Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort. Methods: Isolated TAPVC cases were id...

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Main Authors: Chun-Min Fu, Jou-Kou Wang, Chun-Wei Lu, Shuenn-Nan Chiu, Ming-Tai Lin, Chun-An Chen, Chung-I. Chang, Yih-Sharng Chen, Ing-Sh Chiu, Mei-Hwan Wu
Format: Article
Language:English
Published: Elsevier 2012-06-01
Series:Pediatrics and Neonatology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1875957212000526
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spelling doaj-5a286ba9f5754bb6a0026c955d594b292020-11-24T22:58:14ZengElsevierPediatrics and Neonatology1875-95722012-06-0153316417010.1016/j.pedneo.2012.04.002Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in TaiwanChun-Min Fu0Jou-Kou Wang1Chun-Wei Lu2Shuenn-Nan Chiu3Ming-Tai Lin4Chun-An Chen5Chung-I. Chang6Yih-Sharng Chen7Ing-Sh Chiu8Mei-Hwan Wu9Department of Pediatrics, Hsichu General Hospital, No. 25, Lane 442, Section 1, Jingguo Road, Hsinchu, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Surgery, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Surgery, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Surgery, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, and College of Medicine, National Taiwan University, No. 7, Chung-Shan South Road, Taipei, TaiwanTotal anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort. Methods: Isolated TAPVC cases were identified from our institutional database between 1995 and 2009. We reviewed the medical chart and conducted telephone interviews with those lost to follow-up. Results: There were 78 patients (52% male). The anomalous drainage sites were mainly supracardiac type (42.3%) and cardiac type (39.8%). Before operation, PV stenosis was found in 100% of infracardiac type, and in 69.7% of supracardiac type. Among the 75 patients undergoing operation, the surgical mortality was 9% (7/75). Perioperative arrhythmias (mainly of atrial origin) occurred in 35% of the patients. Of the 68 patients who survived the first operation, 28 (41%) developed pulmonary vein restenosis. Half of them progressed to severe PV stenosis, which required reintervention or resulted in mortality. Preoperative PV stenosis was the most significant predictor for postoperative PV restenosis and PV re-intervention. For the cohort, the 1-year and 5-year survivals were 78.9% and 74.2%, respectively, and the predictor for survival was again preoperative PV stenosis. Conclusion: The surgical mortality of isolated TAPVC is now low. Preoperative PV stenosis not only increased the risk of late PV restenosis and its reintervention, but also the overall mortality. The spectrum of PV drainage, per se, was not associated with worse outcome. PV restenosis remained the most important issue after correction of TAPVC.http://www.sciencedirect.com/science/article/pii/S1875957212000526arrhythmiaoutcomepulmonary vein stenosispulmonary venous obstructionsurgerytotal anomalous pulmonary venous connection
collection DOAJ
language English
format Article
sources DOAJ
author Chun-Min Fu
Jou-Kou Wang
Chun-Wei Lu
Shuenn-Nan Chiu
Ming-Tai Lin
Chun-An Chen
Chung-I. Chang
Yih-Sharng Chen
Ing-Sh Chiu
Mei-Hwan Wu
spellingShingle Chun-Min Fu
Jou-Kou Wang
Chun-Wei Lu
Shuenn-Nan Chiu
Ming-Tai Lin
Chun-An Chen
Chung-I. Chang
Yih-Sharng Chen
Ing-Sh Chiu
Mei-Hwan Wu
Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
Pediatrics and Neonatology
arrhythmia
outcome
pulmonary vein stenosis
pulmonary venous obstruction
surgery
total anomalous pulmonary venous connection
author_facet Chun-Min Fu
Jou-Kou Wang
Chun-Wei Lu
Shuenn-Nan Chiu
Ming-Tai Lin
Chun-An Chen
Chung-I. Chang
Yih-Sharng Chen
Ing-Sh Chiu
Mei-Hwan Wu
author_sort Chun-Min Fu
title Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
title_short Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
title_full Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
title_fullStr Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
title_full_unstemmed Total Anomalous Pulmonary Venous Connection: 15 Years' Experience of a Tertiary Care Center in Taiwan
title_sort total anomalous pulmonary venous connection: 15 years' experience of a tertiary care center in taiwan
publisher Elsevier
series Pediatrics and Neonatology
issn 1875-9572
publishDate 2012-06-01
description Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort. Methods: Isolated TAPVC cases were identified from our institutional database between 1995 and 2009. We reviewed the medical chart and conducted telephone interviews with those lost to follow-up. Results: There were 78 patients (52% male). The anomalous drainage sites were mainly supracardiac type (42.3%) and cardiac type (39.8%). Before operation, PV stenosis was found in 100% of infracardiac type, and in 69.7% of supracardiac type. Among the 75 patients undergoing operation, the surgical mortality was 9% (7/75). Perioperative arrhythmias (mainly of atrial origin) occurred in 35% of the patients. Of the 68 patients who survived the first operation, 28 (41%) developed pulmonary vein restenosis. Half of them progressed to severe PV stenosis, which required reintervention or resulted in mortality. Preoperative PV stenosis was the most significant predictor for postoperative PV restenosis and PV re-intervention. For the cohort, the 1-year and 5-year survivals were 78.9% and 74.2%, respectively, and the predictor for survival was again preoperative PV stenosis. Conclusion: The surgical mortality of isolated TAPVC is now low. Preoperative PV stenosis not only increased the risk of late PV restenosis and its reintervention, but also the overall mortality. The spectrum of PV drainage, per se, was not associated with worse outcome. PV restenosis remained the most important issue after correction of TAPVC.
topic arrhythmia
outcome
pulmonary vein stenosis
pulmonary venous obstruction
surgery
total anomalous pulmonary venous connection
url http://www.sciencedirect.com/science/article/pii/S1875957212000526
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