Intrapartum course of fetuses with isolated hypoplastic left heart syndrome

Once considered universally fatal, the hypoplastic left heart syndrome is now being surgically treated in the newborn period. To help formulate an appropriate management plan for the labor and delivery of these patients, we reviewed the intrapartum course and immediate neonatal outcome of 13 fetuses with known hypoplastic left heart syndrome. Eleven of 13 patients underwent labor, and only one had an abnormal fetal heart pattern. There were no cases with meconium staining of the amniotic fluid. All patients with spontaneous or induced labor were delivered vaginally. There were no Apgar scores less than 8 at 5 minutes, and all umbilical cord blood pH values were greater than or equal to 7.20. All infants survived to undergo initial reconstructive surgery. We conclude that labor does not appear to be a high-risk situation for the fetus with this disorder. Routine intrapartum fetal heart rate monitoring can be used, oxytocin can be used as indicated, and cesarean section should be reserved for traditional obstetric indications.