Current Status

Abstract

Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically altered with staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this improved outlook. At the University of Michigan, 253 patients underwent the Norwood operation for classic hypoplastic left heart syndrome between January 1990 and November 1997. Hospital survival was 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 97%. The Fontan procedure has been completed in 94 of these patients with a hospital survival rate of 88%. 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 = 0.05). 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. Staged reconstruction has significantly improved the intermediate-term outlook for patients with hypoplastic left heart syndrome. Factors addressing improvements in early first-stage survival would be expected to add significantly to an overall improved late outcome.