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Causes of Death in Pulmonary Hypertension

Nancy Blair

V. Arelli

Omar Minai


A. Tonelli

Raed Dweik

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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

Bair N, Arelli, V, Minai, O, Tonelli, A, Dweik, R

Cleveland Clinic, USA

BACKGROUNDPulmonary Arterial Hypertension (PAH) is a progressive disease involving the pulmonary vasculature which ultimately results in right heart failure (RHF) and death. As patients with PAH live longer as a result of advances in treatment and management, the cause of death may be from causes other than PAH.

METHODSAll deaths of PH patients, followed in the Cleveland Clinic Pulmonary Vascular Program, are prospectively reviewed by the PH team. A total of 87 patients (age 61 ± 14 years, 67% females) died and were reviewed between June 2008 and December 2010. PH was confirmed by right heart catheterization in all case. 4th World Symposium PH groups (n=81) were I (70%), II (5%), III (21%), IV (3%), and V (1%).

RESULTSPH was the direct cause of death in 33% and contributed in 52%. In 15% of cases death was not related to PH. When PH was not the direct cause of death, patients died of respiratory disease (other than PH; 32%), cardiovascular (other than RHF; 23%), cancer (11%), sepsis (13%), and miscellaneous (15%). Cause of death was unknown in 3 patients (6%). Death occurred in the intensive care unit (47%), regular nursing floor (25%), or outside the health care environment (28%). Patients received PO, intravenous, and inhaled PH specific therapies in 58%, 40% and 8%, respectively. Of the 26 patients with PH as a direct cause of death, 85% were on prostacyclin analogs. Mechanical ventilation or vasopressors were provided in 40% of the cases, respectively, meanwhile 18% received CPR and 3% received ECMO. Thirty-seven subjects (47%, n=78) had advanced health care directives. Forty-nine patients (72%, n=68) were receiving comfort care measures at the time of their death and fifty-two patients (74%, n=70) were DNR.

CONCLUSIONSMost of the PH patients died in a health care setting. Less than half had advance care directives. PH was the direct cause of death in only 33% of patients.