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


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.
Song
Suk-Won
SW
Department of Thoracic and Cardiovascular Surgery, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
Park
Han Ki
HK
Park
Young-Hwan
YH
Cho
Bum Koo
BK
D000293:Adolescent; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation; D017023:Coronary Angiography; D003326:Coronary Circulation; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery*; D012086:Reoperation / Q000706:statistics & numerical data; D012307:Risk Factors