Keeping the Beat: Pediatric Cardiac Screening and Management Objectives of Congenital Heart Disease Prenatal Diagnosis of Critical Recognize the importance of screening for Critical Congenital Heart Disease Congenital Heart Defects (CCHD) using pulse oximetry Robert Koppel, MD Apply the New Jersey recommended CCHD screening Neonatal/Perinatal Medicine, Pediatrics protocol North Shore-LIJ Medical Group Associate Professor Hofstra North Shore-LIJ School of Medicine Identify the role of the primary care physician in the March 24, 2015 detection of and referral of CCHD New Jersey Critical Congenital Heart Defects New Jersey Critical Screening Program Congenital Heart Defects Screening Program Disclosure The Law “The Commissioner of Health and Senior Services shall require Dr. Koppel has no conflict of interest to each birthing facility licensed by the Department of Health and disclose. Senior Services to perform a pulse oximetry screening, a minimum of 24 hours after birth, on every newborn in its care.” The New Jersey Department of Health does not endorse or promote a specific brand or NJ first state to implement vendor for pulse oximetry supplies and a mandate for pulse oximetry screening equipment. Equipment and/or supplies Legislation signed into law June 2, 2011 presented in the education are for Implementation date August 31, 2011 informational purposes only. P.L. 2011, Chapter 74 New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Dylan’s Story Congenital Heart Defects 8 ‐ 9/1,000 live births Minimum of 32,000 ‐ 40,000 infants affected each year in US On September 1 , a day after the law mandating inclusion of pulse oximetry Dylan was transferred to Approximately 25% of these are critical testing on newborns became effective, a Columbia University Medical hospital pediatrician informed Lisa and congenital heart defects (CCHD) or about 2 in Center, and several days later Bill Gordon of Newton that the test performed on their baby was abnormal had the life ‐ saving surgery 1,000 live births and he had a murmur. correcting the abnormality Dylan was rushed to Morristown discovered from the newly Medical Center, where it was determined mandated newborn testing. he needed specialized pediatric cardiac heart surgery. http://www.state.nj.us/governor/news/news/552011/approved/20111109a.html New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program 1
Rationale for Pulse Ox Screening Morbidity Due to Delayed Diagnosis An estimated 25 ‐ 30% of newborns with CCHD could Shock ‐ global hypoxemic injury with multi ‐ be missed at the time of hospital discharge (Mahle et. organ dysfunction al., 2009) • Hypotension About 1,200 more newborns with CCHD could be • Poor ventricular function identified at birth hospitals using pulse oximetry • Myocardial ischemia (Peterson et al., 2013.) • Pulmonary hypertension Approximately 200 newborns have died each year • Renal dysfunction from missed CCHD and numerous others have • Hepatic dysfunction significant morbidity from delayed diagnoses • Decreased intestinal blood flow ‐ NEC (Hokanson, 2010.) • DIC Compare to an average 66 young athletes each year • Metabolic: hypoglycemia, hypocalcemia, who die suddenly of undiagnosed cardiac defects myoglobinuria, hypoxic ‐ ischemic encephalopathy (Maron et al. 2009) Mahle et al., 2009. New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Detection of CCHD Continuum Detection of CCHD Prenatal Ultrasound 60% Prenatal 20% Clinical 15.6% Pulse Ox 4.4% Diagnostic Gap Physical exam Riede et al., 2010. New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Not As Pink As You Think… The Cyanotic “Blind Spot” Some babies can initially appear healthy Some babies do not have murmurs or cyanosis Physical exam alone failed to identify half of CHDs that were not detected by a prenatal ultrasound It’s estimated that 30% of infant deaths from CCHD occur prior to diagnosis Hokanson , 2010. New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program 2
CCHD Screening with Pulse Oximetry Newborn Screening ‐ New York – 1960’s Robert Guthrie, MD Virginia Apgar, MD Indirectly monitors the oxygen saturation of a patient's blood and variations in blood flow in the skin Can detect mild hypoxemia without apparent cyanosis Can provide continuous and direct values Non ‐ invasive Easy to use and widely available Cost ‐ effective and extensively used The Texas Pulse Oximetry Project, 2013. Source: Museum of Disability History Source: Wikipedia New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Screening Cost Screening Cost per Infant CDC Study in 7 NJ Birthing facilities Hospital – Based Hearing Mean screening time per newborn was 9.1 Screening $36 ‐ $39 (standard deviation 3.4 minutes) Hospitals’ total mean estimated cost per Laboratory Metabolic newborn screened was $14.19 (in 2011 U.S. Screening $20.00 dollars), consisting of $7.36 in labor costs and $6.83 in equipment and supply costs Pulse Ox Screening $14.19 Peterson et al., 2014. These cost estimates exclude follow-up costs, such as further diagnostic testing, as well as administrative overhead costs. New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Case Presentation Screening Case Discussion 40 weeks gestation, C/S, 3600 grams Discharged home on day 3 Brandon ‐ TGA ‐ failed screen; Day 5: returned to ED for poor feeding and early detection decreased activity HLHS ‐ not screened; SpO 2 : 80% late detection ABG: pH 6.8 Coarctation of aorta ‐ passed Echo: HLHS screen (false negative); late detection Pre ‐ op stabilization X 5 days Norwood stage I Post ‐ op ECMO X 8 days New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program 3
Case Presentation Case Presentation Referral to ED for respiratory distress 39 weeks, NSVD, Apgar 9/9 grunting Discharged home on Day 2 retracting unable to measure SpO2 Oximetry screening ‐ post ‐ ductal SpO2 100% Intubated Day 3 Umbilical arterial and venous catheters inserted Lethargy ABG: 7.09/17/199/8/ ‐ 23.3 Decreased PO intake Chemistry: 143/8/104/6/63/5.98 Dry diapers Echo: coarctation, ductus arteriosus closed Tachypnea (history of normal fetal echo) Prostaglandin infusion Evaluated by pediatrician Dialysis prior to repair of coarctation New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program CCHD Screening CCHD Screening Five Secondary Targets: Seven Primary Targets (17 ‐ 31% of all CHDs): Coarctation of the aorta Hypoplastic left heart syndrome Double outlet right ventricle Pulmonary atresia (with intact ventricular septum) Ebstein anomaly Tetralogy of Fallot Interrupted aortic arch Total anomalous pulmonary venous return Single ventricle Transposition of the great arteries Tricuspid atresia Truncus arteriosus New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program Effect of CCHD on O2 Saturations Effect of CCHD on O2 Saturations Normal Heart Transposition of the Hypoplastic Left Heart Tetrology of Fallot Great Arteries (TGA) Syndrome (HLHS) (TOF) Modified diagrams courtesy of the Centers for Disease Control and Prevention, Modified diagrams courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities. National Center on Birth Defects and Developmental Disabilities. www.cdc.gov/ncbddd/heartdefects www.cdc.gov/ncbddd/heartdefects New Jersey Critical New Jersey Critical Congenital Heart Defects Congenital Heart Defects Screening Program Screening Program 4
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