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Clinical Guidelines
February 2011 to January 2012
TABLE OF CONTENTS ............................................................................................2
GENERAL ADMINISTRATION .......................................................................6
UHW Telephone Numbers ............................................................................6
Consultant Medical Staff and Speciality Doctor ...................................6
Secretarial Staff and Specialist Cardiac Nurses.....................................6
Junior Medical Staff/Trainees................................................................7
Technical and Support Staff...................................................................7
Useful E-Mail Addresses .......................................................................7
Useful Contact Numbers........................................................................8
Welsh Hospitals ...........................................................................................10
Specialist Cardiac Nurses ............................................................................11
Contact Numbers .................................................................................11
Roles and Responsibilities ...................................................................11
Nurse-Led Clinic..................................................................................11
Bristol Children’s Hospital ..........................................................................12
Medical Staff – Paeds ..........................................................................12
Medical Staff – GUCH (Bristol Heart Institute)..................................12
Other Useful Numbers .........................................................................12
Bristol E-Mail Addresses.....................................................................12
UK and Ireland Paediatric Cardiac Units.....................................................13
Annual and Study Leave..............................................................................14
On-Call Arrangements .................................................................................14
Consultant Staff ...................................................................................14
Junior Medical Staff.............................................................................14
Audit and Research ......................................................................................14
Computers and CardiobaseВ® ........................................................................15
1.10 Medical Notes and Correspondence Headings ............................................15
Categories/Definitions of Admissions and Reviews............................16
Correspondence Headings ...................................................................16
DAY TO DAY BUSINESS ................................................................................17
Daily Timetable ...........................................................................................17
UHW Clinics................................................................................................18
2.2.1 General Clinics............................................................................................18
2.2.2 Specialist Clinics.........................................................................................18
2.2.3 Echo Clinic..................................................................................................18
Outreach Clinics...........................................................................................18
Teaching Topics...........................................................................................19
Teaching Ward Round .................................................................................19
ADMISSIONS AND REFERRALS .................................................................20
Routine Admissions .....................................................................................20
Day Case Admissions ..................................................................................21
Echocardiogram under sedation...........................................................21
ACE inhibitor protocol ........................................................................22
Carvedilol protocol ..............................................................................23
Adrenaline/Epinephrine challenge for LQTS ......................................24
Brugada syndrome - Flecainide or Ajmaline Challenge......................27
Admissions for Transoesophageal Echocardiography.................................28
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
Non-Cardiac Admissions and Casual Ward Attenders ................................30
Emergency admissions and procedures .......................................................31
Patients known to the Department .......................................................31
New Referrals ......................................................................................31
Neonatal Admissions ...........................................................................31
3.6.4 Protocol for PGE infusion (NB risk of apnoea) ........................................32
Emergency Interventional Procedures .........................................................32
Balloon Atrial Septostomy ...................................................................33
DC Cardioversion (DCCV)..................................................................33
Pericardiocentesis for cardiac tamponade ............................................34
Post Surgical Transfers (in) .........................................................................36
Inpatient Referrals ........................................................................................36
DISCHARGE AND TRANSFER PROCEDURES.........................................37
General Principles ........................................................................................37
Transfers to Other Hospitals ........................................................................38
Discharges Following Cardiac Surgery .......................................................39
Discharge Checklist .....................................................................................39
Death of a Patient .........................................................................................39
Drug Monitoring and/levels .........................................................................40
Electrocardiogram ........................................................................................40
Basic ECG Interpretation .....................................................................41
Echocardiogram ...........................................................................................42
Echocardiogram to rule out cardiac source of embolism.....................42
Exercise Test ................................................................................................43
Indications for exercise test .................................................................43
Bruce protocols ....................................................................................43
Indications for termination:..................................................................44
Difficulties in interpretation .................................................................44
Helpful tips...........................................................................................44
Ambulatory ECG Monitoring ......................................................................45
Holter monitoring.................................................................................45
Event recorders ....................................................................................45
Ambulatory BP Monitoring .........................................................................45
