Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 188, Issue 3, Pages 365-369Publisher
AMER THORACIC SOC
DOI: 10.1164/rccm.201209-1640OC
Keywords
pulmonary hypertension; outcome assessment; cause of death
Categories
Funding
- CTSA KL2 from National Center for Research Resources, a component of the National Institutes of Health (NIH) [RR024990]
- NIH Roadmap for Medical Research
- NIH [HL081064, HL107147, HL095181, RR026231]
- Ohio Department of Development [BRCP 08-049]
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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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