Drakopoulou M, Vlachakis P K, Gatzoulis K A, Giannakoulas G
First Department of Cardiology, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece.
First Cardiology Department, AHEPA University Hospital, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Int J Cardiol Congenit Heart Dis. 2024 Jul 6;17:100525. doi: 10.1016/j.ijcchd.2024.100525. eCollection 2024 Sep.
Pulmonary arterial Hypertension (PAH) is a progressive disease marked by significant morbidity and mortality due to pulmonary vasculopathy and right ventricular (RV) dysfunction. Despite advances in PAH medical therapies which have improved clinical outcomes and survival, patients continue to face severe complications, including a notable incidence of sudden cardiac death (SCD). The high arrhythmic burden, coupled with mechanical complications such as left main compression syndrome, pulmonary artery dissection, rupture, and severe hemoptysis, significantly contribute to the risk of SCD. Close monitoring and a meticulous diagnostic approach are essential for risk stratification and timely intervention, aiming to mitigate the risk of premature death in these patients. The aim of this review is to provide a comprehensive understanding of these risks and highlight strategies for improving patient outcomes through early identification, prevention and effective management.
肺动脉高压（PAH）是一种进行性疾病，因肺血管病变和右心室（RV）功能障碍而具有显著的发病率和死亡率。尽管PAH药物治疗取得了进展，改善了临床结局和生存率，但患者仍面临严重并发症，包括明显的心脏性猝死（SCD）发生率。高心律失常负担，再加上诸如左主支气管压迫综合征、肺动脉夹层、破裂和严重咯血等机械性并发症，显著增加了SCD的风险。密切监测和细致的诊断方法对于风险分层和及时干预至关重要，旨在降低这些患者过早死亡的风险。本综述的目的是全面了解这些风险，并强调通过早期识别、预防和有效管理来改善患者结局的策略。
