Functional single ventricle with extracardiac total anomalous pulmonary venous connection
Functional single ventricle with extracardiac total anomalous pulmonary venous connection
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DOI：
10.1016/j.ejcts.2009.02.060
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发表时间：
2009-07-01
影响因子：
3.4
通讯作者：
Sakamoto, Kisaburo
中科院分区：
文献类型：
--
作者：
Nakata, Tomohiro;Fujimoto, Yoshifumi;Sakamoto, Kisaburo
目的：本研究旨在评价功能性单心室和心外完全性肺静脉异位引流（TAPVC）的外科修复方法。方法：1998年1月至2007年12月，连续26例患者接受了心外TAPVC的手术修复。他们的特征如下：中位年龄，34（范围0-744）天;中位体重3.2（范围2.0-9.6）kg;心上型TAPVC，11例;心下型，5例;混合型，10例;右心房异构，24例;肺动脉闭锁，16例;阻塞型TAPVC，17例。伴随手术包括9例患者的体-肺分流术，5例肺动脉束带术，1例心室-肺动脉分流术，1例诺伍德手术，9例双向Glenn手术和1例Fontan手术。结果如下：TAPVC修复后1年和5年的总生存率分别为58%（95%置信区间[CI]，39-77%）和54%（95% CI，34-73%）。在14例存活患者中（心上型9例;心下型4例;混合型1例），12例接受Fontan完成术; 1例接受双向Glenn术; 1例正在等待双向Glenn术。未发生吻合口狭窄，但在9例患者中观察到复发性肺静脉口狭窄（PVS）。1年和5年时无PVS复发率均为56%（95% CI，34-78%）。6例患者因复发性PVS进行了再次手术;其中2例患者接受了Fontan完成术，但3例双侧和多发性PVS患者的手术失败。经考克斯多因素回归分析，混合TAPVC（p = 0.001，风险比，13.4; 95% CI，2.8-64.4）是死亡率和房室瓣返流的风险因素，需要在姑息期进行手术干预。（p = 0.024，风险比，23.4; 95% CI，1.5-363.4）是PVS复发的风险因素。结论：功能性单心室合并心上或心下TAPVC手术治疗的中期效果是可以接受的。混合性TAPVC和重度房室瓣返流患者的手术治疗前景不乐观，但可以改进。(C)2009年欧洲胸外科协会。Elsevier B. V.出版，保留所有权利。
Objective: The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). Methods: Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0-744) days; median weight 3.2 (range 2.0-9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. Results: The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39-77%) and 54% (95% CI, 34-73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34-78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p = 0.001, hazard ratio, 13.4; 95% CI, 2.8-64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p = 0.024, hazard ratio, 23.4; 95% CI, 1.5-363.4) was a risk factor for recurrent PVS. Conclusions: The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved. (C) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.