Surgical treatment for hypoplastic left heart syndrome

Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically improved with either a protocol of staged reconstruction or cardiac transplantation. Currently, a significant shortage of suitable donor hearts restricts the applicability of transplantation for most newborns. At the University of Michigan, we have adopted a policy of staged reconstruction for all patients with hypoplastic left heart syndrome, reserving transplantation only for those unsuitable for reconstructive techniques. Between January 1990 and September 1998, 303 patients underwent the Norwood operation for classic hypoplastic left heart syndrome with an overall hospital survival of 76%. Among patients considered at standard risk, survival was significantly higher (86%) than that for those patients with important risk factors (42%), p = 0.0001. Adverse survival was most strongly associated with significant associated noncardiac congenital conditions (p = 0.008) and severe preoperative obstruction to pulmonary venous return (p = 0.03). Survival following second stage reconstruction with a hemi-Fontan or bidirectional Glenn procedure was 98%. The Fontan procedure has been completed in 117 of these patients with a hospital survival rate of 91%. Survival after the Fontan procedure improved significantly when the second stage of the reconstruction was completed with a hemi-Fontan procedure compared to a bidirectional Glenn, 98% vs 81%, p < .05. Among the patients considered at standard risk, actuarial survival was 70% at 5 years. The largest decrease in survival occurred in the first month of life and late deaths affected primarily those patients in the high risk group. Neurodevelopmental outcome studies demonstrated normal verbal and performance scores in the majority of patients. Among centers utilizing a protocol of transplantation, donor organ shortages have resulted in a mortality of approximately 25% while awaiting transplantation with 5 year survival rates for those actually receiving organs essentially equal to those for staged reconstruction. Staged reconstruction and transplantation have significantly improved the intermediate-term outlook for patients with hypoplastic left heart syndrome. Factors addressing improvements in early first stage survival following the Norwood would be expected to add significantly to an overall improved late outcome. Outcome following cardiac transplantation is limited by donor availability in addition to the late complications of infection, rejection, graft atherosclerosis, and lymphoproliferative disease.