OBJECTIVETo evaluate the indication and operative program of ventricular septal defect with pulmonary atresia (VSD-PA).

METHODSFrom June 1984 to March 2005, there were 32 patients with VSD-PA, which underwent 33 operations. Among them, 15 were males and 17 were females. The ages ranged from 6 months to 9 years. There were 9 patients with aortopulmonary collateral arteries. The operations included central aorta-pulmonary shunts 3 cases, one stage complete repair 27 cases and one stage unifocalization with VSD open 2 cases.

RESULTSThere were early 5 death including one death after aorta-pulmonary shunt and 4 deaths after one stage complete repair. The causes of death were severe low cardiac output (2 patients), respiratory function failure (1 patient), multiorgan function failure (1 patient) and severe infect (1 patient). Twenty-one patients were followed up from 3 months to 15.5 years. Heart function (NYHA) was class I or II in 19 cases and class III or IV in 2 cases.

CONCLUSIONSThe operative indication and methods mainly depend on the developing of pulmonary arteries and aortopulmonary collateral arteries. Completely surgical repair of patients with VSD-PA can be achieved with acceptable mortality.