Septation of the univentricular heart with left anterior subaortic outlet chamber

Surgical repair by ventricular septation was accomplished in 16 patients with univentricular heart of the left ventricular type, two atrioventricular (AV) valves, and a left anterior subaortic outlet chamber. There were seven hospital deaths (44%; CL 29% to 60%), six from low cardiac output, including each of five patients with outlet foramen obstruction (100%; LC 68% to 100%), In the subset of 11 patients with unobstructed outlet foramen, there were only two deaths (18%; CL 6% to 38%), both in patients with small ventricular size. Multivariate analysis in the group of 16 patients indicates that small ventricular size and again the presence of outlet foramen obstruction are incremental risk factors for hospital death. No late deaths occurred among nine survivors followed between 2 months and 4 years (median = 1.5 years). Only one patient was taking cardiac medication and each was in New York Heart Association Functional Class I. Late postoperative exercise testing in seven showed good exercise tolerance, although values were below normal for children of similar age. Planned initial palliation, appropriate timing to repair, appropriate patient selection, and improving surgical methods should reduce hospital mortality further. The late functional results are encouraging and support continuation of this surgical procedure in patients with suitable anatomy.