly increased, competitive IA sports are permitted. Lв†’R shunt lesions (ASD, VSD, PDA, AVSD, A-P window) Uncomplicated lesion (small shunt, no PHT) – No restrictions. (i) (ii) Lesion complicated by large shunt or mild pulmonary hypertension – closure of lesion is recommended; In ASD class IA competitive sports are allowed pre-repair. If lesion is complicated by arrhythmia – see Rhythm section. (iii) Lesion complicated by Eisenmenger syndrome – no competitive sports allowed. (iv) Lesion post repair: 3-6 months after repair, if ECG, CXR and echo show no residual haemodynamic problem and the patient is not on anti-platelet or anticoagulants, no restrictions. Marfan syndrome Athletes with Marfan syndrome can participate in low and moderate static/low dynamic competitive sports (classes IA and IIA) if they do not have one or more of the following: (i) Aortic root dilatation (i.e., transverse dimension 40 mm or greater in adults, or more than 2 standard deviations from the mean for body surface area in children and adolescents; z-score of 2 or more). (ii) Moderate-to-severe mitral regurgitation. (iii) Family history of dissection or sudden death in a Marfan relative. It is recommended, however, that these athletes have an echocardiographic measurement of aortic root dimension repeated every six months, for close surveillance of aortic enlargement. 78 Athletes with unequivocal aortic root dilatation (transverse dimension 40 mm or greater in adults or greater than 95th percentile for body surface area in children and adolescents), prior surgical aortic root reconstruction, chronic dissection of aorta or other artery, moderate-to-severe mitral regurgitation, or family history of dissection or sudden death can participate only in low-intensity competitive sports (class IA). Athletes with Marfan syndrome, familial aortic aneurysm or dissection, or congenital bicuspid aortic valve with any degree of ascending aortic enlargement (as defined in 1 and 2 above) also should not participate in sports that involve the potential for bodily collision. Pulmonary valve disease (i) Mild PS (gradient < 40 mmHg) with no symptoms – no restrictions (ii) >Mild PS – recommend balloon intervention. (iii) Post-intervention with gradient <40 mmHg and no symptoms, no restrictions, sports can resume 2-4 weeks post intervention. (iv) Severe pulmonary regurgitation with dilated RV, class IA and IB sports are permitted. (v) Recommendations related to aortic regurgitation – see above. (vi) These recommendations are offered independent of whether beta-blockers are administered to mitigate aortic root enlargement. Mitral regurgitation (i) Athletes with mild to moderate MR who are in sinus rhythm with normal LV size and function and with normal pulmonary artery pressures can participate in all competitive sports. (ii) Athletes with mild to moderate MR in sinus rhythm with normal LV systolic function at rest and mild LV enlargement (compatible with that which may result solely from athletic training [less than 60 mm in adults) can participate in some low and moderate static and low, moderate, and high dynamic competitive sports (classes IA, IB, 1C, IIA, IIB, and IIC). (iii) Athletes with severe MR and definite LV enlargement (greater than or equal to 60 mm), pulmonary hypertension, or any degree of LV systolic dysfunction at rest should not participate in any competitive sports. (iv) Patients in atrial fibrillation or a history of atrial fibrillation who are receiving long-term anticoagulation should not engage in sports involving any risk for bodily contact or danger of trauma. Pulmonary hypertension If PA pressure <30 mmHg, no restrictions apply. If PA pressure >30 mmHg, level of participation is at the discretion of the clinician. 6.8.4 Recommendations in Post-Operative Patients Post-operative ventricular dysfunction (i) Normal or borderline low ventricular function (EF≥50%): full participation permitted. (ii) Mild ventricular dysfunction (EF 40-50%): classes IA, IB and IC are permitted. 79 (iii) Moderate or worse ventricular dysfunction (EF <40%): All competitive sports are forbidden. Post-operative Tetralogy of Fallot Evaluation should include physical examination, ECG, CXR, echo, Holter, MRI and exercise testing. Cardiac catheterisation may also be required. (i) Exercise is unrestricted if: RV pressure is normal or near-normal, there is no or minimal RV volume load, there is no significant residual shunt, there is no evidence of arrhythmia. (ii) If there is severe PR with RV volume load, RV hypertension to >50% systemic levels, evidence of arrhythmia, class IA competitive sports participation is permitted. Post-operative arterial switch operation Limited data are available for this situation. The discretion of the clinician is needed. Provided an exercise test is normal: (i) Exercise is unrestricted if there are no significant haemodynamic problems and ventricular function is normal. (ii) If haemodynamic or anatomical problems are more than mild (e.g. moderate branch PS, moderate supra-valvar AS, moderate ventricular dysfunction, or worse), then competitive activities in classes IA, IB, IC and IIA are permitted. (iii) If the aorta is enlarged ≥97th percentile, sports with danger of high impact bodily collision should be avoided. Post-operative Fontan operation Evaluation should include clinical examination, ECG, echo, Holter, MRI and metabolic exercise test with saturation monitoring. (i) If the above are satisfactory and the patient is asymptomatic, IA sports are permitted (body armour for cricket if patient is anticoagulated). (ii) IB sports are permitted if ventricular function and O 2 saturations are normal. Prosthetic valve replacement (i) (ii) (iii) Athletes with a bioprosthetic mitral valve not taking anticoagulant agents and who have normal valvular function and normal or nearnormal LV function can participate in low and moderate static and low and moderate dynamic competitive sports (classes IA, IB, IIA, and IIB). Athletes with a mechanical or bioprosthetic aortic valve, with normal valve function and with normal LV function, can engage in low and moderate static and low and moderate dynamic competitive sports (classes IA, IB, and IIA). Athletes participating in greater than lowintensity competitive sports (class IA) should undergo exercise testing to at least the level of activity achieved in competition to evaluate exercise tolerance and symptomatic and hemodynamic responses. Independent of other considerations, athletes with a mechanical or bioprosthetic mitral valve or aortic valve who are taking anticoagulant 80 agents should not engage in sports involving the risk of bodily contact or the danger of trauma. 6.8.5 Recommendations for Athletes with Arrhythmias SVT (not WPW) (i) Athletes without structural heart disease who have reproducible exerciseinduced SVT prevented by therapy, who are asymptomatic on medication and have a normal exercise test can participate in all competitive sports. (ii) Athletes who do not have exercise-induced supraventricular tachycardia but experience sporadic recurrences should be treated. However, because of the unpredictable nature of the tachycardia, end points for adequate therapy may be difficult to achieve; but once established, these athletes can participate in all activities consistent with their cardiac status. Asymptomatic athletes who have episodes of supraventricular tachycardia of 5 to 15 s that do not increase in duration during exercise can participate in all sports consistent with their cardiac status. (iii) Athletes with syncope, near-syncope, or significant symptoms secondary to arrhythmia or who have significant structural heart disease in addition to the arrhythmia should not participate in any competitive sports until they have been adequately treated and have no recurrence for two to four weeks. At that time they can participate in class IA competitive sports. (iv) For those athletes with no structural heart disease who have had successful catheter or surgical ablation, are asymptomatic, and have no inducible arrhythmia on follow-up electrophysiological testing, all competitive sports are permitted within several days of the confirmatory EP testing. If no EP testing is done but there are no symptoms and the ECG is normal, full participation is permitted if no spontaneous recurrence of tachycardia for two to four weeks after ablation. SVT due to WPW (i) Athletes with pre-excitation on ECG without structural heart disease, without a history of palpitations, or without tachycardia (particularly those 20 to 25 years old or more) can participate in all competitive sports. However, in younger age groups, a more in-depth evaluation including an EP study may be recommended before allowing participation in moderateto high-intensity competitive sports. (ii) Athletes with episodes of AV reciprocating tachycardia should be treated as previously recommended (see section above). However, it should be appreciated that they can develop atrial fibrillation with rapid ventricular rates. Electrical induction of atrial fibrillation to determine the shortest QRS interval between two complexes conducted over the accessory pathway during isoprenaline administration or exercise is recommended. Those athletes in whom the shortest cycle length is less than 250 ms should undergo ablation of the accessory pathway. (iii) Athletes with episodes of atrial flutter/fibrillation and syncope or near syncope whose maximal ventricular rate at rest (without therapy) as a result of conduction over the accessory pathway exceeding 240 beats/min should be considered for catheter ablation therapy of the accessory pathway prior to continuing competition. Those whose ventricular rate 81 ppear to be at low risk for sudden cardiac death. Congenital Complete Heart Block (i) Athletes with a structurally normal heart and normal cardiac function, with no history of syncope or near syncope, a narrow QRS complex, ventricular rates at rest greater than 40 to 50 beats/min increasing appropriately with exertion, no or only occasional premature ventricular complexes, and no VT during exertion can participate in all competitive sports. (ii) Athletes with ventricular arrhythmia, symptoms of fatigue, near-syncope, or syncope should have a pacemaker implanted before they participate in competitive sports. Athletes with pacemakers should not participate in competitive sports when the danger of bodily collision exists because such trauma may damage the pacemaker system. Before allowing athletes to engage in these activities, an exercise test should be conducted at the level of activity demanded by the particular sport so as to be certain that the paced heart rate increases appropriately. (iii) Athletes with abnormal hemodynamic status, such as those with an intracardiac shunt, cannot participate in any competitive sports without a pacemaker. Long QT Syndrome (i) Regardless of QTc or underlying genotype, all competitive sports, except those in class IA category should be restricted in a patient who has previously experienced either: 1) an out-of-hospital cardiac arrest, or 2) a suspected LQTS-precipitated syncopal episode. (ii) Asymptomatic patients with baseline QT prolongation (QTc of 470 ms or more in males, 480 ms or more in females) should be restricted to class IA sports. The restriction limiting participation to class IA activities may be liberalised for the asymptomatic patient with genetically proven type 3 LQTS (LQT3). (iii) Patients with genotype-positive/phenotype-negative LQTS (i.e., identification of a LQTS-associated mutation in an asymptomatic individual with a nondiagnostic QTc) may be allowed to participate in competitive sports. Although the risk of sudden cardiac death is not zero in such individuals, there is no compelling data available to justify precluding these individuals (who are being identified with increasing frequency) from competitive activities. Because of the strong association between swimming and LQT1, persons with genotype-positive/phenotype-negative LQT1 should refrain from competitive swimming. (iv) LQTS patients with an ICD/pacemaker should not engage in sports with a danger of bodily collision because such trauma may damage the pacemaker system. The presence of an ICD should restrict individuals to class IA activities. 