伴有 VSD 和 LVOTO 的 ccTGA 中的限制性 LV-PA 导管

IF 2.3 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
A. Moiroux-Sahraoui, N. Derridj, R. Gaudin, M. Pontailler, P. Vouhe, D. Bonnet, O. Raisky
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引用次数: 0

摘要

导言先天性大动脉转位(ccTGA)是一种罕见、复杂且具有挑战性的结构性心脏病。治疗方法仍存在争议,尤其是对伴有左心室流出道梗阻(LVOTO)的患者。由于伴有左心室流出道梗阻的ccTGA的自然病史总体上非常令人满意,而且增加肺下左心室(LV)压力负荷的干预措施与三尖瓣反流(TR)和系统性右心室(RV)功能障碍的减少有关,因此我们开始使用限制性左心室-肺动脉(LV-PA)导管,但不关闭室间隔缺损(VSD),作为ccTGA/VSD/LVOTO生理修复的一部分。方法1979年至2024年期间,内克尔病童医院(法国巴黎)连续为9名ccTGA/非限制性VSD/LVOTO患者进行了不关闭VSD的限制性LV-PA导管置入术。结果6名患者(66.7%)肺动脉狭窄,3名患者(33.3%)肺动脉闭锁。手术时的年龄和体重中位数分别为 5 岁(IQR:16.6)和 18 公斤(IQR:36.5)。五名(55.6%)患者曾接受过手术姑息治疗。没有患者在术后死亡,中位随访时间为 9.8(IQR:13.4)年。没有患者因完全性房室传导阻滞而需要植入永久起搏器。10年后,再干预的自由度为62.5%(95% CI:22.9-86.1)。晚期再介入包括左心室-左心室导管置换术(3 例)、左心室-左心室支架植入术(1 例)和单半心室修复术(半塞宁、格伦、拉斯特利,1 例)。最后一次随访时,只有一名患者的体力活动明显受限(NYHA III 级)。没有患者出现房性或室性心律失常。结论我们认为,在解剖修复风险过高和/或不切实际的情况下,不关闭 VSD 的限制性 LV-PA 导管置入术是一种值得采用的双心室替代策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Restrictive LV-PA conduit in ccTGA with VSD and LVOTO

Introduction

Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO.

Objective

To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure.

Methods

Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France).

Results

Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10 years. Late reintervention consisted in LV-PA conduit replacement (n = 3), PA stenting (n = 1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n = 1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation.

Conclusion

We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical.

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来源期刊
Archives of Cardiovascular Diseases
Archives of Cardiovascular Diseases 医学-心血管系统
CiteScore
4.40
自引率
6.70%
发文量
87
审稿时长
34 days
期刊介绍: The Journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles and editorials. Topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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