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
BACKGROUND: Pulmonary 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.
METHODS: All 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%).
RESULTS: PH 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.
CONCLUSIONS: Most 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.