Tilt Test ........................................................................................................47
MRI or CT Scan...........................................................................................48
Isotope Scans ...............................................................................................48
Cardiac Nuclear Scanning....................................................................48
Lung perfusion scan .............................................................................48
CLINICAL PROBLEMS ..................................................................................49
Anti-platelet Therapy and Anticoagulation .................................................49
Aspirin and Anti-platelet Therapy11 ...................................................49
Devices and Stents ...............................................................................49
Valve replacement ...............................................................................50
Cavopulmonary shunt / Fontan ............................................................50
Other indications ..................................................................................50
Commencing anticoagulation (heparin and warfarin) .........................51
INR Sampling ......................................................................................53
INR Protocol ........................................................................................53
Warfarin dosage table (doses shown in mg) ........................................55
Cessation of warfarin for surgical or invasive procedure ....................56
Factors that influence the efficacy of warfarin ....................................56
Asplenia and Immunodeficiency .................................................................57
Asplenia ...............................................................................................57
DiGeorge syndrome and chromosome 22 microdeletion ....................58
Cardiac Failure .............................................................................................59
Cardiac Tamponade - See section 3.7.2 .......................................................63
Cardiomyopathy ...........................................................................................63
Chest Pain in Children .................................................................................68
Endocarditis and Endocarditis Prophylaxis .................................................70
Infective Endocarditis ..........................................................................70
Endocarditis prophylaxis .....................................................................71
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
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
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
Definition, Classification and WHO Functional Status.......................97
Pathophysiology ...................................................................................98
Treatment of Pulmonary Hypertension................................................99
Persistent Pulmonary Hypertension of the Newborn (PPHN)...........101
6.20 Rheumatic Fever ........................................................................................105
Diagnostic criteria ..............................................................................105
Secondary Prevention of Rheumatic Fever (Recurrent Attacks) .......106
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
Heart muscle disease..........................................................................107
Heart Rhythm.....................................................................................111
Heart Structure ...................................................................................112
6.23 Supraventricular Tachycardia ....................................................................114
SVT in the Fetus ................................................................................116
6.24 Transplantation ..........................................................................................116
INDEX ......................................................................................................................119
UHW Telephone Numbers
Consultant Medical Staff and Speciality Doctor
Dr Victor Ofoe
07623 905 928
07815 510 833
Dr Obed Onuzo
07623 906 018
07815 902 866
Dr Orhan Uzun
07623 906 121
07967 337 319
Dr Dirk G Wilson
07623 905 734
07968 822 824
Dr Peter Groves
07623 905 821
07899 727 937
Dr Navroz Masani
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
Dr Amos Wong
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
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
Other information
Sarah Wooller (Dr Onuzo)
Angela Butters (Dr Uzun)
Suzanne Cornish (Dr Wilson)
Amanda Doyle (Dr Ofoe)
Sarah Grinter (Dr Masani)
Karen McCarthy (Dr Groves)
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-Wed, Fri
Specialist Cardiac Nurse (Area)
Claire Logan (Cardiff and the Vale +
Rhondda Cynon Taff)
07811 197 136
Wendy Williams (Bridgend and West)
07813 922 441
Alison Pearce (Gwent, western Valleys and
07966 461 421
Ann Jermyn (Transition Care 12-19 years)
07980 635 177
Junior Medical Staff/Trainees
Cardiac Registrar
Paediatric Registrar
5391 or
07623 906359
1.1.4 Technical and Support Staff
Viv Booker (Echo)
Bethany Glasser (Dietician)
Tony Bradley (Social Work)
David “Wally” James (Audit, Data and IT
07623 905 607
Mobile via Switch
1.1.5 Useful E-Mail Addresses
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]
Useful Contact Numbers
Adult Cardiac ICU
Anaesthetic Department
B1 (Adult Cardiology)
Biochemistry (Main Dept)
Biochemistry (Emergency)
Blood Bank
Cardiac Day Case Unit
Catheter Laboratory
Clinical Investigation Unit
Children’s Assessment Unit
Coronary Care Unit
Coroner’s Office
Delivery Suite
Dental Surgeon Paeds
Drug Information
ECG (Main Dept, Pacing)
ECG (Inpatient requests)
Exercise/Tilt Test
Fetal Medicine
General ICU
Hand over room General
Hand over room Specialty
Heulwen Ward
Holter (24 hour tape)
IT help desk
Medical Illustration
MRI/CT CD Copying
20 222 111
Other Information
Bleep for out-of-hours samples
Call Lab for urgent processing
Outside line required
Suite 11 OPD (Holters also)
Bleep for out-of-hours samples
Main Desk
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
Personnel (Human resources)
Postnatal Ward
Public Health
SALT team
Sophie Pearson Room
Teenage Cancer Trust
Theatres (Reception)
72 6894
Other Information
NNU HDU 7847
Adult Congenital Clinic
Emergency 2222
Dr’s Office 2973
Drug levels
Out of hours – call extn 8084
Welsh Hospitals
District General Hospital
External Number
Aberdare General Hospital
01685 872 411
Brecon War Memorial Hospital
01874 622 443
Bronglais General Hospital(Aberyswyth)
01970 623 131
Caerphilly Miner’s Hospital
029 2085 1811
(From UHW
Llandough Hospital
dial 72 then
029 2071 1711
Morriston Hospital (Swansea)
01792 702 222
Neath Port Talbot Hospital
01639 862 000
Nevill Hall Hospital (Abergavenny)
01873 732 732
Prince Charles Hospital (Merthyr)
01685 721 721
Princess of Wales Hospital (Bridgend)
01656 752 752
Prince Phillip Hospital (Llanelli)
01554 756 567
Royal Glamorgan Hospital
01443 443 443
Royal Gwent Hospital (Newport)
01633 234 234
Saint David’s Hospital
029 2053 6666
Singleton Hospital (Swansea)
01792 205 666
West Wales General Hospital (Carmarthen)
01267 235 151
Withybush Hospital (Haverfordwest)
01437 764 545
Ysbyty Glan Clwyd (Rhyl)
01745 583 910
Ysbyty Gwynedd (Bangor)
01248 384 384
Add “100” to the WHTN number for Switchboard Operator or direct-dial if you know
the extension
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
Alison Pearce - Part time Th/Fr
(Gwent, western Valleys, Merthyr)
Ann Jermyn (Transition care, age 1219)
07623 905 758
07811 197 136
07623 906 121
07813 922 441
07623 906 297
07966 461 421
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
 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
 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
 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.
