CURRENT STATUS OF STAGED RECONSTRUCTION FOR HYPOPLASTIC LEFT-HEART SYNDROME

Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically altered wit h staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this i mproved outlook. At the University of Michigan, 253 patients underwent the Norwood operation for classic hypoplastic left heart syndrome bet ween January 1990 and November 1997. Hospital survival was 76%. Among patients considered at standard risk, survival was significantly highe r (86%) than that for those patients with important risk factors (42%, p = 0.0001). Adverse survival was most strongly associated with signi ficant associated noncardiac congenital conditions (p, = 0.008) and se vere preoperative obstruction to pulmonary venous return (p = 0.03). S urvival following second-stage reconstruction with a hemi-Fontan or bi directional Glenn procedure was 97%. The Fontan procedure has been com pleted in 94 of these patients with a hospital survival rate of 88%. S urvival after the Fontan procedure improved significantly when the sec ond stage of the reconstruction was completed with a hemi-Fontan proce dure compared to a bidirectional Glenn (98% vs 81%, p = 0.05). Among t he 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 ver bal and performance scores in the majority of patients. Staged reconst ruction has significantly improved the intermediate-term outlook fur p atients with hypoplastic left heart syndrome. Factors addressing impro vements in early first-stage survival would be expected to add signifi cantly to an overall improved late outcome.