Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries

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).

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).

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.