Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
OBJECTIVE
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.


METHODS
This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20).


RESULTS
For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P < .05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P < .01).


CONCLUSIONS
The data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.
['Mainwaring|Richard D|RD|', 'Patrick|William L|WL|', 'Roth|Stephen J|SJ|', 'Kamra|Komal|K|', 'Wise-Faberowski|Lisa|L|', 'Palmon|Michal|M|', 'Hanley|Frank L|FL|']
D000465:Algorithms D001011:Aorta D001028:Aortopulmonary Septal Defect D005260:Female D006343:Heart Septal Defects D006801:Humans D007223:Infant D007231:Infant, Newborn D008297:Male D011651:Pulmonary Artery D018633:Pulmonary Atresia D011664:Pulmonary Valve D012189:Retrospective Studies
congenital heart disease pulmonary artery pulmonary valve