Complications leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension

Abstract

Pulmonary Arterial Hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. PAH-specific drug therapy led to improvement in clinical outcomes, and survival. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation.

Left Main Compression Syndrome, Pulmonary Artery Dissection, Pulmonary Artery Rupture and severe Hemoptysis are reported as complications leading to Sudden Cardiac Death (SCD), entity encountered more often in PAH patients.

The advent of PAH-targeted drug therapy has reduced referral for lung transplantation programs, however identification of severe complications need appropriate diagnostic management, rapid decision making and successful therapeutic approach, and once recognized, they might be indications for rapid registration on lung transplantation waiting lists. Multidisciplinary approach in PAH referral centers provide emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients.

The aim of the present manuscript is to review the complications leading to sudden death in PAH.