Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries - Experience with 85 patients

Background-Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked hetero geneity of pulmonary blood supply. Traditional management has involved stag ed unifocalization of pulmonary blood supply. Our approach has been to perf orm early 1-stage complete unifocalization in almost all patients. Methods and Results-Since 1992, 85 patients with pulmonary atresia, VSD, an d MAPCAs have undergone unifocalization (median age, 7 months). Complete 1- stage unifocalization and intracardiac repair were performed through a midl ine approach in 56 patients, whereas 23 underwent unifocalization in a sing le stage with the VSD left open, and 6 underwent staged unifocalization thr ough sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. Conclusions-Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair du ring the same operation is achieved in two thirds of patients. There remain s room for improvement: actuarial survival 3 years after surgery is 80%, an d there is a significant rate of reintervention. These results must be appr eciated within the context of the natural history of this lesion: 65% of pa tients survive to 1 year of age and slightly >50% survive to 2 years even w ith surgical intervention.