Korean Journal of Pediatrics 2009;52(2):194-198.
Published online February 15, 2009.
Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection)
Mi Ae Chu1, Byung Ho Choi1, Hee Joung Choi2, Yeo Hyang Kim3, 4, Joon Yong Cho4, Myung Chul Hyun1, Sang Bum Lee1
1Department of Pediatrics, College of Medicine, Kyungpook National University, Daegu, Korea
2Department of Pediatrics, Pochon CHA University, Gumi, Korea
3Department of Pediatrics, College of Medicine, Keimyung University, Daegu, Korea
4Department of Thoracic and Cardiovascular Surgery, College of Medicine, Kyungpook National University, Daegu, Korea
최근 총폐정맥 환류이상의 임상 경과 및 수술 결과 : 단일 대학병원에서의 경험(총폐정맥 환류이상의 최근 결과)
추미애1, 최병호1, 최희정2, 김여향3, 김근직4, 조준용4, 현명철1, 이상범1
1경북대학교 의과대학 소아과학교실
2포천중문의과대학 소아과학교실
3계명대학교 의과대학 소아과학교실
4경북대학교 의과대학 흉부외과학교실
Correspondence: 
Myung Chul Hyun, Email: mchyun@knu.ac.kr
Abstract
Purpose
: Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC.
Methods
: Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included.
Results
: Mean age at diagnosis was 28.1¡¾33.4 days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up.
Conclusion
: In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.
Key Words: Heart defects, Congenital, Pulmonary veins, Reoperation, Mortality


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