Abstract

Background: Among infants with single ventricle congenital heart disease (SVD) requiring Stage I Norwood operation (S1P), the impact of prenatal diagnosis on outcomes has been variably characterized. We sought to further characterize this impact in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Methods: Retrospective analysis of demographic and outcomes data to time of Glenn operation (BDG) in infants enrolled in the NPC-QIC database; eligibility includes SVD requiring S1P and survival to hospital discharge. Results: From 31 contributing surgical centers, 195 infants were enrolled in the NPC-QIC database and had data available to time of BDG (177) or interstage death (18). Median gestational age was 39 weeks (32-41), 65% were male, 64% had variants of hypoplastic left heart syndrome, 8% had major syndromes, and 12% had major non-cardiac organ system anomalies. A prenatal diagnosis (PD) of SVD was made in 147 (75%) and did not vary by race or ethnicity. While infants with PD had slightly lower gestational age at birth (p<0.001) and lower age at S1P, there were no significant differences in birth weight, presence of major syndromes or other organ system anomalies, APGAR scores, lowest pH prior to post-natal intervention, age at intubation, or presence of pre-operative risk factors. Those without PD were more likely to have significant atrioventricular valve regurgitation (p=.03) and acidosis (p=0.02), although rates of ventricular dysfunction, pre-operative mechanical ventilation and shock were similar between groups. In multivariate analysis, length of intubation was not associated with PD, and hospital length of stay was the same between groups (26 days). Interstage readmissions and deaths were not associated with PD. At time of BDG, those with PD were slightly younger (5.0 vs 5.4 months, p=0.03) and smaller (6.1 vs 6.5 kg, p=0.003) but had similar rates of post-operative complications and similar hospital length of stay. Conclusions: In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD had minor associations with pre-operative risk factors and no significant association with subsequent outcomes through time of BDG.

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