Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection)

Chu, Mi Ae; Choi, Byung Ho; Choi, Hee Joung; Kim, Yeo Hyang; ,; Cho, Joon Yong; Hyun, Myung Chul; Lee, Sang Bum

Original Article

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)

추미애^¹^, 최병호^¹^, 최희정^²^, 김여향^³^, 김근직^⁴^, 조준용^⁴^, 현명철^¹^, 이상범^¹

^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