6.9 Fits, Faints and Funny Turns Aetiology: п‚· Reflex syncope (abnormalities of heart rate п‚± vasomotor tone, HOCM) 82 History and Examination: This will provide valuable clues in most cases. Don’t forget to take a medication history and enquire about family members. Ask for eyewitness accounts. Cardiac aetiology is suggested by the following: п‚· Loss of consciousness and posture preceding convulsions п‚· Precipitation by exercise or by being startled п‚· Syncope preceded by palpitations п‚· Family history of sudden and unexplained death п‚· Family history of deafness in the patient or other family member п‚· Patients whose convulsions respond to anticonvulsants but continue to have episodes of loss of consciousness п‚· Abnormal CVS signs, abnormal ECG or both Investigation: п‚· ECG – look for heart block, WPW, LQTS, Brugada syndrome п‚· Echocardiogram – exclude HOCM, AS, arrhythmogenic RV cardiomyopathy (also consider MRI) п‚· Holter п‚· Event recorder п‚· Consider - Exercise test (if relevant history) - Tilt test (if relevant history) - EP study 6.9.1 Reflex Syncope Causes: п‚· Vasovagal syncope п‚· Cough syncope п‚· Micturition syncope п‚· Stretch syncope п‚· Carotid sinus hypersensitivity (more common in the elderly) п‚· Postural orthostatic tachycardia syndrome and orthostatic hypotension (POTS) Vasovagal syncope is characterised by a tendency to have recurrent faints. It arises when there is pooling of blood in the lower half of the body when the individual is in a sitting or standing position. Contraction of the under-filled heart sets up a reflex leading to an increase, then sudden drop in heart rate, profound hypotension or a combination of the above. Although the symptoms may seem disabling, and sometimes physical injury may occur if there are no warning symptoms, the condition is benign and will improve over time. The symptoms occur commonly during adolescence; males and females are equally affected. Symptoms of profound fatigue are commonly associated. Refer the patient/family to www.stars.org.uk Preventing attacks 83 п‚· п‚· п‚· п‚· п‚· Avoidance of vasodilating drugs. Increasing fluid intake: approximately 1.0–2.0 litres (depending on body habitus, age and co-morbidities) of non-caffeinated fluids (preferably plain water) by lunch-time, then sufficient fluid intake to keep urine clear. Caffeinated drinks may be taken in moderation in addition. Increased salt intake. Before standing: repeatedly elevate the heels to increase calf muscle contraction; stand up slowly; adjust to being upright before moving off Sleep with an extra pillow. If early warning symptoms of an attack are present (warmth, mild nausea or a “funny sensation in the stomach”, sweating, “feeling odd”): п‚· Sit, on the floor with head between drawn-up knees, OR п‚· Squat, on haunches if able, OR п‚· Lie, supine elevating the legs (preferred option), OR п‚· Legs crossed, tense stomach muscles, OR п‚· Clench fists and tense forearms repeatedly whilst breathing normally Lying down is the most effective way of preventing syncope. Drug Treatment п‚· Fludrocortisone: 50 Вµg once daily for 1 week, if tolerated increasing to 100 Вµg once daily and reviewed after 1 month. The maximum dose is 300 Вµg once daily. Supine blood pressure monitoring and 4–6 monthly electrolyte monitoring are mandatory. п‚· Midodrine: This пЃЎ-adrenoceptor agonist has no UK licence, but can be made available on a named patient basis. The starting dose (adolescents and older) is 2.5 mg twice daily, increasing to 5 mg twice daily and if necessary 10 mg twice daily. In severe cases, doses of up to 15 mg three times daily have been used (in adults). The last dose should be given no later than 18.00 given the potential side effect of supine systolic hypertension. Some patients benefit from as-needed doses before situations known to precipitate events. Blood test (full blood count, urea and electrolytes, glucose, bone chemistry, liver and thyroid function tests) and supine blood pressure monitoring are vital 4–6 monthly. Combination therapy with these agents may be needed for some patients. If fludrocortisone and/or midodrine therapy are continued beyond a year, 24-hour ambulatory blood pressure monitoring should be undertaken to look for occult nocturnal hypertension. п‚· Salt supplementation may be used in selected patients with no contraindications: the few studies available in small numbers of young patients used 120 mmol of salt (as slow sodium, 12 tablets per day in divided doses) daily in patients with 24-hour urinary sodium estimations of <170 mmol/24 hour. Patients are unlikely to tolerate more than 3–4 tablets twice daily because of nausea and vomiting. Blood pressure should be monitored closely, with discontinuation of salt therapy attempted after 1 year. п‚· Paroxetine: This is not recommended for the treatment of depression in children <18 as there may be an increased incidence of self-harm and suicidal ideation. In isolated cases, paroxetine (starting at 10 mg once daily) may be useful in the management of refractory vasovagal syncope. Its use should 84 probably be limited to patients with concomitant anxiety and depressive features. Where this is the case, clinical psychological and/or psychiatric advice should be sought and the prescription made only after careful discussion of potential adverse effects. Permanent pacing Evidence for the efficacy of permanent pacing in the management of vasovagal syncope is contradictory. Pacing may be considered in rare cases of "malignant" vasovagal syncope where there are repeated, unheralded, often injurious vasovagal syncope, with prolonged asystole on tilt testing, >3 seconds asystole during real-time syncope with pauses (on the table or with a Reveal device). Dual chamber pacing is mandatory, preferably DDI with hysteresis or a specifically designed algorithm for neurally mediated disorders (for example, rate drop response [for example, Adapta DR, Medtronic Inc), closed loop stimulation (for example, Cylos CLS, Biotronik]). Adapted from: Heart 2009;95:416-420 6.10 Hypercyanotic Spells IF A PARENT TELEPHONES REPORTING A SIGNIFICANT SPELL ARRANGE FOR IMMEDIATE HOSPITAL ADMISSION Context: п‚· Tetralogy of Fallot п‚· Other forms of RVOT obstruction п‚· Tricuspid atresia п‚· TGA/VSD/PS п‚· Pulmonary atresia/VSD with MAPCAs Mechanism This remains unclear but probably involves either an increased pulmonary vascular resistance or decreased systemic vascular resistance. This leads to an increased Rп‚®L shunt across the VSD and hence exacerbates the hypoxia, hypercarbia and acidosis further increasing the pulmonary vascular resistance and creating a downward spiralling process. A spell usually occurs for no apparent reason, often early in the morning although some may be precipitated by induction of anaesthesia, following a bath or when upset. The infant becomes pallid or cyanosed, and is irritable with prolonged cry. The intensity of the murmur decreases as less blood is ejected across the pulmonary valve. Spells may be brief (1-2 minutes) and self correct or may progress to a severe, lifethreatening episode. Emergency treatment п‚· Knee/chest position (parents should be taught this if there is a history of spelling) or compress both femoral arteries п‚· Administer 100% O 2 п‚· Continuous ecg and saturation monitoring, frequent bp 85 oring) hild to ICU for full cardio-respiratory support. Consider using: п‚· Phenylephrine (2-10 microgram/kg IV stat; infusion 1-5 microgram/kg/min), or п‚· Metaraminol (0.01 mg/kg IV stat; infusion 0.1-1 microgram/kg/min), or п‚· Norepinephrine, or ketamine. Long-term treatment – consider п‚· Prophylactic long-term propranolol п‚· Surgical shunt (may be needed urgently) п‚· Early surgical repair п‚· Pulmonary valvuloplasty Hypercyanotic episodes may also occur in patients with Eisenmenger syndrome due to a sudden increase in pulmonary vascular resistance or drop in systemic vascular resistance. Give oxygen, volume expansion and consider use of morphine. 6.11 Kawasaki Disease Diagnostic criteria Fever for longer than 5 days plus at least 4 of: п‚· Non-purulent conjunctivitis п‚· Changes in the mouth (strawberry tongue or red, cracked lips) п‚· Changes in the periphery (erythema, oedema, of feet + hands) п‚· Polymorphous rash п‚· Cervical lymphadenopathy (>1.5 cm) Additional features Acute phase (up to10 days) п‚· Irritability (>90%) – may have aseptic meningitis п‚· Sterile pyuria (70%) п‚· Arthritis (40%) п‚· GI symptoms (25%) п‚· ECG changes п‚· Myocarditis п‚· Pericarditis Sub-acute phase (>10 days) п‚· Desquamation п‚· Coronary aneurysms (10-20% if not given IVIG) п‚· Pericardial effusion п‚· Thrombocystosis Atypical Kawasaki Disease 86 п‚· п‚· п‚· п‚· п‚· п‚· May occur in some patients, more often in infants Full diagnostic features not seen – often just fever and 1-2 others; no other explanation for symptoms Should be considered in any infant with fever lasting >5 days and no other explanation Laboratory features are compatible Often complicated by coronary artery aneurysm because of delayed presentation Diagnosis and management are guided by echo features and lab findings Clinical Course of KD Acute phase (up to 10 days) Persistent pyrexia, irritability with bilateral conjunctivitis and rash. Hands and feet develop the erythema and edema. Tongue and oral mucosa become red and cracked. Hepatic dysfunction, myocarditis and pericarditis may develop. Subacute stage (days 11-20) Persistent irritability, anorexia, and conjunctival injection. Resolution of fever (if it persists the greater the risk of cardiac complications). Thrombocytosis develops. Desquamation of the fingertips and toes begins. Aneurysm formation may occur. Convalescent phase (days 21-60) The most significant clinical finding that persists through this phase is the presence of coronary artery aneurysms. Chronic phase This stage is only of clinical importance in patients who have developed cardiac complications. Its duration is of lifetime significance since the aneurysm formed in childhood may thrombose or rupture in adulthood. In many cases of adults presenting with coronary artery aneurysm, careful reviews of past medical histories have revealed febrile childhood illnesses of unknown aetiology. Investigations ESR (often > 100) CRP WBC Platelets (may be > 1000) ASO titre to exclude streptococcal infection There may be evidence of anaemia, pyuria and deranged LFTs Echo features п‚· Coronary artery aneurysms п‚· Myocarditis п‚· Pericarditis п‚· Valve regurgitation п‚· May be normal Treatment Acute phase (up to 10 days): п‚· IVIG 2g/kg single dose as slow i.v. infusion or IVIG 400 mg/kg/day for 4 days п‚· Aspirin is given in an anti-inflammatory dose until o the child has been afebrile for 48 to 72 hours, or o the ESR/CRP normalise, or o day 14 of illness and 48 to 72 hours after fever cessation. o Recommended dose acute phase is either 30-50 mg/kg/day in 4 divided doses (UK recommendations), or 87 80-100 mg/kg/day given in 4 divided doses (USA and Japan recommendations). Then change to low-dose aspirin (3 to 5 mg/kg per day) and maintain it until the patient shows no evidence of coronary changes by 6 to 8 weeks after the onset of illness. Low-dose aspirin should be continued for as long as coronary abnormalities persist. Avoid concomitant use of ibuprofen (antagonises the irreversible platelet inhibition induced by aspirin). Subacute phase (>10 days): aspirin 3-5 mg/kg/day; discontinue at 6-8 weeks if no coronary involvement. If there is no response to IVIG or recurrence of fever within a few days, reconsider the diagnosis and consider a second dose of IVIG. Further failure to respond should can be treated with: п‚· Corticosteroids, either o Intravenous pulse methylprednisolone, 30 mg/kg for 2 to 3 hours, administered once daily for 1 to 3 days, or o Prednisolone 2 mg/kg/day, 3 times daily, given by IV injection until the fever resolves and then orally until the C-reactive protein (CRP) level normalizes, then taper over 15 days in 5-day steps (2 mg/kg/day for 5 days, 1 mg/kg/day for 5 days, and 0.5 mg/kg/day for 5 days). п‚· Infliximab can be considered for resistant cases. Patients presenting >10 days, but with coronary artery aneurysms should receive IVIG even if there is no good evidence of active disease. Monitoring There are no hard and fast rules regarding the follow-up arrangements although the American Heart Association produced guidelines in 2004 (Reference: Circulation 2004;110:2747-71). The acute phase proteins and platelets should be monitored until normal. A baseline echocardiogram should be undertaken at or around the time of diagnosis, then again within 2 weeks if coronary aneurysms are noted, or at 6-8 weeks if the initial echo was normal. If the echocardiogram and ECG are normal at six months then there is no evidence that de novo coronary abnormalities will be identified after this period, although the AHA guidelines suggest continuing follow-up for one year and UK guidelines suggest indefinite follow-up is needed. 