Abstract 13617: Newborn Critical Congenital Heart Disease Screening Fails to Detect Target Lesions or Reduce Mortality

Abstract

Introduction: Congenital heart disease (CHD) affects over one million children in the United States. Studies show mixed outcomes regarding a survival benefit from newborn cardiac screening (NCS). California began a NCS program on 7/1/13. We evaluated the effect of mandatory NCS on surgical outcomes of our program. Hypothesis: Neonates with critical CHD (CCHD), requiring surgery as infants, who are not diagnosed prior to or at the NCS have lower survival than those diagnosed prior to or at the NCS. Methods: We evaluated all infants with CCDH undergoing cardiac surgery at our center between 07/01/13 - 12/31/18. Subjects were stratified by timing of diagnosis (pre-screen, screen positive, screen negative). Sensitivity to detect specific lesions was calculated. Primary end-points were operative mortality, absolute mortality, and actuarial survival. Results: Two-hundred-seventy-four infants with CCHD were included. Seventy-nine percent were diagnosed prior to screening, while 8% were diagnosed by NCS, and 13% were screen negative. Total anomalous pulmonary venous return (TAPVR) (47%) and aortic coarctation (CoA) (40%) were the most likely to be undiagnosed at time of NCS (p<0.001). Sensitivity to detect target lesions was 38% overall [TAPVR 73%, CoA 12%, hypoplastic left heart syndrome (HLHS) 25%, Tetralogy of Fallot (TOF) 29%]. Primary end-points were not different based on timing of diagnosis (p>0.05) (Figure). Conclusions: We found only a 38% sensitivity of mandatory newborn CCHD screening to detect target cardiac lesions requiring surgery in the first year of life. A high proportion of newborns with HLHS, TOF, and CoA remained undiagnosed after screening, though TAPVR was readily detected. There were no differences in survival between the screening groups. Mandatory newborn CCHD pulse oximetry screening can detect some newborns with critical heart disease, but should not replace traditional methods of monitoring in the fetal, newborn, and infant periods.