Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries.

Abstract

BACKGROUND: There is no consensus on the long-term outcome after unifocalization in patients undergoing surgery for pulmonary atresia with ventricular septal defects (VSD) and major aortopulmonary collateral arteries (MAPCAs).

METHODS AND RESULTS: From 1988 to 2006, 40 patients (median age 8.5 months) underwent surgery for pulmonary atresia, VSD, and MAPCAs. The hospital mortality rate for the preparatory procedures was 1.2%; 17 patients had a complete repair (CR) at a median age of 3 years. Patients with a pulmonary artery index greater than 100 mm(2)/m(2) had a higher likelihood of CR. The overall survival rate 15 years after first operation in the CR group was 87.5%. Cox analysis demonstrated that increased number of MAPCAs (P=0.019, HR=1.666) was a significant predictor of poor survival, and CR (P=0.025, HR=0.141) was a significant predictor of favorable prognosis. On angiography, serial measurements of MAPCAs showed a significant decrease in size (from 5.2+/-2.9 to 4.1+/-2.9 mm after a mean of 20 months) (P<0.0001).

CONCLUSIONS: Long-term survival into adulthood can be achieved with an integrated approach. Late survival depends on the number of MAPCAs, and CR. Growth potential of unifocalized MAPCAs was not definite.