6.12 MRSA Infection Methicillin resistant Staphylococcus aureus (MRSA) is resistant to all beta-lactam antibiotics (penicillins, cephalosporins) and may be resistant to other classes of antibiotics (multiple-resistant MRSA). Some strains of MRSA are epidemic in character and may cause serious outbreaks of infection in hospitals. MRSA can colonise patients, staff and the hospital environment. Once established in a hospital, MRSA may never be eradicated. The single most important measure to prevent and contain MRSA is meticulous hand hygiene, particularly with alcohol hand rubs. Each situation must be dealt with individually and more detailed advice should be obtained from the Infection Control Team. 88 Patients with MRSA colonisation or infection prior to cardiac surgery must be highlighted to the surgical team. Precautionary measures such as eradication measures, final slots on the operating list and isolation cubicles may be necessary. 6.13 Nutrition in Cardiac Patients 6.13.1 Faltering Growth Malnutrition and faltering growth are both commonly associated with congenital heart disease, especially in infants with Lв†’R shunt or cyanotic heart lesions like hypoplastic L heart syndrome. There may be increased surgical risks in infants who are malnourished. The reasons for poor growth in infants with CHD are multifactoral and include: п‚· Fatigue on feeding leading to poor intake п‚· Increased metabolic expenditure п‚· Early satiety п‚· Anorexia п‚· Vomiting п‚· Fluid restriction. Nutritional supplementation is often required. Specialist dietetic input is advisable. Maximising Calories Breastfeeding Calories can be added in the following ways: п‚· Hospital setting: o C & G Nutriprem Breast Milk fortifier o SMA Breast milk fortifier п‚· Community setting: o Adding 3-5% infant formula powder to EBM. o Use of a glucose polymer (Maxijul) and/or a fat emulsion (Calogen) as a 'dose' prior to a breast feed Infant Formula п‚· Use of High Energy infant formula e.g. Infatrini, SMA High Energy п‚· Increasing the concentration of normal infant formula (specialist dietetic advice needed – not all formulas are suitable) п‚· Adding further calories to the above by adding a calorie supplement e.g. Duocal, Maxijul, Calogen Protein hydrolysate formula п‚· Hypoallergenic extensively hydrolysed formulae are useful in adverse reactions to normal infant formula or cows’ milk п‚· May be useful in Gastro-oesophageal reflux A wide variety is available and specialist dietetic advice is required. (Source: Miss Bethany Glassar, Paediatric Dietician) 6.13.2 Gastro-Oesophageal Reflux (GOR) This is a common problem in all babies (estimated prevalence at 3-4 months is 50%), but 90-95% of infants are symptom free by 1 year of age. GOR is probably more severe in cardiac babies. GOR disease may lead to anaemia, faltering growth, food refusal, troublesome vomiting, symptoms of pain and haematemesis due to 89 oesophagitis with the risk of oesophageal stricture formation, respiratory problems (wheeze, cough, apnoeas, stridor and recurrent infection secondary to aspiration) and excessive crying/sleep problems. Ensure that the dietician is involved early in the management. If the diagnosis is unclear or symptoms fail to resolve with simple measures then further investigations can clarify the presence and extent of reflux and exclude other problems. These include a barium swallow and 24 hour pH study. A variety of treatment strategies may be employed. Simple measures: п‚· Positioning prone or left lateral during the day (but supine at night) п‚· Head-up tilt of the cot (30-40o) п‚· Thickened feeds (e.g. Carobel) п‚· Enfamil AR to reduce the vomiting п‚· Gaviscon to reduce the oesophagitis. More severe reflux may require: п‚· H 2 antagonist (ranititdine) п‚· Proton pump inhibitors п‚· If there is no clinical improvement to standard treatment discuss further management with the Gastroenterology team. Reference: ADC 2010;25:243-4 Comment on domperidone High dose domperidone (particularly IV) is rarely associated with cardiac arrest, probably through prolongation of the QT interval. It should be avoided in patients with LQTS and should be used with caution in cardiac patients. If the risk/benefit ratio favours treatment, then domperidone may be prescribed but within recommended dosages and with pre- and post-treatment ECGs to permit QT interval monitoring. 6.14 Oxygen Therapy in Cardiac Patients Indications for Home Oxygen Therapy include: п‚· Pulmonary arterial hypertension п‚· Concurrent lung disease When it is decided that home O 2 therapy is needed, a referral should be made to the paediatric respiratory team. п‚· If there is a suspicion of concurrent lung disease, the referral should be made to the consultant. п‚· Where the indication for home O 2 therapy is primarily cardiac, the referral should be made to the paediatric respiratory nurses. An oxygen prescription form should accompany the referral and this should specify: o The desired O 2 flow rate (usually up to 2 L/min if the indication is PHT) o The frequency and type of monitoring (e.g. no monitoring, spot checks, overnight pulse oximetry, combination of spot checks and overnight monitoring) 90 п‚· 6.15 When changes to home O 2 therapy are desired (e.g. O 2 flow rate, type of monitoring), the paediatric respiratory nurses should be informed and a new prescription form should be issued. Premature Beats in Newborn Babies In most cases the finding of “extra beats” in newborn babies is not significant. They represent a developmental phenomenon and the vast majority disappear without any consequence in early infancy. A small proportion (<1-2%) may go on to develop significant arrhythmias such as SVT. In general these extra beats are premature atrial complexes (PACs) but in a small number they are premature ventricular complexes (PVCs). In a small proportion of cases there is an underlying problem with the baby and the following should be considered: п‚· Severe acidosis / hypoxia п‚· Metabolic disturbance o Hyperkalemia o Hypocalcaemia o Renal failure o Hypoglycemia п‚· Infection / septicaemia п‚· Left atrial mass A careful assessment of the baby needs to be undertaken by the neonatal team before referral to cardiology. The assessment should include: п‚· History o Maternal drug history o Birth details – difficult labour resulting in hypoxia п‚· Examination п‚· Bloods electrolytes, renal function, glucose п‚· Baseline ECG, with calculation of QTc If the baby is otherwise well: п‚· The neonatal/midwifery team should observe for symptoms for 2 days. п‚· If the baby remains well and a repeat ECG on Day 2 or 3 of life shows occasional PACs or PVCs, the baby can be discharged with arrangments in place for ward or clinic review and repeat ECG in 1 week. п‚· At the 1 week review, if PACs/PVCs persist, the baby should be referred to the paediatric cardiology outpatient clinic or the speciality doctor echo clinic on semi-urgent priority (2-4 weeks). The identity of the on-call consultant paediatric cardiologist on the day of referral should ascertained and a formal referral should be made, at the same time as requesting an urgent 24 hour Holter (specifying “ copy of result to Dr [Consultant], please”). If the baby becomes symptomatic/unwell at any point, an urgent referral for paediatric cardiac assessment, including echocardiography, should be made. 91 6.16 Prescribing Drugs Safely 6.16.1 Medication Errors Medication Errors are common but fortunately adverse clinical consequences are rare. Prescription errors by doctors account for the majority of errors. Therefore it is essential to ensure the following: 1. An accurate drug history is taken. Bear in mind that parents often know doses in ml not mg and they may use different strengths of liquids at home to those kept in hospital. Always remember to ask about medicines bought over the counter and any alternative medicines (e.g. herbal, homeopathic, Chinese medicines) 2. The prescription chart includes all relevant information, including the weight of the patient, date of birth, allergies and all required medications. 3. All handwriting must be neat and clearly legible. Use CAPITAL LETTERS and write in black indelible ink (blue does photocopy, but black is preferred). 4. Avoid abbreviations for drug names and amounts (e.g. write “microgram” instead of using the symbol, m or "mcg"). Approved names should be used where possible. Take great care in prescribing compound preparations (it is sometimes safer to use brand names in this situation). 5. All prescriptions must be signed and dated at the time of writing. 6. Regular prescriptions can be changed once (dose, frequency or route) – enter the date of change and initial the change. 7. Changes to the chart are discussed with the nursing staff and discontinued medications are crossed out. 8. When drugs are prescribed “as required/prn” the prescription must include the indication for drug administration (e.g. “pain”), the interval between doses and the maximum dose in 24 hours. 9. Use ONE chart per patient, but if the number of medications exceeds one chart, label each chart clearly “chart 1 of 2”, chart 2 of 2” etc. Charts must be regularly reviewed to condense the number of charts to a minimum. Continuation sheets are not allowed. 10. The re-writing of charts is a common source of errors – ensure ALL relevant information is transcribed, including weight and allergies. The start date of a drug is the ORIGINAL start date, not the date the chart was rewritten. Have a colleague or nurse double-check any rewritten chart. 11. Prescription charts should be regularly reviewed for tidiness and legibility. Charts must be rewritten whenever the legibility of a drug is compromised, e.g. something spilled on the chart, 12. When a supplementary chart is used (e.g. steroid, anticoagulant, insulin, etc.) the drug must be identified on the main prescription chart, with time of administration documented, and annotated “see accompanying chart”. 13. Numbers for dosages must be clear and unambiguous – take particular care over 4s and 9s, for example. 14. Decimal points must not be employed unless unavoidable. If the use is necessary, the decimal point must be precisely marked and, if appropriate, preceded by a zero (0), e.g. 0.5 ml, not .5 ml. 15. In cases where the dose is prescribed in units, e.g. heparin and insulin, the dose must be prescribed as UNITS, not u, as there is a risk that a “u” may be mistaken for a “0” and leading to a 10X dose error. 92 16. When prescribing a liquid preparation (injection, oral mixtures etc.), the dose must be in milligrams / micrograms / nanograms, not mls, unless it is avoidable to use a volume. 17. On discontinuation of a prescribed drug, the “crossing off” should occur through the prescribing section of the chart and through the section of the chart used to record the drug’s administration. 18. The weights for all children must be recorded on the drug chart in Kg (the policy only asks for children<12yrs). 19. Ensure that changes to the drugs are communicated to the nursing staff (and patient/parents/carers if appropriate. 