Outcome of infants with hypoplastic left heart and Turner syndromes

Objective: To report the obstetric and neonatal outcomes of ten infants wit h hypoplastic left heart syndrome in association with Turner syndrome. Methods: The pediatric Cardiovascular Surgery database at the University of Michigan was searched from 1990 to 1997, and obstetric and neonatal record s of neonates with hypoplastic left heart syndrome and Turner syndrome were reviewed. Results: There were 406 cases of hypoplastic left heart syndrome admitted d uring 8 years, of which ten (2.5%) also had Turner syndrome. Nine infants w ere delivered at term and one at 36 weeks. The mean (+/- standard deviation [SDI]) gestational age at delivery was 38 +/- 1.2 weeks, and mean (+/-SD) birth weight tvas 2991 +/- 438 g. Delivery was vaginal in all cases, and no infant had an Apgar score at 5 minutes less than 7. Karyotype was 45, X in seven cases, and 45, X mosaic in three. Most infants had dysmorphic featur es at birth. All ten infants had first-stage reconstruction surgery for hyp oplastic left heart syndrome. Only two survived and underwent second-stage palliation; both are alive currently, although with significant medical pro blems. Conclusion: For infants with hypoplastic left heart and Turner syndromes, r egular obstetric management appears appropriate. Although staged reconstruc tion surgery has improved survival for neonates with isolated hypoplastic l eft heart syndrome, for those with Turner syndrome, survival appears marked ly reduced. (Obstet Gynecol 1999;93: 532-5. (C) 1999 by The American Colleg e of Obstetricians and Gynecologists.).