RT Journal Article
SR Electronic
T1 Complications Leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension
JF Respiratory Care
FD American Association for Respiratory Care
SP 1246
OP 1254
DO 10.4187/respcare.02252
VO 58
IS 7
A1 Demerouti, Eftychia A
A1 Manginas, Athanassios N
A1 Athanassopoulos, George D
A1 Karatasakis, George T
YR 2013
UL http://rc.rcjournal.com/content/58/7/1246.abstract
AB 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 has improved 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, an event encountered more often in PAH patients. The advent of PAH-targeted drug therapy has reduced referral for lung transplantation; however, severe complications require rapid diagnosis, decision making, and possible registration on a lung transplantation waiting list. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. We review the complications leading to sudden death in PAH.