Pulmonary hypertension associated with congenital heart disease: Evolution and prognostic factors from the ITINERAIR registry

IF 2.2 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Archives of Cardiovascular Diseases Pub Date : 2025-09-01 DOI:10.1016/j.acvd.2025.06.033

Tarcile Albaret , Cohen Sarah , Sébastien Hascoët , Xavier Iriart , Claire Dauphin , Ali Houeijeh , Laurianne Le Gloan , Pamela Moceri , Fanny Dion , Yves Dulac , Hugues Lucron , Quentin Hauet , Pascal Amédro , Caroline Ovaert , Laurent Bonnemains , Pascale Maragnes , Hélène Bouvaist , Laurence Iserin , Damien Bonnet , Mathieu Albertini

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引用次数: 0

Abstract

Introduction

Patients with congenital heart disease are increasingly reaching adulthood, suggesting a growing exposure to complications, including pulmonary hypertension. Despite improved management, the onset of pulmonary hypertension is a turning point in the evolution of the disease and threatens the prognosis of patients.

Method

This is a prospective, multicenter cohort study from the ITINERAIR registry including children and adults with congenital heart disease and pulmonary hypertension followed from January 2014 to December 2018.

We classified patients into three groups according to the existence of a shunt, whether corrected or persistent, with or without Eisenmenger syndrome, in order to identify prognostic factors for each.

Results

The prognostic factors for major events in our study were a history of right heart failure and saturation level. The specific risk of mortality or heart and/or lung transplantation is determined by right atrial pressure.

Conclusion

The prognosis of patients with congenital heart disease complicated by pulmonary hypertension can be assessed using simple tests. Cardiac catheterization appears to play an essential role in the evaluation of this population.

Further studies with longer follow-up are needed to confirm our findings and lead to recommendations specific to this patient population.

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肺动脉高压与先天性心脏病相关：来自ITINERAIR登记的演变和预后因素

先天性心脏病患者越来越多地进入成年期，这表明他们越来越多地暴露于并发症，包括肺动脉高压。尽管管理有所改善，但肺动脉高压的发病是疾病发展的转折点，并威胁到患者的预后。方法：这是一项来自ITINERAIR注册中心的前瞻性多中心队列研究，包括2014年1月至2018年12月期间患有先天性心脏病和肺动脉高压的儿童和成人。我们根据分流的存在将患者分为三组，无论是矫正的还是持续的，有或没有艾森曼格综合征，以确定每一组的预后因素。结果本研究中主要事件的预后因素为右心衰史和心脏饱和度。死亡或心脏和/或肺移植的具体风险由右心房压决定。结论先天性心脏病合并肺动脉高压患者的预后可通过简单的检查来评估。心导管插入术似乎在这一人群的评估中起着至关重要的作用。需要进一步的研究和更长时间的随访来证实我们的发现，并提出针对这一患者群体的具体建议。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Archives of Cardiovascular Diseases 医学-心血管系统

CiteScore

4.40

自引率

6.70%

发文量

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.