Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection).
10.3345/kjp.2009.52.2.194
- Author:
Mi Ae CHU
1
;
Byung Ho CHOI
;
Hee Joung CHOI
;
Yeo Hyang KIM
;
Gun Jik KIM
;
Joon Yong CHO
;
Myung Chul HYUN
;
Sang Bum LEE
Author Information
1. Department of Pediatrics, College of Medicine, Kyungpook National University, Daegu, Korea. mchyun@knu.ac.kr
- Publication Type:Original Article
- Keywords:
Heart defects;
Congenital;
Pulmonary veins;
Reoperation;
Mortality
- MeSH:
Drainage;
Early Diagnosis;
Follow-Up Studies;
Heart;
Hospital Mortality;
Humans;
Prognosis;
Pulmonary Veins;
Reoperation
- From:Korean Journal of Pediatrics
2009;52(2):194-198
- CountryRepublic of Korea
- Language:Korean
-
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+/-33.4 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.