Bristol Children’s Hospital
Medical Staff – Paeds
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
Via BRI Switchboard
01179 215 411
01179 276 998
0117 342 8854
(Christine McFadden)
0117 342 5967
Via BRI Switchboard
01179 215 411
01179 276 998
0117 342 6576
0117 342 6575
Other Useful Numbers
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)
Medical Staff – GUCH (Bristol Heart Institute)
Dr Stephanie Curtis
Dr Rob Martin
Dr Gareth Morgan
Dr Graham Stuart
Dr Mark Turner
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
Mr Massimo Caputo
Mr Andrew Parry
Mr Serban Stoica
[email protected]
[email protected]
[email protected]
UK and Ireland Paediatric Cardiac Units
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
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
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
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.
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.
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
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
 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
 Be contemporaneous - not written days later (and if they are written “after the
fact” this should be stated)
 Be capable of being read out in court by the patient’s barrister
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
Day Case Admission
Casual Ward Attender
Outpatient Visit
Inpatient Referral
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
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
Status at time of discharge
Weight at discharge
Discharge medication
Risk of endocarditis Y/N
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
Dictate and “Inpatient Report” on
Inpatient Reports (see Section 3.9)
Date of consultation
Referring Doctor
Reason for referral
Pertinent history/examination
Final diagnosis
Advice given
Copies to __________
Top copy of form to patient notes
Bottom copy for
Copies to __________
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
Communication with parents
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
Daily Timetable
Planning Meeting
Sophie Pearson Room
OU and OCO Clinic
Paeds OPD
CVS Science Meeting
Microbiology and
Paeds Seminar Room or
X-Ray Meeting (Paeds)
KRUF Seminar Room
Nurse-Led Clinic
DGW and VDO Clinic
Paeds OPD
Adult ASD closures/ MRI list
Cath Lab / Radiology
Speciality Doctor Echo Clinic
BRHC Weekly M&M Meeting
Meeting Room 6
BRHC Surgical Conference
Meeting Room 6
Echo Meeting
C3 Seminar Room
Academic Session
Sophie Pearson Room
Teaching Ward Round
Sophie Pearson Room
Adult Congenital Clinic
Cardiology OPD
Paediatric Grand Rounds
Academic Seminar Rm
Fetal Clinic (OU)
SHO In-House Teaching
KRUF or Paeds SemRm
CVS Imaging MDT Meeting
4th Thursdays, SP Room
Speciality Doctor Echo Clinic
Paediatric Case Presentations
Paeds Seminar Room
Cardiology Academic Meeting
Nurse-Led Clinic
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.
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.
Bi- monthly
Neath Port Talbot Hospital
Nevill Hall Hospital (Abergavenny)
Prince Charles Hospital (Merthyr)
Princess of Wales Clinic (Bridgend)
Royal Glamorgan Hospital
1.5 / month
Royal Gwent Hospital
3.5 / month
Singleton Hospital (Swansea)
West Wales General Hospital
Withybush Hospital (Haverfordwest)
Dr Ofoe (+ Quarterly
GUCH clinic Dr
Dr Onuzo
Dr Ofoe (+ Dr Wilson
GUCH 2x/yr)
Dr Uzun
Dr Ofoe
Drs Wilson/Onuzo
Drs Wilson / Onuzo /
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)
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.
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
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.
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)
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
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
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.
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
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.
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
Protocol for institution of ACE inhibitors in older children:
Ascertain that the patient is physically fit, including baseline BP
Check renal function BEFORE the dose is given
Give test dose of CAPTOPRIL (0.1 mg/kg)
Arrange for nursing staff to check BP every 15 minutes for 90-120
If there is no marked hypotensive response, prescribe a maintenance dose
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
Remember the GP note and brief typed discharge summary
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.
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 -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:
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
0.25 mg/kg bd
Third increment (1 week
later, if clinically indicated)
0.5 mg/kg bd
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
Day Case admission for Test Dose
 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).
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.
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)
 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)
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)
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
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,
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).
Comparison of the change in absolute QT intervals (О”QT) among the genotypes.
>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%).
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).
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
Brugada syndrome - Flecainide or Ajmaline Challenge
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.
п‚· Suspected Brugada syndrome (abnormal ECG with symptoms of palpitation or
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
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.
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.
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.
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.
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
To evaluate myocardial perfusion reserve
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
Give Adenosine at 140 пЃ­g/kg/min for 6 minutes using an infusion pump.
(i.e 140 g X weight = ………..g of adenosine per minute for a total of 6
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
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
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
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”)
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
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.
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.
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
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:
Critical PS
Low pulmonary blood flow Pulmonary atresia
Tricuspid atresia
Critical AS
Severe CoA
Low systemic blood flow
Interrupted aortic arch
Inadequate mixing
Transposition of the great
Profound cyanosis
п‚± Murmur
(Cardiogenic shock)
Cardiac failure
Cardiogenic shock
Poor or unequal pulses
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%
1 mL/hr п‚є 10 nanogram/kg/min
(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.
