The hypoplastic left heart syndrome: evidence of preoperative myocardial and hepatic infarction in spite of prostaglandin therapy.

From February, 1983, to November, 1984, 11 infants with hypoplastic left heart syndrome were managed medically prior to operative intervention. Only 2 of the 11 infants appeared to be in mild distress at birth. Despite relatively normal Apgar scores, 9 of the infants were seen in shock. All patients were managed medically with fluid restriction, diuretics, and the institution of prostaglandin E1. There was a marked improvement in arterial pH from a mean value of 7.13 prior to prostaglandin to 7.42 following prostaglandin infusion. There was also a dramatic decrease in the serum creatinine level from a mean pretreatment level of 1.5 mg/dl to 0.7 mg/dl following infusion. Ten of the 11 infants were operated on at a mean age of 4 days with a Norwood or conduit procedure. Three infants who died at 3, 13, and 24 days of age had sustained myocardial infarctions prior to operation. Three patients also had multiple infarcts of the liver. Three patients are alive 23, 22, and 6 months after operation, and are growing and developing normally. The ability to maintain the infant with hypoplastic left heart syndrome in a stable hemodynamic condition has made surgical palliation a realistic option for this otherwise fatal anomaly. Nevertheless, preoperative infarctions of the heart and viscera continue to play a major role in mortality.