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在Fontan循环衰竭儿童中建立经导管开窗术:重点关注心外管道连接

Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection.

作者信息

Rupp Stefan, Schieke Christin, Kerst Gunter, Mazhari Nona, Moysich Axel, Latus Heiner, Michel-Behnke Ina, Akintuerk Hakan, Schranz Dietmar

机构信息

Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.

Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Vienna, Austria.

出版信息

Catheter Cardiovasc Interv. 2015 Dec 1;86(7):1189-94. doi: 10.1002/ccd.26042. Epub 2015 Jun 29.

DOI:10.1002/ccd.26042
PMID:26119855
Abstract

OBJECTIVES

We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients.

BACKGROUND

In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure.

METHODS AND RESULTS

From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.2 years, interquartile range (IQR) 2.7-3.7 years)) with failing Fontan circulation and exceeding systemic venous pressure. In 16 patients, the procedure was performed for acute postoperative failure 1-24 days after surgery. After perforation of the conduit and atrial wall by a Brockenbrough needle and gradual balloon dilation, premounted stents were expanded to create a diabolo configuration with flaring stent edges, leaving a slight but definitive central waist. The procedure resulted in regression of pleural effusions and a significant decrease in systemic venous pressure. Clinical improvement was observed in 16 of the 19 treated patients. Follow-up demonstrated sustained fenestration in 85% of treated patients for at least 24 months.

CONCLUSION

Transcatheter creation of a Fontan fenestration is a safe approach despite the anatomic gap between the extracardiac conduit cavity and the atrial wall. Stent implantation allows defining the diameter of the fenestration, reduces spontaneous occlusion, and ensures sustained clinical improvement.

摘要

目的

我们报告一种经导管技术的经验,该技术绕过肺部,从而改善单心室前负荷并减轻Fontan患者的静脉淤血。

背景

在缺乏专门为肺循环供血的电源且经肺压力梯度显著升高的情况下,Fontan循环开窗术是通过减轻过高的体静脉压力来改善患者血流动力学的一种选择。

方法与结果

2005年至2011年,对19例(中位年龄3.2岁,四分位间距(IQR)2.7 - 3.7岁)Fontan循环功能衰竭且体静脉压力过高的患者进行了22次经导管开窗术,无任何重大并发症。16例患者在术后1 - 24天因急性术后功能衰竭接受该手术。使用Brockenbrough针穿通管道和心房壁并逐步进行球囊扩张后,展开预安装的支架以形成带有扩张支架边缘的双锥形结构,留下轻微但明确的中央腰部。该手术导致胸腔积液消退,体静脉压力显著降低。19例接受治疗的患者中有16例临床症状改善。随访显示,85%的接受治疗患者的开窗至少持续了24个月。

结论

尽管心外管道腔与心房壁之间存在解剖间隙,但经导管创建Fontan开窗术是一种安全的方法。支架植入可确定开窗直径,减少自然闭塞,并确保临床持续改善。

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