All issues > Volume 52(2); 2009

Original Article

Korean J Pediatr. 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 MA Chu¹, Byung Ho BH Choi¹, Hee Joung HJ Choi², Yeo Hyang YH Kim³, Joon Yong JY Cho⁴, Myung Chul MC Hyun¹, Sang Bum SB 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

Correspondence Myung Chul MC 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.

Keywords :Heart defects, Congenital, Pulmonary veins, Reoperation, Mortality