Anesthesia for hypoplastic left heart syndrome: use of high-dose fentanyl in 30 neonates.

The anesthetic management of the first stage palliative surgical repair of hypoplastic left heart syndrome with a high-dose fentanyl (50 micrograms/kg)-pancuronium (0.14 mg/kg) anesthetic technique is described in 30 neonates. Previously this severe form of congenital heart disease carried a 100% rate of mortality. The pathophysiology that leads to this mortality is explained and related to the surgical procedure utilized for palliation. The major problem encountered intraoperatively was control of pulmonary blood flow. Management of the effect of anesthesia and intraoperative events on pulmonary blood flow was important because the rapid flux in pulmonary vascular resistance during the postnatal and perioperative period is the major destabilizing factor in these neonates. Ventricular fibrillation with surgical manipulation was a minor problem in the present series but was a major problem in an earlier series where a low dose narcotic-nitrous oxide technique was used. Ventricular fibrillation probably occurred due to the relative coronary insufficiency resulting from the anatomy present in this syndrome.