Emergency Interventional Procedures
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.
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
Prior to carrying out DCCV, ensure the following:
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
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.
 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.
Pericardiocentesis for cardiac tamponade
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
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
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).
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
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
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.
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
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).
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
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
Has an inpatient report been dictated, checked, signed and dispatched?
Are any outpatient arrangements in place?
See Section 1.10 for Inpatient Report headings.
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
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
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
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.
 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
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
Is the GP letter/TTH complete?
Are suitable follow-up arrangements in place?
Has the discharge summary been dictated?
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.
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)
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.
Drug Monitoring and/levels
and consider
(changes are
Lithium heparin
U&E, LFT (must
specify ALT and
AST); add FBC if
Lithium heparin
bottle (plus
EDTA if having
Drug level
EDTA bottle
Drug level
Drug level and
and drug levels
Specimen bottle
for Tacrolimus EDTA
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
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
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.
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
The most commonly-used method for QTc calculation is the Bazett formula:
R-R interval
QT interval
пѓ–RR interval
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)
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:
 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).
 All other echo requests are regarded as a “referral for cardiology opinion”.
 Ensure the referral has been discussed by the child’s lead consultant. Ensure
the formal referral request document is filled in with adequate information.
 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.
 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.
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
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.
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
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
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
п‚· Document reason for cessation
 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)
Difficulties in interpretation
Breathlessness due to other cause (e.g. asthma)
Pre-existing BBB, especially LBBB
Digoxin (affects ST segments – consider stopping drug prior to exercise test)
-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
 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)
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
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
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.
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
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)
Daytime percentile *
Nighttime percentile †50th
120 (33)
130 (62)
140 (102)
150 (108)
160 (115)
170 (83)
180 (69)
120 (40)
130 (58)
140 (70)
150 (111)
160 (156)
170 (109)
180 (25)
*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
Tilt Test
п‚· Investigation of unexplained collapse
п‚· Suspicion of vasovagal syncope (a.k.a. neurocardiogenic syncope or neurally
mediated syncope)
п‚· Assessment of therapy
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
 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
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
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
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
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.
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).
Anti-platelet Therapy and Anticoagulation
6.1.1 Aspirin and Anti-platelet Therapy
Indications for use of aspirin in children (plus see tables below):
 Anti-inflammatory action – treatment of
o Acute pericarditis
o Kawasaki disease (acute phase)
o Acute rheumatic fever
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).
Devices and Stents
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
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.
Valve replacement
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
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
Cavopulmonary shunt / Fontan
Glenn / superior
cavopulmonary shunt
Aspirin 3-5 mg/kg once
To continue until Fontan /
daily, usual maximum 75
mg once daily*
Fontan / TCPC
Warfarin – target INR =
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.
Other indications
Modified Blalock-Taussig
Aspirin 3-5 mg/kg once
daily, usual maximum 75
mg once daily
Kawasaki Disease
Refer to AHA or UK
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
Pulmonary Arterial
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.
Guideline on antiplatelet and anticoagulation management in cardiac surgery Eur J Cardiothorac Surg
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
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)
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
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):
Hold next
Repeat anti-Xa
Increase by
4 hours after next dose
Increase by
0.35 - 0.49
4 hours after next dose
Next day, then 1 week later,
0.5 - 1.0
and monthly thereafter (4
hours after morning dose)
Decrease by
1.1 - 1.5
Before next dose
Decrease by
Before next dose then 4 hours
1.6 - 2.0
3 hours
after next dose
Before next dose, then every
Until anti-Xa 0.5 Decrease by
12 hours until anti-Xa level
<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:
Age group
Young children
Older children
Loading dose
1 – 2 mg
3 – 5 mg
5 – 8 mg
Day 1: 8 mg / Day 2: 8 mg
10 / 10 / 5 mg over successive
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:
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)
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
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
INR 8-10 with no or minor
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
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]
Warfarin dosage table (doses shown in mg)
9.5 / 10 / 10
9.5 / 10
9.5 / 9.5 / 10
9 / 9.5 / 9.5
9 / 9.5
9 / 9 / 9.5
8.5 / 9 / 9
8.5 / 9
8.5 / 8.5 / 9
8 / 8.5 / 8.5
8 / 8.5
8 / 8 / 8.5
7.5 / 8 / 8
7.5 / 8
7.5 / 7.5 / 8
7 / 7.5 / 7.5
7 / 7.5
7 / 7 / 7.5
6.5 / 7 / 7
6.5 / 7
6.5 / 6.5 / 7
6 / 6.5 / 6.5
6 / 6.5
6 / 6 / 6.5
5.5 / 6 / 6
5.5 / 6
5.5 / 5.5 / 6
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 / 4.5 / 4.5
4 / 4.5
4 / 4 / 4.5
3.5 / 4 / 4
3.5 / 4
3.5 / 3.5 / 4
3 / 3.5 / 3.5
3 / 3.5
3 / 3 / 3.5
2.5 / 3 / 3
2.5 / 3
2.5 / 2.5 / 3
2 / 2.5 / 2.5
2 / 2.5
2 / 2 / 2.5
1.5 / 2 / 2
1.5 / 2
1.5 / 1.5 / 2
1 / 1.5 / 1.5
1 / 1.5
1 / 1 / 1.5
0.5 / 1 / 1
0.5 / 1
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
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
 Remember – a full lab coagulation screen is needed for patients on heparin and
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
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
Enhanced metabolism of warfarin
o Phenytoin
o Carbamazepine
o Phenobarbitone
Reduced synthesis of coagulation factors II, VII, IX and X
o Phenytoin
o Salicylates
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)
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.
