title: Causes and circumstances of death in pulmonary arterial hypertension.
abstract: BACKGROUND
  The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH).
  
  
  OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Adriano R
authors.initials: AR
authors.lastname: Tonelli
authors.forename: Vineesha
authors.initials: V
authors.lastname: Arelli
authors.forename: Omar A
authors.initials: OA
authors.lastname: Minai
authors.forename: Jennie
authors.initials: J
authors.lastname: Newman
authors.forename: Nancy
authors.initials: N
authors.lastname: Bair
authors.forename: Gustavo A
authors.initials: GA
authors.lastname: Heresi
authors.forename: Raed A
authors.initials: RA
authors.lastname: Dweik
mesh_terms: D002423:Cause of Death; D003430:Cross-Sectional Studies; D003645:Death, Sudden / Q000453:epidemiology / Q000209:etiology*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006333:Heart Failure / Q000209:etiology* / Q000401:mortality; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000401:mortality*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009820:Ohio / Q000453:epidemiology; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors