3/26/2014 PULSE OXIMETRY SCREENING FOR CRITICAL CONGENITAL HEART DISEASE (CCHD) DR. ROBERT GINGELL 1846 n tya Sa Satyan Lakshminrusimha, Vivien Carrion, Umang Gupta Division of Neonatology and Pediatric Cardiology Department of Pediatrics, University at Buffalo FAMILY THE FISHERMAN ARTIST OUTLINE • Physiology • Why do we need one more screening for newborns? • Procedure • Cost effectiveness in newborn nursery • Do we need CCHD screening in the NICU? • What conditions can be picked by CCHD screening? • Conditions missed in spite of CCHD screening • Post-screen follow-up 1 3/26/2014 PRINCIPLES OF CCHD SCREENING CASE • Full term baby 40 weeks 4 days gestation, 3225 g) admitted to normal newborn nursery delivered by vaginal delivery • Normal fetal echocardiogram • Uneventful examination • CCHD Screen was performed at 28 hours of life • Right upper limb saturations – 91% • Left foot saturations – 90% • Result – failed screen • Baby transferred to NICU • Echo showed tetralogy of Fallot • Approximate oxygen saturations in various vessels during fetal life is shown in the figure • The highest oxygen saturation is in the umbilical vein (approximately 80%) • This corresponds to a PO2 level of 30 – 35 mmHg • Umbilical arterial PO2 level is 19-22 mmHg (approximately 55-60% saturation) # 1: AN OPEN DUCTUS (AND FORAMEN OVALE) CAN SUSTAIN LIFE IN MANY CCHDS CYANOTIC CHD • Left sided lesions 100 mmHg 100% • Coarctation of Aorta • Critical Aortic Stenosis • Hypoplastic left heart syndromes 30 mmHg 60% • Mixing Lesions ?? mmHg ??% • Transposition • Right sided lesions • Tricuspid atresia • Severe pulm stenosis • An infant with tetralogy of Fallot is in room air. Assuming a left atrial outflow of 100ml/kg/min and right atrial outflow of 100ml/kg/min and complete admixture due to a large VSD, what is the SpO2 in the ascending aorta? Fetal Circulation with TGA BASIS OF CCHD SCREENING TEST • Large amount of oxygen chemically combines with hemoglobin • Very little is present in blood in dissolved form 50 ml of blood PaO2 – 30 mmHg 100 ml of blood with PaO2 – 65? 50 ml of blood PaO2 – 100 mmHg MEAN OF OXYGEN SATURATION (NOT PO2) • Oxygen content of blood has a direct relationship with oxygen saturation (and not oxygen tension) 50 ml of blood PaO2 – 30 mmHg 50 ml of blood PaO2 – 100 mmHg 100% 60% 20 ml 12 ml 100 ml of blood with PaO2 – 45 mmHg 80% 16 ml 2 3/26/2014 HYPEROXIA TEST 50 ml of blood PaO2 – 30 mmHg • Oxygen content of blood has a direct relationship with oxygen saturation (and not oxygen tension) 50 ml of blood PaO2 – 600 mmHg 100% 60% 21.8 ml 12 ml 100 ml of blood with PaO2 – 45 mmHg EVEN A SMALL ADDITION OF DESATURATED BLOOD DROPS OXYGEN SATURATION 10 ml of blood PaO2 – 30 mmHg • Oxygen content of blood has a direct relationship with oxygen saturation (and not oxygen tension) 100 mmHg 100% 20.9 ml 60% 12.1 ml 60 ml of blood with PaO2 – 53 mmHg 80% 16 ml 50 ml of blood PaO2 – 93% #3: PRE-POST DUCTAL SATURATION DIFFERENCE IS NORMAL IN THE FIRST 24H #2: Admixture of even a small amount of deoxygenated blood will drop arterial oxygen saturation < 95% 600 mmHg 100% 30 mmHg 60% 45 mmHg 80% Mariani et al, J Pediatrics 2007 ACCEPTABLE AND UNACCEPTABLE PULSE OX A.K.A TURKOVICH TABLE #4: DUCTAL SHUNTS INCREASE PREPOST OXYGENATION DIFFERENCE Differential Cyanosis Acceptable Any Foot FOOT RIGHT HAND Ductal closure 100 99 98 97 Right 96 hand 95 94 93 92 91 90 100 100 100 100 100 100 100 100 100 100 100 Reverse Differential Cyanosis 99 99 99 99 99 99 99 99 99 99 99 98 98 98 98 98 98 98 98 98 98 98 97 97 97 97 97 97 97 97 97 97 97 96 96 96 96 96 96 96 96 96 96 96 95 95 95 95 95 95 95 95 95 95 95 94 94 94 94 94 94 94 94 94 94 94 93 93 93 93 93 93 93 93 93 93 93 92 92 92 92 92 92 92 92 92 92 92 91 91 91 91 91 91 91 91 91 91 91 90 90 90 90 90 90 90 90 90 90 90 Central (global) cyanosis 3 3/26/2014 CRITICAL CONGENITAL HEART DISEASE (CCHD) • 8/1000 newborns born with CHD of which 2/1000 have CCHD. WHY DO WE NEED TO SCREEN? • The purpose of the Congenital Heart Disease (CHD) Screening Program is to identify newborns with CHD prior to clinical deterioration of the affected infant. • 10-15% of all forms of congenital heart disease (frequency 1.2-1.7/1000). • Delayed diagnosis of critical congenital heart disease (CCHD) can result in death or injury to infants. • Delays in identification of CCHD range from 20-25 % - 43%. (US missed diagnosis in 7/100,000) CCHD FACT SHEET WHY DO WE NEED ONE MORE NEWBORN SCREENING? • Critical CHD (CCHD) is defined as CHD that requires surgical intervention during infancy for survival. • Frequency: 1 to 2/ 1000 live births • Congenital heart defects (CHDs) account for 24% of infant deaths due to birth defects. • In the United States, about 4,800 (or 11.6 per 10,000) babies born every year have one of seven critical congenital heart defects (CCHDs, which also are known collectively in some instances as critical congenital heart disease). SCREENING • Screening exists for metabolic, hematologic and endocrine disorders as well as hearing loss. • CHD: “most life-threatening condition in the first month of life and represents 20% of neonatal deaths”. • Only 50% of these CCHD are diagnosed prenatally • Physical examination may identify 10-20% of CCHD during initial newborn nursery stay before discharge • 30-40% of patients may be discharged from newborn nursery and brought to ED with distress/ apnea/ circulatory failure WHO ENDORSES THIS? • The Department of Health and Human Services here in the United States made this CHD (also called CCHD—Critical Congenital Heart Disease) screening recommendation September of 2011. • In January of 2012, the American Academy of Pediatrics endorsed this recommendation. • We have been conducting pulse oximeter screening in the NICU since March 2012 (one of the first NICUs to adapt CCHD screening) 4 3/26/2014 PRENATAL AND POSTNATAL DIAGNOSIS • Prenatal overall detection rate for CHD of 28%, (range of 23% - 80%). CCHD screen • Postnatal detection by exam only misses up to 50%. • If CCHD screening is not conducted, almost 20% of these babies may be discharged from the hospital to return to PMD or ED with acidosis, shock and/or severe hypoxemia DETECTION CCHD • New Jersey: 1st state to legally mandate pulse oximetry screening to detect CCHD. • 75,324 births and 72,694 neonates screened with pulse oximetry. • 49 failed for a failure rate of 0.067%. 30 of these had no signs or symptoms. (3 of these had CCHD). Eur J Pediatr (2010) 169:975–981 COST SURVEY IN NEW JERSEY • Mean screening time per newborn was 9.1 (standard deviation = 3.4) minutes. • Hospitals' total mean estimated cost per newborn screened was $14.19 (in 2011 U.S. dollars), consisting of $7.36 in labor costs and $6.83 in equipment and supply costs. PublicHeath Rep. 2014 Jan-Feb;129(1):86-93 PROCEDURE A screen is considered positive if (1) any oxygen saturation measure is <90% (in the initial screen or in repeat screens); (2) oxygen saturation is <95% in the right hand and foot on three measures, each separated by one hour; or (3) a >3% absolute difference exists in oxygen saturation between the right hand and foot on three measures, each separated by one hour. Any screening that is ≥95% in the right hand or foot with a ≤3% absolute difference in oxygen saturation between the right hand or foot is considered a negative screen and screening would end. Any infant with a positive screen should have a diagnostic echocardiogram, which would involve an echocardiogram within the hospital or birthing center, transport to another institution for the procedure, or use of telemedicine for remote evaluation. The infant’s pediatrician should be notified immediately and the infant might need to be seen by a cardiologist for follow-up. 5 3/26/2014 INCREASING SENSITIVITY & SPECIFICITY WHEN ARE BABIES SCREENED? • Pulse oximetry is a simple