Obstetric management of 219 infants with hypoplastic left heart syndrome

OBJECTIVE: The objective of this study was to describe the obstetric parameters from 219 deliveries of infants with hypoplastic left heart syndrome. STUDY DESIGN: The Pediatric Cardiovascular Surgery Database at the University of Michigan was searched, and cases of neonates with the diagnosis of hypoplastic left heart syndrome were found, Obstetric records were then reviewed. RESULTS: One hundred sixty-one infants (74%) were delivered vaginally and 58 (26%) were delivered by the cesarean route. The mean gestational age at delivery was 38.9 weeks. Mean Apgar scores at 1 and 5 minutes were 7 and 8, respectively. Ninety percent were delivered at term and 10% were delivered before 37 weeks. The diagnosis of hypoplastic left heart syndrome was made antenatally in 82 cases (37%) and neonatally in 137 cases (63%). In the antenatal group the mean gestational age at diagnosis was 27 weeks. Karyotype analysis was performed in 32 of all cases (15%), with 8 fetuses revealing an abnormal karyotype. Seven cases were 45,X and 1 was trisomy 21. CONCLUSION: Staged reconstruction surgery has markedly improved survival for neonates born with isolated hypoplastic left heart syndrome. Our review suggests that, aside from determining the karyotype, no further obstetric interventions seem warranted. While awaiting spontaneous labor at term, the planned mode of delivery should be vaginal, with cesarean delivery performed for routine obstetric indications only.