20. If in any doubt, speak to the Pharmacy Department. Reference: http://nww.cardiffandvale.wales.nhs.uk/pls/portal/docs/PAGE/POLICY_PAGEGRO UP/LIBRARY/REF%20242%2030JAN07.PDF Drug interactions are not uncommon in paediatric cardiology. Common potential interactions are: warfarin – amiodarone / antibiotics digoxin – amiodarone / macrolides / carvediolol clopidogel – proton pump inhibitors (reduced antiplatelet effect) ciclosporin – macrolides Management of Medication Errors: Errors or interactions that result in a change in the clinical status of the patient should be dealt with in the following manner: 1. Record the event in the case record notes 2. Discuss the event with the appropriate consultant 3. Fill in a clinical incident form 4. Discuss the event with the relatives (the consultant may choose to do this) 6.16.2 Quick Calculations of Drug Concentrations for Infusions Quick Calculations: Drug Concentrations For infusions DESIRED CONCENTRATION Mcg/kg/min 0.01mcg/kg/min = 1ml/hr CALCULATION DOSE in MG in a 50ml Syringe 0.03 X Weight (Kg) 0.02 mcg/kg/min =1ml/hr 0.06 X Weight (Kg) 0.05 mcg/kg/min = 1ml/hr 0.15 X Weight (Kg) 0.1mcg/kg/min=1ml/hr (concentrated) 0.3 X Weight (Kg) 0.33mcg/kg/min = 1ml/hr 1 mcg/kg/min = 1ml/hr 1.0 3 X Weight (Kg) X Weight (Kg) 93 DRUGS DOSE RANGE Mcg/kg/min Epinephrine 0.01 - 1.0mcg/kg/min Norepinephrine 0.01 - 0.5mcg/kg/min PGE1 0.005 - 0.05mcg/kg/min Isoprenaline 0.025 - 1.0mcg/kg/min Epinephrine 0.01 - 1.0mcg/kg/min Norepinephrine 0.01 - 0.5mcgkg/min Epinephrine 0.01 - 1.0mcg/kg/min Norepinephrine 0.01 - 0.5mcg/kg/min Epinephrine 0.01 - 1.0mcg/kg/min Norepinephrine 0.01 - 0.5mcg/kg/min Isoprenaline 0.025 - 1.0mcg/kg/min Milrinone 0.33 - 0.99mcg/kg/min Nitroprusside 0.5 - 10mcg/kg/min Nitroglycerin 0.5 - 10mcg/kg/min 2 5 mcg/kg/min mcg/kg/min = 1ml/hr = 1ml/hr 6 15 X Weight (Kg) X Weight (Kg) 10 mcg/kg/min = 1 ml/hr 30 X Weight (Kg) 20 mcg/kg/min 25 mcg/kg/min 50 mcg/kg/min = 1ml/hr = 1ml/hr = 1ml/hr 60 X Weight (Kg) 75 X Weight (Kg) 150 X Weight (Kg) Mcg/kg/hr 10 mcg/kg/hr = 1ml/hr 20 mcg/kg/hr = 1ml/hr Units/kg/hr DOSE in MG in a 50ml Syringe 0.5 X Weight (Kg) 1 X Weight (Kg) Midazolam Midazolam Dopamine Amiodarone Dobutamine Dopamine Amiodarone Dobutamine Lidocaine Esmolol Esmolol Morphine Morphine 1 - 6mcg/kg/min 1 - 6mcg/kg/min 5 - 20mcg/kg/min 5 - 15mcg/kg/min 2 - 20mcg/kg/min 5 - 20mcg/kg/min 5 - 15mcg/kg/min 2 - 20mcg/kg/min 20 - 50mcg/kg/min 100 - 300mcg/kg/min 100 - 300mcg/kg/min Mcg/kg/hr 10 - 40mcg/kg/hr 10 - 40mcg/kg/hr *Titrate up as required DOSE in UNITS in a 50ml Syringe 10 X 50 X Weight (Kg) Heparin – standard concentration Mg/kg/hr 0.25 mg/kg/hr = 1ml/hr DOSE in MG in a 50ml Syringe 12.5 X Weight (Kg) Mg/kg/hr Frusemide Mg/kg/day 1 mg/kg/day = 1ml/hr DOSE in MG in a 50ml Syringe 2 X Weight (Kg) Mg/kg/day Phenoxybenzamine 0.5 - 2 mg/kg/day DOSE in UNITS in a 50ml Syringe 0.3 X Weight (Kg) (Max: 50 Units/50ml) Units/kg/min 10 Units/kg/hr = 1ml/hr Units/kg/min 0.0001 Unit/kg/min = 1ml/hr Nanogram/kg/min 5 nanogram/kg/min = 1ml/hr (0.005 mcg/kg/min = 1ml/hr) Standard concentration titrate as per order DOSE in MICROGRAM in a 50ml Syringe 15 X Weight (Kg) DOSE in GRAM in a 40ml Syringe 1G in 40ml of sterile water = 25mg/ml Units/kg/hr 0.25 - 0.5mg/kg/hr Vasopressin Sepsis: 0.0001 - 0.001units/kg/min Vasodilatory Shock post CV surgery: 0.001- 0.002 units/kg/min Brain death: 0.0003 units/kg/min Diabetes Insipidus: 0.0001 - 0.00025 units/kg/min Nanogram/kg/min Epoprostenol 40nanogram/kg/min (Prostacyclin or Flolan) Mg/kg/hr Thiopentone 2 - 4 mg/kg/hr Refer to Paediatric Critical Care Medicine: Handbook of Clinical Practice. Department of Critical Care Medicine. Rev Nov 2001, Critical Care Unit, Hospital For Sick Children. 6.17 Propranolol for the Treatment of Capillary Haemangiomas Patients needing Propranolol for treatment of Haemangiomas should be referred to the Paediatric Cardiology Echo clinic for a full cardiology clinical assessment, ECG and echocardiogram prior to starting the medication. If the assessment is satisfactory, patient will then be admitted and follow-up under the respective team. 94 5 - Dosage regime Week 1: п‚· 1mg/kg/day divided into three doses (i.e. 0.33 mg/kg per dose given tds) Week 2: п‚· Increase the dose up to 2mg/kg/day divided into three doses (i.e. 0.66 mg/kg per dose given tds). There should be a monthly up-titration of dose in line with weight gain up to 9 months of age if there is no clinical improvement. From 9 months: п‚· Keep the patient on the same dose without weight adjustment until month 12 unless there is a need to continue. Stopping medication: п‚· Propranolol should be stopped by halving the dose for 1 week, halving that dose for the second week, then stopping. Monitoring of patients on admission п‚· Baseline blood glucose (BM stick) prior to first dose, prior to first discharge and before all GA. п‚· HR and BP must be checked every half hour for the first four hours in the hospital after starting and increasing treatment (week 1 and week 2). п‚· Then check observations locally twice a week for the first two weeks, then once a week for the entire treatment period. Side Effects These are uncommon, but must be explained to parents/guardian before commencing Propranolol therapy. They include: п‚·пЂ Bradycardia п‚·пЂ Heart failure п‚·пЂ Hypotension п‚·пЂ Cardiac conduction disorder п‚·пЂ BronchospasmпЂЄ п‚·пЂ Peripheral vasocostriction п‚·пЂ Weakness and fatigue п‚·пЂ Sleep disturbance п‚·пЂ Hypoglycaemia пЂЄ Propranolol should not be given with Salbutamol or any others selective ОІ2 -agonists. If bronchodilatation is needed, Ipratropium Bromide (Atrovent) should be used. NB – PATIENTS MUST AVOID PROLONGED FASTING ON A ОІ-BLOCKER – parents must be told that if the patient has vomiting and is not tolerating fluids they should temporarily stop the treatment and telephone for advice. There should be a low threshold for giving NG rehydration (risk of hypoglycaemia). References: 1 Enjorlas O, Mulliken JB. Vascular tumours and vascular malformations.Adv Dermatol 1997;13:375-423 2 LГ©autГ©-LabrГЁze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taieb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008 Jun 12;358(24):2649-51 95 6.18 Protein-losing enteropathy (PLE) / Plastic bronchitis (PB) after Fontan Definition/Incidence/Aetiology PLE is a condition characterised by excessive gastrointestinal protein loss. The liver is unable to compensate and this leads to hypoalbuminaemia, hyopoproteinaemia and reduced serum immunoglobulins. Cardiac causes include the Fontan circulation (1015% of patient long-term), severe CCF and constrictive pericarditis. The risk factors for PLE following the Fontan operation include: п‚· Systemic RV п‚· Perioperative renal failure п‚· High venous pressures п‚· History of perioperative chylothorax or chyloperitoneum п‚· Preoperative infection п‚· ?Lack of fenestration Clinical features п‚· Oedema (facial and lower body) п‚· Abdominal distension п‚· Ascities п‚· Anorexia п‚· Loose stools/flatus Any report of these symptoms in Fontan patients should prompt investigation. Plastic Bronchitis is the pulmonary equivalent of PLE. The features are: п‚· Cough п‚· Choking п‚· The expectoration of tenacious material which, if spread out, takes the shape of the bronchial tree – this is a “cast” – the material is proteinaceous Laboratory and Other Investigations It may be advisable to perform annual LFTs in all postoperative Fontan patients. Patients with clinical features should have the following investigations: п‚· FBC (with differential) п‚· U&E/creat/LFT/Ca2+ п‚· Serum immunoglobulins п‚· Stool vs serum пЃЎ-1 antitrypsin п‚· Urinary protein (to rule out renal protein loss) п‚· ECG/24 hour tape – to rule out arrhythmia п‚· Echo – to rule out haemodynamic cause п‚· Cardiac catheterisation – to rule out haemodynamic cause and consider intervention (e.g. transcatheter fenestration) п‚· ?MRI (relationship of lateral tunnel to native RA) п‚· Consider need for GI investigations, including endoscopy, to exclude primary GI causes п‚· Baseline bone densitometry studies (dexa scan) – heparin and prednisolone, which are both used to treat PLE, cause bone demineralisation. 96 Treatment PLE and PB are complex clinical problems and are managed on a case-by-case basis with close involvement of the surgical centre (п‚± other tertiary centre such as GOS or Birmingham). General supportive measures include: п‚· High-protein, MCT diet п‚· ACE inhibitors + diuretics should be considered, particularly if there is significant AV valve regurgitation п‚· Consideration of sildenafil (to reduce PVR) Anti-PLE treatments also include: п‚· High-dose steroids (2 mg/kg per day, tail according to response) п‚· Subcutaneous heparin injections (the dose is usually sub-therapeutic [doses described are 1500 unit subcutaneous bd empirically, regardless of weight, OR 5000 U/m2 bd] and warfarin should be continued – monitor KCCT and INR (NB – follow up bone density needed) п‚· Consider IV albumin if the serum albumin is < 25 and peripheral oedema is severe п‚· Septrin if evidence of immunosupression. Anti-PB treatments also include: п‚· Nebulised steroids п‚· Nebulised normal saline, TPA, urokinase, or N-acetyl cysteine п‚· Chest physiotherapy п‚· Bronchoscopy Therapeutic interventions for PLE and PB include: } transcatheter or п‚· Addressing any haemodynamic problem } operative п‚· Fenestration п‚· Transplantation (but in up to 50% of cases the PLE persists) Outcomes The 5 year mortality in patients with a venous pressure of 16 mmHg is 50%. 25% of patients may respond to general supportive measures and anti-PLE medications. Papers suggest that surgical outcomes are poor, but this may relate to case selection. Patients with PLE, therefore, require close surveillance. 6.19 Pulmonary Hypertension in Childhood 6.19.1 Definition, Classification and WHO Functional Status Pulmonary Hypertension The normal range for pulmonary artery (PA) pressure is dependent on age. In general, resting peak PA pressures should be around 1/3 of systemic blood pressure (i.e. 25-40 mmHg depending on age) and mean PA pressure should be no more than 22-25 mmHg). Pressures in excess of these values indicate pulmonary hypertension. WHO Classification of pulmonary hypertension (2003, “Venice classification”) п‚· Pulmonary arterial hypertension (includes idiopathic [IPAH], familial and that due to congenital heart disease) 97 п‚· п‚· п‚· п‚· Pulmonary hypertension with pulmonary venous hypertension due to left heart disease Pulmonary hypertension with lung disease В± hypoxaemia Pulmonary hypertension due to thrombo-embolic disease Miscellaneous causes (e.g. inflammatory diseases, malignancy) Functional Status In 1998 the World Health Organisation published a functional classification modified after the New York Heart Association (termed modified NYHA class): Class I Class II Class III Class IV No symptoms or limitation in activity No resting symptoms. Ordinary physical activity causes undue breathlessness, fatigue, chest pain or syncope No resting symptoms. Minimal (less than ordinary) activity results in undue breathlessness, fatigue, chest pain or syncope Unable to carry out any activity without symptoms of breathlessness, fatigue, chest pain or syncope. Symptoms may be present at rest. 6.19.2 Pathophysiology Pumonary vascular resistance (PVR) is high in fetal life, but should fall rapidly at birth and should attain adult levels by 4-6 weeks of life. In some individuals there is a failure of pulmonary resistance to fall to normal levels, with a consequent continued high pressure in the pulmonary artery (persistent pulmonary hypertension of the newborn). In idiopathic pulmonary arterial hypertension there is a rise in pulmonary vascular resistance and pulmonary artery pressure in later life, the cause of which is unclear. In many of these cases there may be a familial or genetic component (familial pulmonary arterial hypertension). Molecular genetic studies have identified mutations in the BMPR2 gene of chromosome 2q33 in some individuals with pulmonary arterial hypertension. Other genetic associations exist. In some individuals an associated disease process within or outside of the lungs leads the resistance vessels to constrict down again, with consequent elevation in pulmonary resistance and pressure. The vasoconstriction can rapidly progress to an obstructive process of the pulmonary vessels due to local