304 Impact of Health-Related Quality of Life on Survival in Pulmonary Arterial Hypertension

Abstract

Purpose: Health-related quality of life (HRQoL) is an important outcome measure in the assessment of chronic disease, especially those without known cure, such as pulmonary arterial hypertension (PAH). However little is known about HRQoL in PAH. We sought to characterize the relationship between HRQoL and outcomes in a cohort of patients with PAH. Methods and Materials: HRQoL was assessed using the Short-Form 36 (SF-36) in consecutive patients who presented to our pulmonary hypertension clinic. Demographic, clinical, and hemodynamic data were collected and patients were followed prospectively. The relationship between HRQoL and survival was assessed using Cox Proportional Hazards and Kaplan-Meier methods. Results: 99 patients with PAH (31 idiopathic PAH, 43 scleroderma-related PAH, 25 PAH related to other diseases) who completed the SF-36 survey were included. The majority of the cohort were Caucasian (n 70, 71%) women (n 72, 73 %), with a mean age of 57 13 years. Less than half were married (n 47, 48%), had graduated from college (n 33, 35%), or were actively employed (n 21, 22%). Most were in NYHA functional class III (n 45, 46%) and had a mean 6MWD of 346 121 meters. Hemodynamics revealed moderate PAH (mean RAP 9 6 mmHg, mean PAP 42 16 mmHg, mean CI 2.5 0.8 L/min/m2, and mean PVR 8.1 5.9 Wood units). During a follow-up period of 2.5 years, 25 subjects died. SF-36 scores showed significant impairments in all 8 domains compared to US norms. When controlling for disease severity by cardiac index in multivariable analysis, poor physical function was associated with an increased risk of death (HR 1.02 (95% CI 1.00-1.04, p 0.03 for every 1 unit decrease in PF score). Conclusions: These data suggest HRQoL is significantly impaired in patients with PAH. Further, HRQoL independently predicts survival in this cohort, emphasizing the role of HRQoL in disease outcome. Further studies are needed to characterize the relationship between HRQoL and outcomes and to identify interventions to address poor HRQoL in this population.