Causes and circumstances of death in pulmonary arterial hypertension

Am J Respir Crit Care Med. 2013 Aug 1;188(3):365-9. doi: 10.1164/rccm.201209-1640OC.


Rationale: The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH).

Objectives: We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH.

Methods: All deaths of patients with pulmonary hypertension (PH) followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the PH team. A total of 84 patients with PAH (age 58 ± 14 yr; 73% females) who died between June 2008 and May 2012 were included.

Measurements and main results: PH was determined to be the direct cause of death (right heart failure or sudden death) in 37 (44%) patients; PH contributed to but did not directly cause death in 37 (44%) patients; and the death was not related to PH in the remaining cases (n = 7; 8.3%). In three (3.6%) patients the final cause of death could not be adequately assessed. Most patients died in a healthcare environment and most received PH-specific therapies. In our cohort, 50% of all patients with PAH and 75.7% of those who died of right heart failure received parenteral prostanoid therapy. Less than half of patients had advanced healthcare directives.

Conclusions: Most patients with PAH in our cohort died of their disease; however, right ventricular failure or sudden death was the sole cause of death in less than half of patients.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cause of Death*
  • Cross-Sectional Studies
  • Death, Sudden / epidemiology
  • Death, Sudden / etiology*
  • Familial Primary Pulmonary Hypertension
  • Female
  • Heart Failure / etiology*
  • Heart Failure / mortality
  • Hospital Mortality / trends
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / mortality*
  • Incidence
  • Male
  • Middle Aged
  • Ohio / epidemiology
  • Prospective Studies
  • Risk Assessment*
  • Risk Factors