thrombosis and fibrosis. Associated causes include: п‚· Congenital heart disease with systemic-to-pulmonary shunting (see below) п‚· Exposure to certain drugs and toxins п‚· Human immunodeficiency virus infection. Pathophysiological basis for pulmonary arterial hypertension associated with CHD Presence of a Lв†’R shunt at atrial, ventricular or great artery level leads to increased flow of blood to the lungs and, in the case of a large VSD or PDA, the transmission of increased pressures to the pulmonary circulation. Left unrepaired these lesions 98 frequently lead to irreversible “pulmonary vascular disease” with hypertrophy and fibrosis within the pulmonary resistance vessels. Once pulmonary vascular disease is established it renders the original heart lesion inoperable; if surgery were undertaken, it would expose the right ventricle to high resistance (and therefore pressure), leading to acute or chronic right heart failure. Surgical repair for CHD with associated PAH is ordinarily undertaken early in the first year of life – ideally in the first 6 months. This permits the pulmonary vasculature to return to normal and life expectancy is excellent. Where repair is delayed, the pulmonary pressures may fall, but not to normal levels. Subsequent stresses in later life, such as pregnancy, may lead to further changes in the primed pulmonary vascular bed and severe pulmonary hypertension may develop. Eisenmenger syndrome There is a subset of patients with CHD where the defect is not repaired in a timely manner. Once irreversible changes develop they tend to progress, leading to occlusion of small pulmonary blood vessels. This reduces the size of the pulmonary vascular bed and increases pulmonary vascular resistance further. When PVR > SVR, this leads to “right-to-left shunt”, “shunt reversal”, or Eisenmenger syndrome (after Dr Victor Eisenmenger’s description in 1897). Complications of Eisenmenger syndrome include: п‚· Polycythaemia п‚· Hyperviscosity of blood п‚· Gout п‚· Finger clubbing п‚· Cerebral abscess and cerebral thrombosis п‚· Progressive exercise intolerance п‚· Haemoptysis п‚· Arrhythmia п‚· Syncope (due to failure to increase cardiac output acutely) and “pulmonary hypertensive crises” (due to sudden increase in PVR) п‚· Sudden death 6.19.3 Treatment of Pulmonary Hypertension Left untreated, the median survival of children with idiopathic or familial pulmonary arterial hypertension is around 4 years. The outlook is better for patients with Eisenmenger syndrome, with many patients surviving beyond their 20s, but developing significant limitation by their 30s. Patients with significant PAH should be referred to the specialist PAH Team from GOS. 99 Empirical treatment of Pulmonary Hypertension Treatment Action Diuretics Increase urine output and reduce fluid retention Oxygen Pulmonary vasodilator Digoxin Slows heart rate and increases force of contraction of the heart Improves cardiac output acutely in patients with IPAH Warfarin Anticoagulation In some studies, nearly doubles 3-year survival in primary PHT; some studies have shown no benefit. Aspirin Anti-platelet action No evidence of benefit Cause pulmonary and systemic vasodilation Benefit shown in patients with IPAH, good cardiac output and a “vasodilator” response at cardiac catheterisation Non-selective vasodilators, e.g. nifedipine Evidence Base Lacking, but considerable clinical experience. May benefit Eisenmenger patients with right heart failure or significant valve regurgitation. Benefit shown in patients with chronic lung disease and nocturnal hypoxia Permits Rп‚®L shunting Non-controlled trials have at atrial level, shown improved cardiac Atrial preserving cardiac septostomy output following the output at the expense of procedure systemic desaturation IPAH – idiopathic pulmonary arterial hypertension Comment Provide symptomatic relief in patients with right heart failure. May cause hypovolaemia and reduce cardiac output by reducing right ventricular preload. No controlled evidence of benefit in IPAH or Eisenmenger syndrome No evidence of chronic benefit in IPAH; no evidence of any benefit in Eisenmenger syndrome No evidence of benefit in Eisenmenger patients, in whom fatal haemoptysis is described. Maintaining INR between 1.5-2 is proposed by some authorities. May be used in paediatric patients with pulmonary hypertension, in whom formal anticoagulation is problematical or contraindicated. May benefit between 6-10% of children with idiopathic pulmonary arterial hypertension. Use not recommended in Eisenmenger syndrome – may worsen Rп‚®L shunt if systemic vascular bed preferentially dilated. High-risk procedure (mortality up to 16%), only performed in specialist centres. “Targeted” therapy in PAH Treatment Action Prostaglandin therapy Vasodilation, inhibition of platelet aggregation, antiproliferative, promotion of remodelling Bosentan Endothelin I and II antagonist Evidence Base Continuous i.v. epoprostenol improves quality of life, exercise capacity and life expectancy Nebulised iloprost improves exercise capacity and haemodynamics Safety and efficacy shown in open label and randomised controlled trials in IPAH; increasing 100 Comment ВЈВЈВЈ - epoprostenol ВЈВЈВЈ - iloprost (the unlicensed iv form of iloprost given via nebuliser is cheaper ВЈ). Epoprostenol and iloprost are not licensed for use in children. ВЈ Available in oral form. Bosentan licensed for use in PAH and Eisenmenger syndrome Sildenafil Phosphodiesterase 5 inhibitor – causes vasodilation of pulmonary and systemic vascular beds evidence of efficacy in Eisenmenger patients. Safety and efficacy for treatment in IPAH shown in short-term trials. Growing evidence of benefit in Eisenmenger syndrome. in over 12s. ВЈ Available in oral and IV forms. Has license for treatment of PAH. Patients require treatment with more than one form of therapy. 6.19.4 Persistent Pulmonary Hypertension of the Newborn (PPHN) Introduction: In utero only 10-15% of the cardiac output reaches the lungs via the pulmonary circulation. Oxygenated umbilical venous blood is streamed to the left atrium (and the brain) and the aorta (and the body) by the combined effects of the foramen ovale, the ductus arteriosus and pulmonary arterial vasoconstriction. After delivery, inflation of the lungs and increased PaO2 are the principle factors promoting pulmonary vasodilatation, reduced pulmonary vascular resistance (PVR) and improved pulmonary perfusion. Failure to achieve this expected fall in PVR and therefore failure of oxygenation of the venous blood returned to the heart is described by the term Persistent Pulmonary Hypertension of the Newborn (PPHN). PPHN is being increasingly recognised in neonatal practice with an estimated incidence of 2-6/1000 births. It can occur in both term and preterm neonates and is perhaps the most common cause of death in infants of birth weight > 1000g. Predisposing factors: п‚· Hypothermia, hypoglycaemia, hypoxia and acidosis п‚· Bacterial pneumonia, meconium aspiration syndrome, surfactant deficiency lung disease п‚· Chronic fetal hypoxia, placental insufficiency, postmaturity, polycythaemia п‚· Congenital diaphragmatic hernia п‚· Primary pulmonary hypoplasia п‚· Congenital alveolar capillary dysplasia Clinical features: п‚· The most important clinical feature is difficulty in oxygenating the neonate with persistent low O 2 saturations despite increasing FiO 2 and ventilatory support. п‚Ё The blood gas (arterial) is likely to show severe hypoxemia. п‚Ё There is significant difference in pre and post ductal PaO 2 (>2.5 kpa) or O 2 saturations (5-10%). п‚· A prominent right ventricular impulse may be noted and murmurs due to tricuspid regurgitation or pulmonary regurgitation may be heard. п‚· Signs of heart failure may be present. Investigations: п‚· Chest X-Ray should be done in any neonate who is difficult to oxygenate to look for evidence of parenchymal lung disease and cardiomegaly. In PPHN it is likely to show pulmonary oligaemia. 101 п‚· п‚· п‚· п‚· Septic screen including FBC, CRP and blood cultures may be indicated. Blood glucose Hyperoxia-hyperventilation test may not be very useful in differentiating PPHN from cyanotic heart disease. Echocardiography is the gold standard and should be used to establish the diagnosis and inform management. Echocardiographic assessment of pulmonary hypertension1: Tricuspid regurgitation: п‚· RV pressure can be calculated from the TR jet (4v2 + add estimated RA pressure) п‚· Ensure the envelope is complete п‚· Interpret in the context of systemic BP Atrial shunting and other shunts: п‚· Some degree of right-to-left atrial shunting through the patent foramen ovale is common, although it is rare for this to be purely right-to-left (Pure right-to-left flow indicates total anomalous pulmonary venous connection [TAPVC] until proved otherwise). п‚· Bowing of the interatrial septum to the left is commonly seen. п‚· Right-to-left atrial shunting reflects right atrial filling (diastolic) pressure п‚· If a VSD is present, bidirectional shunting may be noted. Ductal flow: п‚· The direction and velocity of ductal blood flow can give useful information on PAP. п‚· Pure right-to-left flow indicates PAP is higher than aortic pressure throughout cardiac cycle. п‚· Bidirectional flow occurs when the aortic and pulmonary pressures are approximately equal. Flow is left-to-right during diastole and right-to-left during systole (as the pulmonary arterial pressure wave reaches the duct before the aortic pressure wave). п‚· Bidirectional flow is common in healthy babies in the first 12 hours but changes to pure left-to-right when aortic pressures become higher than pulmonary pressures. Other parameters are reserved for more specialist evaluation and include п‚· TPV/RVET ratio п‚· RPEP/RVET ratio п‚· IVRT (from TV annulus tissue Doppler) Cardiac function and output: п‚· There may be enlargement of the RV and RA, as well as the main pulmonary artery. п‚· There may be flattening (RV:LV pressure >0.5) or even bowing (RV:LV pressure ≥1.0) of the interventricular septum to the left as RV pressure rises. п‚· As cardiac output is dependent on venous return to the RA and LA, cardiac output (both RVO and LVO) is frequently reduced with PPHN. Severe PPHN may be associated with LVO below 100ml/kg/min (normal 150-300ml/kg/min) п‚· Quantitative assessment of cardiac function may assist with decisions and assessments of the roles of inotropes and inhaled nitric oxide. 