title: Outcome of infants with hypoplastic left heart and Turner syndromes.
abstract: OBJECTIVE
  To report the obstetric and neonatal outcomes of ten infants with 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 records of neonates with hypoplastic left heart syndrome and Turner syndrome were reviewed.
  
  
  RESULTS
  There were 406 cases of hypoplastic left heart syndrome admitted during 8 years, of which ten (2.5%) also had Turner syndrome. Nine infants were delivered at term and one at 36 weeks. The mean (+/- standard deviation [SD]) gestational age at delivery was 38 +/- 1.2 weeks, and mean (+/-SD) birth weight was 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 features at birth. All ten infants had first-stage reconstruction surgery for hypoplastic left heart syndrome. Only two survived and underwent second-stage palliation; both are alive currently, although with significant medical problems.
  
  
  CONCLUSIONS
  For infants with hypoplastic left heart and Turner syndromes, regular obstetric management appears appropriate. Although staged reconstruction surgery has improved survival for neonates with isolated hypoplastic left heart syndrome, for those with Turner syndrome, survival appears markedly reduced.
authors.affiliation: Department of Obstetrics and Gynecology, University of Michigan School of Medicine, Ann Arbor 48109-0264, USA.
authors.forename: P M
authors.initials: PM
authors.lastname: Reis
authors.forename: M R
authors.initials: MR
authors.lastname: Punch
authors.forename: E L
authors.initials: EL
authors.lastname: Bove
authors.forename: C J
authors.initials: CJ
authors.lastname: van de Ven
mesh_terms: D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D007231:Infant, Newborn; D011247:Pregnancy; D011296:Prenatal Diagnosis; D016896:Treatment Outcome; D014424:Turner Syndrome / Q000150:complications* / Q000175:diagnosis