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

Authors
 Suk-Won Song  ;  Han Ki Park  ;  Young-Hwan Park  ;  Bum Koo Cho 
Citation
 Circulation Journal, Vol.73(3) : 516-522, 2009 
Journal Title
 Circulation Journal 
ISSN
 1346-9843 
Issue Date
2009
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.
Files in This Item:
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DOI
10.1253/circj.CJ-08-0324
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실) > 1. Journal Papers
Yonsei Authors
Park, Young Hwan(박영환)
Park, Han Ki(박한기) ORCID logo https://orcid.org/0000-0002-7472-7822
Song, Suk Won(송석원) ORCID logo https://orcid.org/0000-0002-9850-9707
Cho, Bum Koo(조범구)
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/103604
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