102 п‚· If the LA and LV appear under-filled, it is critical to exclude TAPVD. Demonstration of a left-to-right shunt at atrial level essentially excludes TAPVD. Aims of Management Lower pulmonary vascular resistance Maintain systemic blood pressure Reverse right-to-left shunting Improve arteriolar oxygen saturation and oxygen delivery to the tissues Minimise barotrauma Oxygen and Ventilation Oxygen: Always start with 100% oxygen and reduce the FiO2, rather than starting on 25% and increasing. In the short term there is no risk to a term baby using such measures. Ventilation: Ventilate to achieve adequate lung expansion and aim for normal pCO 2 (5-7 kPa), normal PO 2 (7-12 kPa, if this can be achieved) and normal pH (7.40-7.45). Use conventional ventilation initially. Avoid hyperventilation, as hypocapnia has been shown to increase the risk of long-term neurological disability and in particular sensori-neural deafness. Consider early surfactant, as it can reduce the requirement for ECMO. If conventional ventilation is failing, HFOV should be used to achieve adequate lung recruitment. Combination of HFOV and iNO was the most effective therapy for infants who failed to respond to either one. HFOV may be valuable in establishing adequate lung volumes such that iNO therapy may then be efficacious.2,3 Ensure adequate analgesia, sedation, muscle relaxation Many babies with PPHN are very unstable. Consider early use of narcotic infusions (morphine/fentanyl) and midazolam for analgesia and sedation. Muscle relaxation (regular pancuronium or vecuronium infusion) may be necessary to gain initial control in very vigorous babies who are not adequately sedated with narcotics and are fighting the ventilator. Maintain systemic BP Invasive blood pressure monitoring is mandatory. Myocardial function is frequently poor, despite reasonable blood pressures. Aim to keep the mean arterial pressures above 50mm Hg in term infants. Use volume (initially normal saline) and dopamine and/or dobutamine. Adrenaline infusion may be indicated if there is severe myocardial dysfunction or hypotension. Milrinone (a phosphodiesterase III inhibitor) may be a useful adjunctive inotrope as it potentiates the effects of iNO, causes pulmonary vasodilatation and improves diastolic function. There have been reported association of IVH in patients requiring milrinone and larger trials are needed to clarify risk benefit ratio.4,5 103 Pulmonary Vasodilators Inhaled nitric oxide (iNO) iNO is the first pulmonary vasodilator of choice. Cochrane review has shown that use of iNO in PPHN decreases oxygen index and need for ECMO significantly6. It should be started at 20ppm can be added to conventional ventilation or HFOV. Methaemoglobin and Nitrogen dioxide (NO2) levels should be monitored during administration of iNO Prostacyclin Prostacyclin acts to elevate cellular cAMP levels. It causes vasodilatation of both pulmonary and systemic circulation and hence systemic hypotension may be a problem. Continuous infusion of 520 nanograms/k/min can be used if systemic BP is high enough. Inhaled Prostacyclin (two small studies have shown improvement in oxygenation at doses of 20-50ng/kg/min) may also be used with less systemic hypotensive effect.7,8 Sildenafil It has been shown to be effective in decreasing oxygen index in a randomised controlled trial as well as in observational studies in the treatment of PPHN. 9,10,11 (For dosing see Sildenafil protocol below) Magnesium It has been used as a pulmonary vasodilator12. Toxicity appears low and the effects on systemic circulation are limited unless used in high dose in the presence of myocardial ischaemia. Aim plasma levels 2-4mmol/l. Adenosine for PPHN It acts via adenosine receptors on endothelium, to elevate intracellular cAMP, causing smooth muscle relaxation. Continuous infusion of 25-50mcg/k/min has been used in small studies with success.13,14 ECMO ECMO is used as last resort if above therapies fails to achieve adequate oxygenation (Oxygen Index >40). A policy of using ECMO in mature infants with severe but potentially reversible respiratory failure results in significantly improved survival without increased risk of severe disability amongst survivors. For babies with diaphragmatic hernia ECMO offers short term benefits but the overall effect of employing ECMO in this group is not clear.15 Sildenafil Dose Protocol Because of cost advantages, sildenafil is being used increasingly, particularly in the newborn period (e.g. PPHN amd chronic lung disease patients). It should be instituted under consultant direction as follows: (GOS protocol, unpublished) Starting dose: 0.2 mg/kg/dose 6 hourly Reassess in 24-48 hours Increase sildenafil dose to 0.5 mg/kg/dose 6 hourly Repeat echo assessment Increase dose to 0.5 mg/kg/dose 4 hourly Maximum dose up to 1 mg/kg/dose 4 hourly (doses of up to 2 mg/kg/dose have been used in some patients) References: 1. Skinner J, Alverson D, Hunter S (eds). Echocardiography for the Neonatologist. Churchill Livingstone 2000 2. Kinsella JP, Truog WE, Walsh WF, Goldberg RN, Bancalari E, Mayock DE, Redding GJ, deLemos RA, Sardesai S, McCurnin DC, et al. Randomized, multicenter trial of inhaled nitric oxide and high-frequency oscillatory ventilation in severe, persistent pulmonary hypertension of the newborn. J Pediatr 1997; 131:55-62. 3. Clark RH, Yoder BA, Sell MS. Prospective, randomized comparison of high-frequency oscillation and conventional ventilation in candidates for extracorporeal membrane oxygenation. J Pediatr 1994; 124:447-54. 104 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. McNamara PJ, Laique F, Muang-In S, Whyte HE. Milrinone improves oxygenation in neonates with severe persistent pulmonary hypertension of the newborn. J Crit Care. 2006 Jun;21(2):217-22. Bassler D, Choong K, McNamara P, Kirpalani H. Neonatal persistent pulmonary hypertension treated with milrinone: four case reports. Biol Neonate. 2006; 89(1):1-5. Epub 2005 Sep 8. Finer NN, Barrington KJ. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Rev. 2006 Oct Chotigeat U, Jaratwashirakul S Inhaled iloprost for severe persistent pulmonary hypertension of the newborn J Med Assoc Thai. 2007 Jan; 90(1):167-70. De Luca D, Zecca E, Piastra M, Romagnoli C Iloprost as 'rescue' therapy for pulmonary hypertension of the neonate. Paediatr Anaesth. 2007 Apr; 17(4):394-5. Ho JJ, Rasa G. Magnesium sulfate for persistent pulmonary hypertension of the newborn. Cochrane Database Syst Rev. 2007 Jul 18;(3) Shah PS, Ohlsson A. Sildenafil for pulmonary hypertension in neonates. Cochrane Database Syst Rev. 2007 Jul 18 ;( 3):CD005494. Baquero H, Soliz A, Neira F, Venegas ME, Sola A. Oral Sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded study. Pediatrics. 2007 Jan; 119(1):215-6; author reply 216 W D Carroll, R Dhillon Sildenafil as a treatment for pulmonary hypertension Arch. Dis. Child. 2003;88;827-828 Ng C, Franklin O, Vaidya M, Pierce C, Petros A. Adenosine infusion for the management of persistent pulmonary hypertension of the newborn. Pediatr Crit Care Med. 2004 Jan; 5(1):10-3. Konduri GG, Garcia DC, Kazzi NJ, Shankaran S. Adenosine infusion improves oxygenation in term infants with respiratory failure. Pediatrics. 1996 Dec; 98(6 Pt 1):1224-5. Elbourne D, Field D, Mugford M. Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database of Systematic Reviews 2002 6.20 Rheumatic Fever Features (major criteria shown in bold, minor criteria shown in italics) п‚· Immune-mediated sequel of group A strep infection (1-4 wk) п‚· Pallor, malaise, fatigue п‚· Arthritis (70%)- subsides within weeks п‚· Carditis (50%)- may result in permanent valvar or myocardial damage п‚· Chorea (15%)- subsides п‚· Erythma marginatum rash (10%) – truncal non-pruritic п‚· Subcutaneous nodules (5%) п‚· Fever п‚· Arthralgia п‚· Prolonged PR interval п‚· Elevated acute phase reactants 6.20.1 Diagnostic criteria Requires evidence of streptococcal infection and 2 major or 1 major and 2 minor criteria Management п‚· Eradicate organism (oral penicillin or amoxicillin for 10 days) п‚· Bed rest (varying duration depending on degree of carditis) п‚· Anti-inflammatory therapy (aspirin В± prednisolone [if carditis]) Prevention of Recurrence – AHA recommendations The AHA has given the following recommendation for secondary prevention and the duration of antibiotic prophylaxis: 105 6.20.2 Secondary Prevention of Rheumatic Fever (Recurrent Attacks) Agent Dose Benzathine penicillin G 600 000 U for children 27 kg (60 lb), 1 200 000 U for those >27 kg (60 lb) every 4 wk* Penicillin V 250 mg twice daily Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb), 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Macrolide or azalide Variable Mode Rating IM IA Oral Oral IB IB Oral IC Rating indicates classification of recommendation and level of evidence (eg, IA indicates class I, LOE A). *In high-risk situations, administration every 3 weeks is justified and recommended. 6.20.3 Duration of Secondary Rheumatic Fever Prophylaxis Category Rheumatic fever with carditis and residual heart disease (persistent valvular disease*) Rheumatic fever with carditis but no residual heart disease (no valvular disease*) Rheumatic fever without carditis Duration After Last Attack Rating 10 years or until 40 years of age (whichever is longer), sometimes lifelong prophylaxis (see text) 10 years or until 21 years of age (whichever is longer) IC 5 years or until 21 years of age (whichever is longer) IC IC Rating indicates classification of recommendation and level of evidence (eg, IC indicates class I, LOE C). *Clinical or echocardiographic evidence. Ref: Circulation. 2009;119:1541-1551 6.21 RSV Infection in Cardiac Patients Respiratory syncytial virus is the most common cause of bronchiolitis and pneumonia among infants and children under 1 year of age. The illness generally begins with coryzal symptoms, progressing to cough and sometimes wheezing. During their first RSV infection, between 25% and 40% of infants and young children have signs or symptoms of bronchiolitis or pneumonia, and 0.5% to 2% require hospitalization. Most children recover from illness in 8 to 15 days. The illness can be severe in cardiac babies, particularly those with Lв†’R shunt or with pulmonary hypertension. 106 In 2010 the Joint Committee for Vaccinations and Immunisation recommended that the following should receive passive immunization with palivizumab: п‚· Infants under 6 months of age who are born prematurely with serious heart disease, and п‚· Children under 2 who need long-term ventilation and who have serious heart disease. The committee does not recommend routine RSV prophylaxis for cardiac babies. An RSV information sheet is available on the shared directory “Info sheet” section). It should be given to the parents of all infants with haemodynamically significant congenital heart disease and the precautions apply during the baby’s first RSV season (as a minimum). 6.22 Screening for Cardiac Disease (genetic, familial, etc) Causes of familial cardiac disease include: п‚· Heart muscle disease o Arrhythmogenic right ventricular dysplasia (ARVC) o Dilated cardiomyopathy (DCM) o Duchenne muscular dystrophy and other dystrophin problems o Hypertrophic cardiomyopathy (HCM) o Post-chemotherapy screening п‚· Heart rhythm problems o Brugada syndrome o Long QT syndrome (LQTS) п‚· Heart structure problems o Ehlers-Danlos syndrome o Marfan syndrome o Turner syndrome 6.22.1 Heart muscle disease ARVC screening In first degree relative (parent or sibling) – assume 50% risk of inheriting the gene: п‚· Ensure involvement of medical genetics (consider predictive testing if available) п‚· Full history and examination, including family history п‚· ECG (looking for epsilon wave in V1), SAECG п‚· Echo (looking for RV dilatation, reduced RV function, RV thinning – LV can rarely be involved) п‚· Consider need for MRI scanning п‚· Annual screening, particularly during adolescence п‚· Transfer to adult cardiology age 16-18 If features of ARVC noted, or if presymptomatic genetic diagnosis is made: п‚· For annual risk factor assessment, including Holter monitoring (looking for ventricular arrhythmia) and exercise test (exercise-induced arrhythmias) п‚· Consider need for EPS/ICD 107 п‚· Discuss with / transfer to adult cardiology at age 16-18. ARVC in more distant relative: п‚· Ensure involvement of medical genetics п‚· Full history and examination п‚· ECG, echo п‚· If normal, it may be appropriate to discharge the patient (consider the need to screen again at age 11-12 and 15-17) п‚· If abnormal features noted, see above – regular screening needed with transfer to adult cardiology beyond age 16-18 Dilated cardiomyopathy screening DCM in first degree relative (parent or sibling) – assume 50% risk of inheriting the gene, although not all DCM has a genetic origin: п‚· Ensure involvement of medical genetics (consider predictive testing if available) п‚· Full history and examination, including family history п‚· ECG (looking for LVH, ST/T changes) п‚· Echo (looking for increased LV size, or abnormal function on M-mode and tissue Doppler) п‚· Annual screening, particularly during adolescence п‚· Discuss with / transfer to adult cardiology at age 16-18. If features of DCM noted, or if presymptomatic genetic diagnosis is made: п‚· Holter and exercise test assessment п‚· Give advice about athletic participation (see separate section) п‚· Consider the need for drug or device intervention (CRT/ICD) п‚· Discuss with / transfer to adult cardiology at age 16-18. DCM in more distant relative: п‚· Ensure involvement of medical genetics п‚· Full history and examination п‚· ECG, echo п‚· If normal, it may be appropriate to discharge the patient(consider the need to screen again at age 11-12 and 15-17) п‚· If abnormal features noted, see above – regular screening needed with transfer to adult cardiology beyond age 16-18 Duchenne Muscular Dystrophy Screening Most boys with DMD will develop cardiac dysfunction as they mature. Use of noninvasive ventilation has improved respiratory outcomes so most deaths are now due to cardiac complications. 