Asplenic children under 5 years have a particularly high infection risk of
overwhelming sepsis (>10%). They should receive
 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.
Standard vaccination schedule, including live vaccines
Influenza vaccination each Autumn (reduces the risk of secondary bacterial
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
o >24 months (if not immunized so far), give 2 doses of conjugate
vaccine at monthly intervals, followed by a dose of polysaccharide
o Booster pneumovax doses should be given every 5 years
п‚· Meningococcal A vaccine if travelling into an endemic area
Advice for family:
Parents should be made aware of the excess infection risk and the need for
immediate treatment of suspected infection.
They should have a reserve supply of antibiotics at therapeutic doses to
take on holiday.
They should be given the Asplenia Warning Card to carry.
The excess risk from malaria should preclude unnecessary travel to
endemic areas.
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.
Isomerisms can be familial therefore other family members should be
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
not always present and when present it may be of variable severity. The features
п‚· Characteristic facial appearance
п‚· Low serum calcium concentration
п‚· Congenital abnormality of the heart or great vessels (particularly conotruncal
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
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)
Cardiac Failure
Anomalous origin of left coronary artery
Cardiac tamponade
Cor pulmonale (BPD, CF)
Critical aortic stenosis
Interrupted aortic arch
Large Lп‚®R shunt
Severe valve regurgitation
Truncus arteriosus
Infective endocarditis
Severe anaemia
Severe hypertension
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
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.
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:
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
п‚· ECG
п‚· Chest x-ray
п‚· Echocardiogram
п‚· Oximetry
п‚· Capillary gas (if unwell)
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
 Treat fluid overload – fluid restriction and diuretics
 Prevent further salt and water retention – diuretics, angiotensin coverting
enzyme (ACE) inhibitors, and angiotensin receptor blockers
 Tachyphylaxis – -Blockers and digoxin (maintain digoxin level at 0.51.0ng/ml)
 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.
Heart rate
Blood pressure
Arterial waveform
Peripheral perfusion
Core-toe temperature gap
Urine output
Base deficit
Mental status
Adequate CO
large area under curve;
late dicrotic notch
peripheral pulses easy to
capillary refill < 2sec
< 2oC
>1 ml/kg/hr
<2 mmol/L
Inadequate CO
normal or low
small area under curve;
early dicrotic notch
peripheral pulses weak
central pulses palpable
capillary refill >2sec
> 2oC
<1 ml/kg/hr
agitated, disorientated
Pre-load affects cardiac output. If there is a low preload then the cardiac output is
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:
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
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
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.
Cardiac Tamponade - See section 3.7.2
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
features at all. It is therefore very important that a carefully structured investigation
protocol is followed to prevent these being overlooked.
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
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
п‚· Myocardial ischaemia may have an unusual presentation in children who are
unable to localise pain
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
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
Clotting – 1mL paeds citrate sample
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
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.
Cardiomyopathy Screen - Investigations
Unit Number:
Biochemistry Lab:
Test (units)
Total Protein
Uric acid
Date Result
Carnitine Total and Free
Acyl Carnitine Profile
Lithium Heparin (LH)
LH (to calculate anion gap)
LH (to calculate anion gap)
LH 0.5 ml (ice sample
–send within 10 min)
LH or white Guthrie
LH or white Guthrie
LH (0.5 ml)
Plasma Amino acids
Blood gas
Anion gap
(mmol/L) BM Capillary or
oxalate (yellow top) 0.5
(mmol/L) BM capillary or oxalate
Thyroid Function tests
Calculate make sure requesting
HCO 3 and Chloride in
Biochemistry sample
Free T4
Free T3
Auto antibodies
Plain top 1 ml
Haematology Lab
Blood Film
EDTA 4mls
Citrate (Green top)
Citrate (Green top)
Citrate (Green top)
Plain top 1 ml to
Urine tests
Urine organic acids
Urine amino acids
Urine Glucose
Urine Protein
Urine Ketones
Standard Virology assay
Coxsackie *Viral titres and
Plain top 1 ml
*Plain top and discuss with
virology for coxsackie
** for enterovirus send with stool
Extn 3325
Chest Radiograph
Extn 3027
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
Reference: JACC 2010;55:1377-84 – results of 189 patients, excludes pts exposed to anthracycline (small
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
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.
