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Comparative Study
. 2013 Aug 1;188(3):365-9.
doi: 10.1164/rccm.201209-1640OC.

Causes and circumstances of death in pulmonary arterial hypertension

Adriano R Tonelli  1 , Vineesha Arelli, Omar A Minai, Jennie Newman, Nancy Bair, Gustavo A Heresi, Raed A Dweik
Affiliations
Comparative Study

Causes and circumstances of death in pulmonary arterial hypertension

Adriano R Tonelli et al. Am J Respir Crit Care Med. .

Abstract

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.

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Figures

<i>Figure 1.</i>
Figure 1.
Patient inclusion flow chart. PH = pulmonary hypertension.
<i>Figure 2.</i>
Figure 2.
Treatments provided to patients with pulmonary arterial hypertension (PAH) that died of worsening pulmonary hypertension (PH). *Number of patients is 81 because we excluded three patients with undetermined cause of death. All these patients received PH-specific therapies. Comorbidities include renal failure; cirrhosis; schizophrenia; coronary artery disease; bacteremia; and chronic gastrointestinal bleeding (arteriovenous malformation). Values are presented as n (%). ILD = interstitial lung disease; IV = intravenous; NYHA = New York Heart Association; P = patients; SQ = subcutaneous; Tx = treatment.
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