108 “Infantile onset” DMD (group A in figure above) have the following features: п‚· Early significant motor and intellectual impairment presenting <2 years of age п‚· High CK at presentation п‚· Early incidence of cardiac dysfunction These patients need annual screening from diagnosis. For other patients with “typical” DMD, he following is a practical approach to screening of myocardial dysfunction in this group of patients: п‚· Referral for baseline CVS assessment at 4-6 years of age п‚· Repeat CVS assessment including ECG and echo at loss of ambulation п‚· 2 yearly assessments if no problem is detected п‚· After age 10-12, annual assessments depending on the clinical picture; if reduced cardiac function or pulmonary hypertension noted, more frequent follow-up may be needed п‚· CVS screening should be undertaken before any elective surgery (data <6 months old are acceptable) п‚· Intervention with ACE inhibitors (В± пЃў-blocker) if FS <25% (EF<50%) or if LVDD > 2 Z-scores from mean п‚· Discuss with / transfer to adult cardiology at age 16-18. References: PLOSone 2009;4:e4347 and http://www.treat-nmd.eu/userfiles/The_diagnosis_and_management_of_DMD_Lancet_complete.pdf Pediatrics 2005;116 (6):1569-1573 Becker muscular dystrophy п‚· Referral for baseline CVS assessment at diagnosis п‚· Repeat CVS assessment including ECG and echo 3-5 yearly assessments if no problem is detected п‚· If reduced cardiac function noted, more frequent follow-up may be needed п‚· Intervention with ACE inhibitors (В± пЃў-blocker) if FS <25% or if LVDD > 2 Zscores from the mean п‚· Discuss with / transfer to adult cardiology at age 16-18. References: Pediatrics 2005;116 (6):1569-1573 Neuromuscular Disorders 2003;13:166-72 Screening of female first-degree relatives of males with Duchenne or Becker muscular dystrophy п‚· Known carriers of the dystrophin gene should be made aware of the risk of developing cardiomyopathy and educated about the signs and symptoms of heart failure (the risk is ~10%). They should undergo CVS screening to assess 109 п‚· LV function at the time of confirmation of the diagnosis and 3 to 5 yearly after that depending on the clinical findings. Abnormalities in LV function are more likely to develop after late adolescence and CVS screening needs to continue into adult life. Female first-degree relatives of males with Duchenne muscular dystrophy whose gene status is not known should only undergo CVS screening if there are concerning clinical or laboratory features (e.g. muscle weakness, elevated CK levels). Consideration can be given to screen for CVS abnormalities from late adolescence onwards, although screening is likely to be cost-effective only in known carriers. Treatment of cardiac disease in carriers is similar to that outlined for boys with DMD or BMD. (Pediatrics 2005;116 (6):1569-1573, Neuromuscular Disorders 2003;13:166-72) Hypertrophic cardiomyopathy screening HCM in first degree relative (parent or sibling) – assume 50% risk of inheriting the gene: п‚· Ensure involvement of medical genetics (consider predictive testing if available) п‚· Full history and examination, including family history п‚· ECG (looking for LVH, ST/T changes) п‚· Echo (looking for septal or apical hypertrophy, function, LVOT velocity) п‚· Annual screening, particularly during adolescence п‚· Discuss with / transfer to adult cardiology at age 16-18. If features of HCM noted, or if presymptomatic genetic diagnosis is made: п‚· For annual risk factor assessment, including 48 hour Holter (looking for nonsustained VT) and exercise test (looking for abnormal BP response, exerciseinduced arrhythmias) п‚· Give advice about athletic participation (see separate section) п‚· Consider need for ICD п‚· Discuss with / transfer to adult cardiology at age 16-18. HCM in more distant relative: п‚· Ensure involvement of medical genetics п‚· Full history and examination п‚· ECG, echo п‚· If normal, it may be appropriate to discharge the patient(consider the need to screen again at age 11-12 and 15-17) п‚· If abnormal features noted, see above – regular screening needed with transfer to adult cardiology beyond age 16-18 Screening post chemotherapy exposure CCLG guidelines define abnormal LV function as having an LV fractional shortening of ≤28%. Drugs associated with risk of developing cardiac dysfunction: п‚· Daunorubicin } п‚· Doxorubicin } Anthracycline group п‚· Epirubicin } of drugs 110 п‚· phosphamide Radiotherapy to thorax, thoracic spine, or mediastinum (including left flank and total body irradiation) All patients who have received anthracyclines require an echocardiogram following treatment. п‚· Echocardiogram performed 1-3 months after last planned dose of anthracycline; if echo shows FS>28%, repeat echo 5 yearly + at beginning and end of pubertal growth spurt. п‚· Echocardiogram performed later than 3 months post last anthracycline exposure – if echo shows FS>28%, repeat echo 3 yearly + at beginning and end of pubertal growth spurt. Patients at high risk and in whom more frequent surveillance may be warranted: п‚· Those in whom early anthracycline toxicity is noted п‚· Total anthracycline dose of >250 mg/m2 п‚· Combination of anthracycline and radiotherapy п‚· Patients on growth hormone or sex steroid therapy п‚· Patients with CHD п‚· Pre- and during pregnancy Transition to adult services: п‚· Those with abnormal LV function should be referred to adult cardiology age 16-18 for ongoing management п‚· Those with normal LV function o Females should be advised to have CVS assessment prior to planned pregnancy o Otherwise, repeat assessment via local adult echo services should be advised (referral by GP or oncology team) 6.22.2 Heart Rhythm Brugada Syndrome Screening In first degree relative (assume 50% risk): п‚· Ensure involvement of medical genetics п‚· Full history and examination, including family history п‚· ECG (looking for characteristic changes) п‚· Echo (to rule out features of ARVC – the ECG changes can overlap and there may be some diagnostic confusion) п‚· Consider need for ajmaline/flecainide challenge in children > age 8-10 (mandatory if symptomatic) п‚· Consider need for VStim study (if +ve ECG or drug challenge, or if symptomatic) п‚· Consider need for ICD 111 п‚· п‚· п‚· Annual screening, but if features of Brugada syndrome noted, or if presymptomatic genetic diagnosis is made, for 6 monthly review Give advice regarding athletic participation Discuss with / transfer to adult cardiology at age 16-18. Brugada syndrome in more distant relative: п‚· Ensure involvement of medical genetics п‚· Full history and examination п‚· ECG, echo п‚· If normal, it may be appropriate to discharge the patient (consider the need to screen again at age 11-12 and 15-17) п‚· If abnormal features noted, see above – regular screening needed with transfer to adult cardiology beyond age 16-18 Long QT Syndrome Screening LQTS in first degree relative (assume 50% risk): п‚· Ensure involvement of medical genetics п‚· Full history and examination, including family history п‚· ECG (looking for characteristic changes), consider echo п‚· Consider need for epinephrine challenge in children > age 8-10 (mandatory if symptomatic) п‚· Consider need for VStim study (if +ve ECG or drug challenge, or if symptomatic) п‚· Consider need for intervention (пЃў-blockers, ICD В± pacing) п‚· Annual screening, but if features of LQTS syndrome noted, or if presymptomatic genetic diagnosis is made, for 6 monthly review п‚· Give advice regarding athletic participation п‚· Discuss with / transfer to adult cardiology at age 16-18. Long QT syndrome in more distant relative: п‚· Ensure involvement of medical genetics п‚· Full history and examination п‚· ECG п‚· If normal, it may be appropriate to discharge the patient (consider the need to screen again at age 11-12 and 15-17) п‚· If abnormal features noted, see above – regular screening needed with transfer to adult cardiology beyond age 16-18 6.22.3 Heart Structure Ehler’s Danlos Syndrome Screening Patients with benign forms of EDS may rarely have mitral valve prolapse and regurgitation, but intervention is seldom needed. Long-term CVS screening is rarely indicated. EDS type IV (vascular EDS) is a rare autosomal dominant condition with a high cardiovascular mortality due to vessel rupture. Children of adults with EDS IV need to be assessed by medical genetics and if the condition is suspected in the child, they should undergo repeated cardiovascular assessment through to adult life. 112 Marfan Syndrome Screening If MFS in first degree relative (assume 50% risk): п‚· Refer to paediatric Marfan syndrome clinic п‚· Full history and clinical examination п‚· ECG, echo (aortic enlargement, mitral or tricuspid valve prolapse, MR) п‚· Consider need for fibrillin mutation analysis п‚· 6-12 monthly review п‚· Consider need for пЃў-blockers or other vasoactive drugs п‚· Give advice about athletic participation, contraception, pregnancy (where relevant) п‚· Transfer to GUCH service age 16-18 Turner Syndrome Screening 50% of Turner patients have aortic enlargement, irrespective of presence or absence of a bicuspid aortic valve (Cardiol Young 2009;19:568-72). The risk of aortic dissection is 6x the general population (but is lower than Marfan patients). Best practise: п‚· Review 1-3 yearly, depending on findings п‚· Full history and clinical examination п‚· ECG and echo – plot aortic measurements on centile charts п‚· Consider пЃў-blockage if aorta >95th percentile for size п‚· Refer for surgical aortic root replacement if aorta >55 mm in an adult (or equivalent centile in a child) 113 6.23 Supraventricular Tachycardia Diagnosis п‚· Inappropriate tachycardia (usually > 180-220/min) п‚· Narrow QRS complex п‚· No beat-to-beat variation п‚· Absent/abnormal P-waves Treatment algorithm - Inform relevant consultant Continuous ECG monitoring Haemodynamically stable? Shocked? See algorithm for shocked SVT (below) Diving Reflex: Facial immersion in iced water for 5 seconds (neonates only) Facial cooling (ice pack on face) Carotid sinus massage Valsalva manouvre RECORD RHYTHM STRIP OR 12-LEAD DURING INTERVENTION Unresponsive? Adenosine – 150 micrograms/kg IV via large vein using 3-way tap Flush with saline (5ml if < 1yr, 10ml if >1 yr) Document location of IV access Unresponsive? Adenosine – 300 micrograms/kg IV via large vein using 3-way tap Flush with saline (5ml if < 1yr, 10ml if >1 yr) Unresponsive? Adenosine - 300 micrograms/kg IV via large vein using 3-way tap Flush with saline (5ml if < 1yr, 10ml if >1 yr) Responsive? Start prophylaxis Unresponsive? Discuss with consultant cardiologist Consider IV amiodarone, 5 mg/kg given through largest possible vein over 60-120 minutes. After infusion, if still in SVT, give further dose of adenosine, 300 microgram/kg. 114 SVT – haemodynamically unstable (Shocked SVT) Oxygen, Airway, Breathing CONSIDER NEED FOR SEDATION OR GENERAL ANAESTHESIA Synchronous DC Cardioversion, 1 J/kg Unresponsive? Synchronous DC Cardioversion, 2 J/kg Unresponsive? Consider IV Sodium Bicarbonate between shocks Responsive? Unresponsive? Start prophylaxis Discuss with cardiologist, consider IV amiodarone (5 mg/kg over 60-120 min) SVT Prophylaxis ECG WPW No WPW Check no obstructive heart lesion, ensure good cardiac function Flecainide 1 to 3 mg/kg/dose twice daily (safest to start as an inpatient and keep in for 4 to 6 hours) <1 yr: Propranolol 0.5 mg/kg/dose tds >1 yr: Atenolol 1 mg/kg/dose od, or 0.5 to 1 mg/kg/dose bd Consider flecainide (see below) Trough flecainide levels (6 hours post-dose, e.g. dose given 8 AM, check level 2 PM) & 12-lead ECG after 5-7 days. Consider toxicity if level is elevated or if QRS duration increases >25% from baseline. Some individuals may require combination therapies. Effective, safe combinations are: Flecainide – Atenolol (or propranolol) Flecainide – Digoxin Sotalol – Digoxin Atenolol (or propranolol) – Digoxin 115 6.23.1 SVT in the Fetus Fetal SVT can be treated by administering anti-arrhythmic drugs to the mother. For cases of sustained fetal tachycardia: п‚· Perform fetal echo to look for structural heart disease, ventricular function, AV valve regurgitation, evidence of hydrops п‚· If treatment is intended, ensure no heart disease in the mother, check maternal ECG and electrolytes; consider need for maternal echocardiogram п‚· Treatment No hydrops: Flecainide 100 mg tds + Digoxin 0.25 mg tds Hydrops: Flecainide 100 mg tds + Digoxin 0.5 mg/ 0.25 mg / 0.25 mg in first 24 hours, then 0.25 mg tds п‚· Daily fetal heart assessment, daily maternal ECG for at least 48 hours п‚· Reduce medications to bd if o Tachycardia settles o Maternal QRS duration increases to >25% of baseline o PR >240 msec o QT >480 msec Check drug levels, elctrolytes and ECG 5 days after starting therapy (trough drug levels). The patient will require close follow-up between the fetal cardiac and fetal medicine teams. 