bedside test to determine the amount of oxygen in a baby’s blood and the baby’s pulse rate. Low levels of oxygen in the blood can be a sign of a CCHD. • De-Wahl method: conducting simultaneous preductal and postductal pulse oximetry • Screening is done when a baby is 24 to 48 hours of age, or as late as possible if the baby is to be discharged from the hospital before he or she is 24 hours of age. • Koppel method: screening after 24h • Pulse oximetry screening does not replace a complete history and physical examination. WCHOB NICU No CCHD screening CCHD screening (1/2010 – 12/2011) (3/2012 – 2/2014) Total number of admissions 1533 1247 1888 1483 16 4 12 n/a 465 15 4 11 1421 472 68 62 5 4 Number of direct discharges home (excluding transfers, transfer of service and deaths) Number of CCHD diagnosed during this period Antenatal diagnosis Postnatal diagnosis Number of CCHD screens performed Infants with echocardiograms performed for clinical indications Infants discharged home on oxygen (or unable to wean off oxygen prior to CCHD screen) Infants unable to be weaned to room air prior to screen who have not had an echo for clinical indication No CCHD screening (1/2010 – 12/2011) Number of failed N/A screens (false positive) True positives N/A Missed patients – 1 – interrupted readmissions aortic arch and intervention CCHD screening (3/2012 – 2/2014) 9 1 – mild PS 1-tiny PFO (>3% diff) 1 – normal (>3% diff) 1-PFO, AV malformation 1-not recognized (discharged without an echo) 1-discharged home on oxygen and hence echo done – PDA PFO < 35 weeks: 2 – PFO; 1- normal PULSE OXIMETRY IN CRITICAL CONGENIAL HEART DISEASE – PITFALLS PUTTING IN PERSPECTIVE Umang Gupta, MBBS, DCH, FAAP, FACC 1 – TAPVR* 0 * This echocardiogram was done for clinical indications as a term baby developed an oxygen need 6 3/26/2014 RECOMMENDATIONS • Recommendations based on analysis by Mahle* and the group. • Pulse oximetry obtained as late as possible prior to discharge ( >24 hrs after birth) • Recommendations to obtain in right arm and one lower extremity PHYSIOLOGY • Physiological basis : For low arterial saturations - Mixing of oxygenated and deoxygenated blood (e.g. HLHS, Tricuspid atresia) • >95% in any extremity and < 3% difference is Pass • < 90% would need immediate referral - Reduced Pulmonary blood flow (TOF,PS) - Right to left shunt (TOF) • In between values need repeat measurements * Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the American Heart Association and American Academy of Pediatrics; Mahle WT et al; Circulation. 2009 Aug 4;120(5). PITFALLS PHYSIOLOGY For differential saturations - Ductal dependent left sided lesions (Critical AS, Coarctation of aorta, DTGA with Coarctation) - Concern more with negative screen - Saturations may exceed 95% if - Excessive PBF in mixing lesions (TA) - Fick Principle: QP/Qs = (SaO2 – SvO2) /(PvO2-PaO2) + dissolved O2 PITFALLS PITFALLS - Say Qp: Qs = 5 - Normal cardiac output ( A-V sat difference would be 25 %) - Pulmonary venous saturations – fully saturated (100 %) - In non mixing lesions – initial obstruction may not be severe to cause reduced PBF (PS, Pink TOF) or significant right to left shunt (TOF) - Rearranging the previous equation – (PvO2-PaO2) = (SaO2 – SvO2) / Qp: Qs 100 – PaO2 = 25/5 Or 100-5= PaO2= 95% 7 3/26/2014 PITFALLS The differential saturations may not be seen - If the lesion is dependent on ductal left to right shunt (Critical PS or TOF) - Non ductal dependent (AVSD, TAPVC) Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the American Heart Association and American Academy of Pediatrics; Mahle WT et al; Circulation. 2009 Aug 4;120(5). Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the American Heart Association and American Academy of Pediatrics; Mahle WT et al; Circulation. 2009 Aug 4;120(5). To Err is human, to forgive is against hospital Policy 8
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