Complications Leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension

Abstract

SummaryPulmonary 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 PAHtargeted 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. Key words: pulmonary hypertension; pulmonary artery dissection; pulmonary artery rupture; left main compression syndrome. [Respir Care 2013;58(7):1246 -1254.

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…Mechanical complications in PAH patients are usually related to progressive dilatation of the PA and include PA aneurysms, rupture and dissection and compression of different intrathoracic structures such as the left main coronary artery, pulmonary veins, main bronchi and the recurrent laryngeal nerves [279][280][281]. Symptoms and signs are not specific and are variable according to the different complications, including chest pain (angina-like or not), dyspnoea, regional lung oedema and sudden death.…”

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…Powikłania mechaniczne u chorych z PAH są zwykle związane z postępującym poszerzeniem PA i obejmują powstanie tętniaka PA, jego pęknięcie, rozwarstwienie, ucisk na różne struktury w klatce piersiowej, takie jak pień lewej tętnicy wieńcowej, żyły płucne, oskrzela główne i nerwy krtaniowe wsteczne [279][280][281]. Objawy podmiotowe i przedmiotowe nie są specyficzne i są zmienne w zależności od różnych powikłań; może występować ból w klatce piersiowej (o charakterze stenokardialnym lub nie), duszność, regionalny obrzęk płuc i nagły zgon.…”

Pulmonary hypertensive vascular changes in lungs of patients with sudden unexpected death. Emphasis on congenital heart disease, Eisenmenger syndrome, postoperative deaths and death during pregnancy and postpartum

“…Left main coronary artery compression syndrome, pulmonary artery dissection, pulmonary artery rupture and severe haemoptysis are reported as complications leading to sudden death encountered more often in patients with PH 23 24. We had none of these complications in our series.…”