п‚· Diuretics
п‚· ?Digoxin
п‚· ACE inhibitors
 -blockade – once features of acute heart failure have been stabilised (see
section 3.2.3)
п‚· Anti-platelet or anticoagulation therapy
п‚· Cardiac transplantation
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.
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
 May be reproduced by pressure on the affected area or “springing” the ribs
п‚· Usually acute and subsides over days.
 Tietze’s syndrome
o Costo-chondritis usually of the 2nd to 5th costo-chondral junctions
o Swelling of the junctions may occur
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.
 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.
 Mitral valve prolapse – can be associated with non-specific chest pain (cause
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
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.
Endocarditis and Endocarditis Prophylaxis
6.7.1 Infective Endocarditis
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
п‚· Insidious onset (usually): fatigue, fever, anorexia, pallor
п‚· Murmur (especially changing), fever, splenomegaly, clubbing
 Petechiae, nail splinters, Osler’s nodes, Janeway lesions, embolic phenomena
(PE, cerebral, haematuria, Roth spots)
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
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
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
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.
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
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
Hypertrophic cardiomyopathy
Commotio cordis
Coronary artery anomalies
Left ventricular hypertrophy of indeterminate causation
Ruptured aortic aneurysm (Marfan syndrome)
Arrhythmogenic right ventricular cardiomyopathy
Tunneled (bridged) coronary artery
Aortic valve stenosis
Atherosclerotic coronary artery disease
Dilated cardiomyopathy
Myxomatous mitral valve degeneration
Other cardiovascular cause
Long QT syndrome
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.
In general the following principles should apply:
Patients with known HCM, AS, DCM, congenital coronary artery
anomalies, enlarged aorta and LQTS should avoid high
intensity/explosive sports.
Patients with known HCM, AS, DCM, congenital coronary artery
anomalies, LQTS and WPW should avoid unaccompanied crosscountry running and swimming.
Patients with LQTS, particularly LQTS type 1, should avoid diving
into cold water.
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.
Patients on anticoagulants or anti-platelet agents should avoid highimpact contact sports.
Patients with exercise-induced SVT should avoid the activities that
precipitate symptoms, or should take prophylactic anti-arrhythmics
prior to sports participation.
Patients with VT should avoid sports.
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.
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
Classification of Competitive Exercise
A. Low
B. Moderate
C. High
III. High
Track & Field (throwing
Martial arts*
Rock climbing
Water skiing*С„
Weight lifting*С„
Auto racing*С„
Diving (pool) *С„
Body building*С„
Downhill skiing*С„
Wrestling (GrecoRoman)
American football*
Track & Field
(jumping events)
Figure skating*
Running (sprint)
Table tennis
Ice hockey*
Cross-country skiing
(skating technique)
Running (middle
II. Moderate
Billiards, snooker, pool
Cross-country skiing
10-pin bowling
(classical technique)
Field hockey*
Race walking
Cricket (NB minimum
of helmet and body
Running (long
armour needed for
patients on
Increasing Dynamic Component→
* = Danger of bodily collision – avoid if on anticoagulants
Р¤ = Risk is greater if likelihood or history of syncope
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.
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:
LV function, wall motion, and cardiac dimensions return to normal (based
on echocardiographic and/or radionuclide studies at rest and with
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.
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).
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
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:
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.
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.
In patients with a history of Kawasaki Disease:
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.
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.
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.
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.
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
Severity of AR
Gauged by…
Absent or slight signs of AR; normal LV size
(<60 mm in adults, taking into account
athletic training
Peripheral signs of AR with mild-moderate
increase in LV size
Peripheral signs of AR with severe LV
enlargement В± LV dysfunction
Mild AR, aorta <97th percentile in diameter: no restrictions.
Moderate AR with normal LV systolic function: class I and II sports
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
Doppler gradient
Peak echo Doppler
gradient (peak
Mild AS – If ECG is normal and there are no symptoms, activities are
unrestricted (NB exercise test recommended).
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.
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
BAV with moderate aortic enlargement (40-45 mm in adults, equivalent
percentile in children): Non-contact IA, IB, IIA, IIB permitted.
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.
Coarctation of the aorta
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.
>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
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
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
Ebstein’s malformation
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
(iii) Severe Ebstein’s: no competitive sports are permitted
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.
L→R shunt lesions (ASD, VSD, PDA, AVSD, A-P window)
Uncomplicated lesion (small shunt, no PHT) – No restrictions.
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
(iii) Lesion complicated by Eisenmenger syndrome – no competitive sports
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:
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).
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.
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
Pulmonary valve disease
Mild PS (gradient < 40 mmHg) with no symptoms – no restrictions
>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.
Severe pulmonary regurgitation with dilated RV, class IA and IB sports
are permitted.
Recommendations related to aortic regurgitation – see above.
These recommendations are offered independent of whether beta-blockers
are administered to mitigate aortic root enlargement.
Mitral regurgitation
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.
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.
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
Normal or borderline low ventricular function (EF≥50%): full
participation permitted.
Mild ventricular dysfunction (EF 40-50%): classes IA, IB and IC are
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.
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.
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:
Exercise is unrestricted if there are no significant haemodynamic
problems and ventricular function is normal.
