Abstract
Idiopathic pulmonary arterial hypertension is a debilitating disease that leads to right ventricular heart failure and death. There is limited research on how this disease affects patients. The purpose of this study was to describe differences in health status, health-related quality of life, and psychological states among functional classes along with predictors of health-related quality of life. A convenience sample of 104 participants with idiopathic pulmonary arterial hypertension was recruited. Participants completed a sociodemographic and clinical data form; Medical Outcomes Study Short-Form 36, US Cambridge Pulmonary Hypertension Outcome Review, and Profile of Mood States surveys. The mean age was 51.9 (SD, 16.6) years, and 81 (78%) were female. Patients with functional class IV had significantly worse self-reported physical health status, health-related quality of life, and psychological distress than the other functional classes. Predictors of health-related quality of life were role-emotional (Medical Outcomes Study Short-Form 36), symptoms and activity (US Cambridge Pulmonary Hypertension Outcome Review), depression (Profile of Mood States), employment, and diuretic and oxygen use, accounting for 86% of the variance. These factors need to be monitored and assessed with patients with idiopathic pulmonary arterial hypertension especially as functional class increases. Effective symptom management is necessary to reduce the negative impact on health-related quality of life. A case study is presented to illustrate these points.