Sudden cardiac death in pulmonary arterial hypertension

Abstract

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.

Keywords: Pulmonary arterial hypertension; Sudden cardiac death.

Fig. 1

Arrhythmic and non-arrhythmic causes of sudden cardiac death. Created with Biorender.com.

Fig. 2

67 year-old woman with severe CTEPH and advanced right heart failure was admitted with sharp, tearing chest pain of abrupt onset. Non-invasive imaging with cardiac computed tomography (A, B) and transthoracic echocardiography (C) demonstrated a dissection of a dilated pulmonary artery. chronic thrombo-embolic pulmonary hyperetension.