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.
If the above are satisfactory and the patient is asymptomatic, IA sports
are permitted (body armour for cricket if patient is anticoagulated).
IB sports are permitted if ventricular function and O 2 saturations are
Prosthetic valve replacement
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
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
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)
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.
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.
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
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.
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
ppear to be at low risk for sudden
cardiac death.
Congenital Complete Heart Block
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.
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
Long QT Syndrome
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.
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
(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.
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.
Fits, Faints and Funny Turns
п‚· Reflex syncope (abnormalities of heart rate п‚± vasomotor tone, HOCM)
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
 ECG – look for heart block, WPW, LQTS, Brugada syndrome
 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
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
Preventing attacks
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
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
Hypercyanotic Spells
п‚· Tetralogy of Fallot
п‚· Other forms of RVOT obstruction
п‚· Tricuspid atresia
п‚· Pulmonary atresia/VSD with MAPCAs
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
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),
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.
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)
 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
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
Laboratory features are compatible
Often complicated by coronary artery aneurysm because of delayed
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.
ESR (often > 100)
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
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
80-100 mg/kg/day given in 4 divided doses (USA and Japan
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.
There are no hard and fast rules regarding the follow-up arrangements although the
American Heart Association produced guidelines in 2004 (Reference: Circulation
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.
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.
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 L→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
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
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
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:
The desired O 2 flow rate (usually up to 2 L/min if the indication is
The frequency and type of monitoring (e.g. no monitoring, spot
checks, overnight pulse oximetry, combination of spot checks and
overnight monitoring)
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
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.
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
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.
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.
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
0.01mcg/kg/min = 1ml/hr
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)
X Weight (Kg)
0.33mcg/kg/min = 1ml/hr
1 mcg/kg/min = 1ml/hr
X Weight (Kg)
X Weight (Kg)
0.01 - 1.0mcg/kg/min
Norepinephrine 0.01 - 0.5mcg/kg/min
0.005 - 0.05mcg/kg/min
0.025 - 1.0mcg/kg/min
0.01 - 1.0mcg/kg/min
Norepinephrine 0.01 - 0.5mcgkg/min
0.01 - 1.0mcg/kg/min
Norepinephrine 0.01 - 0.5mcg/kg/min
0.01 - 1.0mcg/kg/min
Norepinephrine 0.01 - 0.5mcg/kg/min
0.025 - 1.0mcg/kg/min
0.33 - 0.99mcg/kg/min
0.5 - 10mcg/kg/min
0.5 - 10mcg/kg/min
= 1ml/hr
= 1ml/hr
X Weight (Kg)
X Weight (Kg)
10 mcg/kg/min
= 1 ml/hr
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)
10 mcg/kg/hr = 1ml/hr
20 mcg/kg/hr = 1ml/hr
DOSE in MG in a 50ml Syringe
0.5 X Weight (Kg)
X Weight (Kg)
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
10 - 40mcg/kg/hr
10 - 40mcg/kg/hr
*Titrate up as required
DOSE in UNITS in a 50ml
10 X 50 X Weight (Kg)
Heparin – standard concentration
0.25 mg/kg/hr = 1ml/hr
DOSE in MG in a 50ml Syringe
12.5 X Weight (Kg)
1 mg/kg/day = 1ml/hr
DOSE in MG in a 50ml Syringe
2 X Weight (Kg)
Phenoxybenzamine 0.5 - 2 mg/kg/day
DOSE in UNITS in a 50ml
0.3 X Weight (Kg)
(Max: 50 Units/50ml)
10 Units/kg/hr
= 1ml/hr
0.0001 Unit/kg/min = 1ml/hr
5 nanogram/kg/min = 1ml/hr
(0.005 mcg/kg/min = 1ml/hr)
Standard concentration titrate as
per order
50ml Syringe
15 X Weight (Kg)
DOSE in GRAM in a 40ml
1G in 40ml of sterile water =
0.25 - 0.5mg/kg/hr
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
(Prostacyclin or Flolan)
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.
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.
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).
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
Protein-losing enteropathy (PLE) / Plastic bronchitis (PB) after Fontan
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)
 ECG/24 hour tape – to rule out arrhythmia
 Echo – to rule out haemodynamic cause
 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
 Baseline bone densitometry studies (dexa scan) – heparin and prednisolone,
which are both used to treat PLE, cause bone demineralisation.
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
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)
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.
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)
Pulmonary hypertension with pulmonary venous hypertension due to left heart
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
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 L→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
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
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
 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.
Empirical treatment of Pulmonary Hypertension
Increase urine output
and reduce fluid
Pulmonary vasodilator
Slows heart rate and
increases force of
contraction of the heart
Improves cardiac output
acutely in patients with
In some studies, nearly
doubles 3-year survival in
primary PHT; some studies
have shown no benefit.
