From April 1986 to September 1988, 12 patients with double- or common-inlet left ventricle and left anterior rudimentary right ventricle underwent septation while 17 patients with double- or common-inlet left or right ventricle underwent the Fontan operation. In the septation group, three patients who had pressure gradients ranging from 10 to 85 mm Hg between the left ventricle and the aorta underwent enlargement of the outlet foramen, and all survived. One of the 12 patients had a common atrioventricular valve that was repaired by separating the atrioventricular valve by the procedure used for atrioventricular septal defect. One had complete heart block before septation and the other one had it after separation. One 17-year-old woman, who had the smallest left ventricle (168% of normal), died in the hospital (mortality, 8.3%). In the Fontan group, one patient who died in the hospital (5.9%) had high pulmonary resistance of 4.4 U/m2, and one late death (5.9%) occurred in a patient who had complete heart block and a high mean pulmonary arterial pressure of 20 mm Hg. Because of suprasystemic pressure in the pulmonary ventricle, two patients had immediate takedown of the septation repair and substitution of the Fontan repair. Although right atrial pressure was almost equal in both groups after operation, the cardiac index was significantly higher in the septation group than in the Fontan repair group (p less than 0.01). These data suggest that patients who are candidates for either septation or Fontan repair might fare better with septation.