Improved outcomes with expanding treatment options for patients with pulmonary arterial hypertension present the opportunity to consider additional end-points in approaching therapy, including factors that influence health-related quality of life. However, comparatively little is known about quality of life and its determinants in patients with pulmonary arterial hypertension. The purpose of this study was to evaluate health-related quality of life in adults with pulmonary arterial hypertension, and identify factors associated with better or worse status.
Health-related quality of life was evaluated in 216 outpatients with pulmonary arterial hypertension using generic and respiratory-disease specific measurement tools. Most patients had either World Health Organization functional Class II or III symptoms. Demographic, hemodynamic and treatment variables were assessed for association with quality of life scores.
Patients with pulmonary arterial hypertension suffered severe impairments in both physical and emotional domains of health-related quality of life. Patients with idiopathic (“primary”) pulmonary arterial hypertension had the best, and those with systemic sclerosis the worst health-related quality of life. Greater six-minute walk distance correlated with improved quality of life scores, as did functional Class II versus Class III symptoms. Hemodynamic measurements, however, did not correlate with quality of life scores. No differences in health-related quality of life were found between patients treated with calcium channel antagonists, bosentan or continuously infused epoprostenol.
Health-related quality of life is severely impaired in patients with pulmonary arterial hypertension and is associated with measures of functional status.
Specific determinants of impaired quality of life suggest areas for targeted intervention.
Darren Taichman, Consultant fee, speaker bureau, advisory committee, etc. Advisory board or speaker bureau for Actelion, Pfizer and CoTherix.