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
e.g. nifedipine
Evidence Base
Lacking, but considerable
clinical experience. May
benefit Eisenmenger
patients with right heart
failure or significant valve
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
preserving cardiac
output following the
output at the expense of
systemic desaturation
IPAH – idiopathic pulmonary arterial hypertension
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
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
“Targeted” therapy in PAH
Prostaglandin therapy
Vasodilation, inhibition
of platelet aggregation,
promotion of
Endothelin I and II
Evidence Base
Continuous i.v.
epoprostenol improves
quality of life, exercise
capacity and life
Nebulised iloprost
improves exercise
capacity and
Safety and efficacy
shown in open label
and randomised
controlled trials in
IPAH; increasing
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
Available in oral form.
Bosentan licensed for
use in PAH and
Eisenmenger syndrome
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
in over 12s.
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)
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:
Hypothermia, hypoglycaemia, hypoxia and acidosis
Bacterial pneumonia, meconium aspiration syndrome, surfactant deficiency lung
Chronic fetal hypoxia, placental insufficiency, postmaturity, polycythaemia
Congenital diaphragmatic hernia
Primary pulmonary hypoplasia
Congenital alveolar capillary dysplasia
Clinical features:
The most important clinical feature is difficulty in oxygenating the neonate with
persistent low O 2 saturations despite increasing FiO 2 and ventilatory support.
The blood gas (arterial) is likely to show severe hypoxemia.
There is significant difference in pre and post ductal PaO 2 (>2.5 kpa) or O 2
saturations (5-10%).
A prominent right ventricular impulse may be noted and murmurs due to tricuspid
regurgitation or pulmonary regurgitation may be heard.
Signs of heart failure may be present.
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.
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:
RV pressure can be calculated from the TR jet (4v2 + add estimated RA pressure)
Ensure the envelope is complete
Interpret in the context of systemic BP
Atrial shunting and other shunts:
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).
Bowing of the interatrial septum to the left is commonly seen.
Right-to-left atrial shunting reflects right atrial filling (diastolic) pressure
If a VSD is present, bidirectional shunting may be noted.
Ductal flow:
The direction and velocity of ductal blood flow can give useful information on
Pure right-to-left flow indicates PAP is higher than aortic pressure throughout
cardiac cycle.
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).
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
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:
There may be enlargement of the RV and RA, as well as the main pulmonary
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.
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)
Quantitative assessment of cardiac function may assist with decisions and
assessments of the roles of inotropes and inhaled nitric oxide.
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
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
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
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 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
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
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
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)
Skinner J, Alverson D, Hunter S (eds). Echocardiography for the Neonatologist. Churchill Livingstone 2000
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.
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.
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 ;(
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
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
 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
1 major and 2 minor criteria
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:
6.20.2 Secondary Prevention of Rheumatic Fever (Recurrent Attacks)
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
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
Macrolide or azalide
Mode Rating
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
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
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)
5 years or until 21 years of age
(whichever is longer)
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 L→R shunt or with pulmonary
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).
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
п‚· Full history and examination, including family history
п‚· ECG (looking for epsilon wave in V1), SAECG
 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
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)
 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
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)
 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.
“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
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.
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
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
п‚· Ensure involvement of medical genetics (consider predictive testing if
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)
 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
Radiotherapy to thorax, thoracic spine, or mediastinum (including left flank
and total body irradiation)
All patients who have received anthracyclines require an echocardiogram following
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
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)
 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
п‚· Consider need for ICD
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)
 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
п‚· Consider need for VStim study (if +ve ECG or drug challenge, or if
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)
 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
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.
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
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
п‚· Review 1-3 yearly, depending on findings
п‚· Full history and clinical examination
 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)
6.23 Supraventricular Tachycardia
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?
See algorithm for shocked SVT
Diving Reflex: Facial immersion in iced water for 5 seconds (neonates only)
Facial cooling (ice pack on face)
Carotid sinus massage
Valsalva manouvre
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
Adenosine – 300 micrograms/kg IV via large vein using 3-way tap
Flush with saline (5ml if < 1yr, 10ml if >1 yr)
Adenosine - 300 micrograms/kg IV via large vein using 3-way tap
Flush with saline (5ml if < 1yr, 10ml if >1 yr)
Start prophylaxis
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.
SVT – haemodynamically unstable (Shocked SVT)
Oxygen, Airway, Breathing
Synchronous DC Cardioversion,
1 J/kg
Synchronous DC Cardioversion,
2 J/kg
Consider IV Sodium Bicarbonate between shocks
Start prophylaxis
Discuss with cardiologist, consider IV
amiodarone (5 mg/kg over 60-120 min)
SVT Prophylaxis
Check no
obstructive heart
lesion, ensure good
cardiac function
Flecainide 1 to 3 mg/kg/dose twice
(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
Flecainide – Atenolol (or propranolol)
Flecainide – Digoxin
Sotalol – Digoxin
Atenolol (or propranolol) – Digoxin
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
The patient will require close follow-up between the fetal cardiac and fetal medicine
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.
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.
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
п‚· Fever, malaise, anorexia, vomiting
п‚· Breathlessness
п‚· Hepatosplenomegaly
п‚· Gallop rhythm
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
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
Candida or aspergillus
Herpes simplex
oral aciclovir
none available
amphotericin (caution – renal
impairment), ambisome
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.
"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,
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,
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
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,
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
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
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,
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
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