6.24 Transplantation The main considerations are immunosupression (maintenance therapy and monitoring of drug levels), prevention and treatment of opportunistic infections and detection of rejection. Close liaison with the transplant centre is essential. Routine Investigations When reviewing a patient check the following: п‚· BP п‚· Urinalysis п‚· Weight п‚· FBC п‚· U&E, creatinine, LFT п‚· Trough ciclosporin level п‚· ECG summated voltages (add peak to peak (R-S or Q-R) voltages in leads I, II, III, V1 & V6). п‚· Echocardiogram (the need for this reduces with time - liaise with the consultant). Record measurements on the flow sheet in the case record notes and communicate significant changes to the transplant centre. Immunosupression Tacrolimus or Neoral (ciclosporin A) and mycophenolate mofetil or azathiaprine are the most commonly prescribed drugs. Ciclosporin (cyclosporin), a calcineurin inhibitor, is a potent immunosuppressant which is virtually non-myelotoxic but markedly nephrotoxic. 116 Tacrolimus is a calcineurin inhibitor. Although not chemically related to ciclosporin it has a similar mode of action and side-effects, but the incidence of neurotoxicity and nephrotoxicity appears to be greater; cardiomyopathy has also been reported. Disturbance of glucose metabolism also appears to be significant; hypertrichosis appears to be less of a problem than with ciclosporin. Azathioprine is widely used for transplant recipients and it is also used to treat a number of auto-immune conditions, usually when corticosteroid therapy alone provides inadequate control. It is metabolised to mercaptopurine, and doses should be reduced when allopurinol is given concurrently. Blood tests and monitoring for signs of myelosuppression are essential in long-term treatment with azathioprine. Mycophenolate mofetil (MMF) is metabolised to mycophenolic acid which has a more selective mode of action than azathioprine. It is licensed for the prophylaxis of acute rejection in renal or cardiac transplantation when used in combination with ciclosporin and corticosteroids. There is evidence that compared with similar regimens incorporating azathioprine, mycophenolate mofetil reduces the risk of acute rejection episodes; the risk of opportunistic infections (particularly due to tissueinvasive cytomegalovirus) and the occurrence of blood disorders such as leucopenia may be higher. Drug levels and FBC are used to monitor efficacy of immunosupression. Be guided by the transplant centre. General advice Immunisations - Live vaccines should not be used. Inactive vaccines are suitable (e.g. Salk polio vaccine). Siblings should receive all immunisations including MMR. If a patient has contact with chicken pox, oral Aciclovir +/- Zoster immunoglobulin (HZIG) should be given. Liaise with the PHLS Virologists. Endocarditis prophylaxis - NICE guidance should be followed (not given routinely). Diet should be as normal as possible but the following should be avoided due to the risk of bacterial colonisation: undercooked chicken or turkey, shellfish, raw eggs, live yoghurt, blue or soft cheeses, sheep or goats’ milk, unpasteurised milk or cream, and possibly pre-prepared salads. Avoid grapefruit juice as mentioned above. Transplant Rejection Symptoms/Signs: п‚· Fever, malaise, anorexia, vomiting п‚· Breathlessness п‚· Hepatosplenomegaly п‚· Gallop rhythm Investigations: п‚· WBC, CRP п‚· 25% or more reduction in ECG summated voltages п‚· CXR shows cardiomegaly п‚· Echocardiogram changes include п‚Їfractional shortening, ejection fraction, п‚LVDD and dimensions of LVPW and IVS, and new pericardial effusion 117 п‚· Endomyocardial biopsy may show histological changes and can be graded according to severity Treatment: Liase with transplant team п‚· Mild rejection - prednisolone 1 mg/kg/day п‚· Acute severe rejection - intravenous Methylprednisolone. Monitor BP every 15 minutes for first hour. Give Ranitidine to prevent gastric ulceration. High dose steroids increase Ciclosporin levels Opportunistic infections Organism Prevention Candida or aspergillus nystatin Pneumocystis Herpes simplex CMV co-trimoxazole oral aciclovir none available Treatment amphotericin (caution – renal impairment), ambisome fluconazole high dose intravenous co-trimoxazole intravenous acyclovir intravenous ganciclovir Lymphoproliferative Disease EBV-related lymphoproliferative disease is a serious and not uncommon (20%) complication of paediatric cardiac transplantation. It presents with non-specific lymphadenopathy. Tonsillar enlargement is not uncommon. There is a UKCCSG protocol for the investigation and management of this disorder – liaise with the local oncology team and the transplant centre. 118 INDEX "open door" policy, 31 “captopril cough”, 22 “Infantile onset” DMD, 109 “Targeted” therapy in PAH, 100 “typical” DMD, 109 ACE inhibitor protocol, 22 ACE inhibitors, 22, 46, 63, 68 Acute Myocarditis, 75 Adenosine for PPHN, 104 Adenosine Stress Test by MRI, 29 Adjusting LMW heparin in children, 52 Admissions for MRI or CT, 28 Ajmaline, 27 Ambulatory BP Monitoring, 45 ambulatory BP values, 46 Ambulatory ECG Monitoring, 45 amiodarone, 114, 115 angiotensin II antagonist, 22 Annual And Study Leave, 14 anthracyclines, 111 Anticoagulation Control, 49 Anti-platelet therapy, 49 Anti-platelet Therapy, 49 Aortic regurgitation, 76 Aortic stenosis and bicuspid aortic valve, 77 ARVC screening, 107 aspirin for a B-T shunt, 49 Asplenia, 57 Atypical Kawasaki Disease, 86 Audit and Research, 14 Balloon Atrial Septostomy (BAS), 33 Bazett formula, 41 Becker muscular dystrophy, 109 Bethesda Conference, 74 bodily collision, 74 Bosentan, 100 Bristol Children’s Hospital, 12 Bristol E-Mail Addresses, 12 bronchiolitis, 106 Bruce protocols, 43 Brugada syndrome, 27, 83, 112 Brugada Syndrome Screening, 111 candesartan, 23 Cardiac Failure, 59 Cardiac Liaison Nurses, 11, 53 Cardiac Nuclear Scanning, 48 cardiac output, 61 cardiac source of embolism, 42 cardiac tamponade, 34 Cardiac tamponade, 59 Cardiobase out of hours, 31 CardiobaseВ®, 15 cardiomyopathy, 67 Cardiomyopathy, 63 Cardiomyopathy - Investigations, 64 Carvedilol protocol, 23 Casual Ward Attenders, 30 Catecholamines table, 63 Categories of Admission and Review, 16 CCLG guidelines, 110 Cessation of warfarin for surgical or invasive procedure, 56 Chest Pain, 68 Chloral hydrate, 21 chromosome 22 microdeletion, 58 Classification of Competitive Exercise, 74 clopidogrel, 49 coagucheck, 53 Coarctation of the aorta, 77 Commencing anticoagulation, 51 Congenital Complete Heart Block, 82 Congenital coronary anomalies, 75 Congenitally corrected transposition of the great arteries, 78 Correspondence Headings, 15, 16 Costo-chondritis, 68 CT scan, 28 Daily Timetable, 17 Day Case Admissions, 21 DC Cardioversion, 33 Death of a Patient, 39 DiGeorge syndrome, 58 Dilated cardiomyopathy screening, 108 Discharge Checklist, 39 Discharges Following Cardiac Surgery, 39 domperidone, 90 double discordant TGA, 78 Drug Concentrations for Infusions, 93 Drug interactions, 57, 93 119 high-impact contact sports, 73 Holter monitoring, 45 Home Oxygen Therapy, 90 Hypercyanotic Spells, 85 Hypertrophic cardiomyopathy, 69, 72 Hypertrophic Cardiomyopathy, 75 Hypertrophic cardiomyopathy screening, 110 idiopathic pulmonary arterial hypertension, 98 Immunodeficiency, 57 Immunosupression, 116 Infective Endocarditis, 70 Influenza vaccination, 58 Inhaled nitric oxide, 104 iNO, 104 Inpatient referral checklist, 37 Inpatient Referrals, 36 inpatient report, 37 INR Protocol, 53 INR Sampling, 53 Interventional Cardiac Catheterisation, 32 intracardiac thrombus, 67 Isotope Scans, 48 IVIG, 87 Kawasaki disease, 34, 49, 69 Kawasaki Disease, 76, 86 Knee/chest position, 85 Loading with warfarin, 52 Long QT syndrome, 72, 112 Long QT Syndrome, 82 Long QT Syndrome Screening, 112 losartan, 22 low molecular weight heparin, 51 LQTS type 1, 73 Lung perfusion scan, 48 Lymphoproliferative Disease, 118 Magnesium, 104 Marfan syndrome, 78 Marfan Syndrome Screening, 113 Maximising Calories, 89 Medical Notes, 15 Medication Errors, 92 Midazolam, 21 Midodrine, 84 Milrinone, 63 Mitral regurgitation, 79 Monitoring Chart for Adenosine Stress Test, 30 Drug Monitoring, 40 Duchenne Muscular Dystrophy Screening, 108 Ebstein’s malformation, 78 ECG Interpretation, 41 Echo Clinic, 18 echo under sedation, 21 Echocardiogram, 42 Echocardiogram under sedation, 21 Echocardiographic assessment of pulmonary hypertension, 102 ECMO, 104 EDS type IV, 112 Ehler’s Danlos syndrome, 112 Eisenmenger syndrome, 78, 99 Electrocardiogram, 40 E-Mail Addresses, 7 Emergency admissions, 31 Endocarditis, 70 Endocarditis prophylaxis, 71, 117 Endocarditis Prophylaxis, 70 Enhanced anticoagulation, 56 enterovirus myocarditis, 67 Epinephrine challenge for LQTS, 24 Event recorders, 45 Exercise, 72 Exercise advice by lesion, 74 Exercise Test, 43 Exercise test protocol, 44 exercise-induced supraventricular tachycardia, 81 Faltering Growth, 89 familial aortic aneurysm, 79 familial pulmonary arterial hypertension, 98 FASTING ON A ОІ-BLOCKER, 95 Fetal SVT, 116 Fits, Faints and Funny Turns, 82 Flecainide, 28 flecainide levels, 115 Fludrocortisone, 84 Fontan operation, 80 Framingham formula, 42 Frank-Starling mechanism, 60 Gastro-Oesophageal Reflux, 89 Graft-versus-Host immune reaction, 59 Haemangiomas, 94 Heart failure, 60 heparin, 51, 52, 53, 56, 92, 97 high INR, 53, 54 120 Quick Calculations, 93 Reduced anticoagulant effect, 56 Reflex Syncope, 83 Respiratory syncytial virus, 106 Rheumatic Fever, 105 Routine Admissions, 20 Screening for Cardiac Disease, 107 Screening post chemotherapy exposure, 110 Severity of AS, 77 Shocked SVT , 115 Sildenafil, 101, 104 Sildenafil Dose Protocol, 104 sports participation, 73 Supraventricular Tachycardia, 114 SVT, 81 SVT due to WPW, 81 SVT in the Fetus, 116 SVT Prophylaxis, 115 Teaching, 17 Teaching Topics, 19 Teaching Ward Round, 19 Tetralogy of Fallot, 80 Tilt Test, 47 Transfer Checklist, 38 Transfers to Other Hospitals, 38 Transoesophageal Echocardiography, 28 Transplant Rejection, 117 Transplantation, 116 Turner Syndrome Screening, 113 UHW Clinics, 18 UHW Telephone Numbers, 6 Useful Contact Numbers, 8 vaccination schedule, 58 valsartan, 23 Vasovagal syncope, 83 warfarin, 52, 53, 54, 56, 57, 93, 97 Warfarin dosage table, 55 weight-lifting, 73 Welsh Hospitals, 10 Wound Care, 39 MRI, 28 MRI or CT Scan, 48 MRSA Infection, 88 Musculoskeletal chest pain, 68 Neonatal Admissions, 31 nephrogenic fibrosing sclerosis, 48 Non-Cardiac Admissions, 30 Nurse-Led Clinic, 11 Nutrition, 89 On-Call Arrangements, 14 Outreach Clinics, 18 Oxygen, 90 Paediatric Cardiac Units, 13 Pericardial effusion, 34 pericardiocentesis, 36 Pericardiocentesis, 34, 35 Pericarditis, 69 Phosphodiesterase inhibitors, 63 plastic bronchitis, 96 Pneumococcal vaccine, 58 Post Surgical Transfers, 36 Post-operative arterial switch operation, 80 Post-Operative Patients, 79 PPHN, 101 Premature Atrial Contractions in Newborn Babies, 91 Prescribing, 92 Propranolol for the Treatment of Capillary Haemangiomas, 94 Prostacyclin, 104 prostaglandin E, 32 prosthetic heart valves, 56 Prosthetic valve replacement, 80 Protein-losing enteropathy, 96 Protocol for PGE infusion, 32 pulmonary arterial hypertension associated with CHD, 98 Pulmonary hypertension, 69, 79 Pulmonary Hypertension, 97 Pulmonary valve disease, 79 Pumonary vascular resistance, 98 QT interval, 41 121
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