Predicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention with Planned Pediatric Cardiac Operating Room Delivery

Abstract

ABSTRACTBackgroundDistances between delivery centers and cardiac services can make the care of fetuses with cardiac disease(CD) at risk of acute cardiorespiratory instability(ACRI) at birth a challenge. In 2013 we implemented a fetal echocardiography(FE)-based algorithm targeting fetuses considered high-risk for ACRI at ≤2 hours of birth for Caesarian section(CS) delivery in our pediatric cardiac operating room(PCOR) of our children’s hospital. We examine the experience and outcomes of affected newborns.MethodsWe reviewed maternal and postnatal medical records of all fetuses with CD at high-risk for ACRI encountered January 2013-March 2022. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum(d-TGA/IVS) and hypoplastic left heart syndrome(HLHS) encountered over the study period.ResultsForty fetuses were considered high-risk for ACRI: 15 d-TGA/IVS and 7 HLHS with restrictive atrial septum(RAS), 4 absent pulmonary valve syndrome, 3 obstructed anomalous pulmonary veins, 2 severe Ebstein anomaly, 2 thoracic/intracardiac tumors and 7 others. PCOR delivery occurred for 33 but not for 7 (5 d-TGA/IVS, 2 HLHS with RAS). For high-risk cases, FE had a positive predictive value of 50% for intervention/ECMO/death at ≤2 hours and 70% at ≤24 hours. Of “low-risk” cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. FE predicted intervention/ECMO/death at ≤2hours with a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 87% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively.ConclusionsFE predicts need for urgent intervention in majority with d-TGA/IVS and HLHS, and in half of the entire spectrum of high-risk CD.