Staged reconstruction for hypoplastic left heart syndrome. Contemporary results

Annals of Surgery
Edward L Bove, T R Lloyd


The authors review their experience with staged reconstructive surgery for hypoplastic left heart syndrome (HLHS) and assess current outcome for this condition. Once considered a uniformly fatal condition, the outlook for newborns with HLHS has been altered dramatically with staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this improved outlook. The authors reviewed their experience with 158 consecutive patients undergoing stage 1 reconstruction with a Norwood procedure from January 1990 to August 1995. All patients had classic HLHS, defined as a right ventricular dependent circulation in association with atresia or severe hypoplasia of the aortic valve. There were 120 hospital survivors. Among the 127 patients considered at standard risk, survival was significantly higher than that for the 31 patients with important risk factors. Adverse survival was associated most strongly with significant associated noncardiac congenital conditions and severe preoperative obstruction to pulmonary venous return. Second-stage reconstruction with the hemi-Fontan procedure was performed in 106 patients, with 103 hospital survivors and one late death. Three of the...Continue Reading


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