Complete repair of pulmonary atresia with nonconfluent pulmonary arteries

From 1973 through 1979, 16 patients with pulmonary atresia, two normal-sized ventricles, and bilateral but nonconfluent pulmonary arteries underwent complete repair at the Mayo Clinic. Mean age at operation was 9.1 years (standard deviation [SD] 5.2 years). Sources of pulmonary flow were previous surgical shunts, patent ductus arteriosus, and discrete systemic-pulmonary arterial collaterals. The complete surgical repair included interruption of extracardiac shunts, closure of ventricular septal defect (15 patients), closure of atrial septal defect (3 patients), and establishment of right ventricular-pulmonary arterial continuity with a porcine-valved extracardiac conduit anastomosed to a transverse limb (T graft in 12 patients) or to a side-limb (Y graft in 4 patients). There were no operative deaths. Morbidity included reoperation for bleeding in 3 patients and compression of the prosthetic graft by the sternal closure in 1. The mean postrepair ratio of right ventricular peak systolic pressure to left ventricular peak systolic pressure (pRV/pLV) was 0.64 (SD, 0.23). Follow-up ranged from 12 to 72 months (means, 34.6 months; SD, 20.7 months). One patient died 12 months after operation (postrepair pRV/pLV, 1.3). Conduit obstruction has been proved in 4 patients, of whom 3 underwent reoperation for extracardiac conduit obstruction at 46, 47, and 48 months. The remaining 11 patients are alive and free of major symptoms.