Surgical management of aortopulmonary septal defect.

Fourteen patients with malseptation of the aortopulmonary trunk underwent operative repair from 1978 to 1988. Age ranged from 1 year to 35 years (mean age, 9.6 years). Five patients had type I, 6 had type II, and 3 had type III lesions. The hemodynamic disturbance in all patients was the consequence of a large left-to-right shunt (mean pulmonary/systemic flow ratio, 2.38:1) with increased pulmonary vascular resistance (mean value, 4.47 units/m2). Our initial surgical experience with closure under cardiopulmonary bypass through the transaortic route in 3 patients and the transpulmonary approach in two patients resulted in 3 deaths. In the 9 subsequent patients, division and repair of the defect in the great vessels yielded uniformly good results. During follow-up, which ranged from 3 months to 2 years, all 11 survivors had good clinical improvement and none showed residual defects on restudy. Pulmonary artery pressure and pulmonary vascular resistance decreased in all patients except 1.