Abstract
The evolution of the present approach to the newborn with hypoplastic left heart is outlined. Preoperatively, maintenance of ductal patency with prostaglandin E 1 and balancing of systemic and pulmonary blood flow are essential. Operative details of the first-stage palliation and the definitive second-stage procedure are described. The more recent adoption of an intermediate-stage hemi-Fontan procedure is also described. Since January 1989, 151 patients have been treated using this three-stage approach, with 109 early survivors. Seventy-eight have undergone the hemi-Fontan operation with nine deaths (5 of whom came to this stage early nonelectively because of shunt failure or ventricular dysfunction). Twenty-seven of the 78 patients undergoing hemi-Fontan operation have subsequently undergone definitive Fontan procedures with no deaths.
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