Abstract
BackgroundImproved 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 health-related quality of life and its determinants in patients with pulmonary arterial hypertension.MethodsHealth-related quality of life was evaluated in a cross sectional study of 155 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 health-related quality of life scores.ResultsPatients 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 better health-related quality of life scores, as did functional Class II versus Class III symptoms. Hemodynamic measurements, however, did not correlate with health-related quality of life scores. No differences in health-related quality of life were found between patients who were being treated with calcium channel antagonists, bosentan or continuously infused epoprostenol at the time of quality of life assessment.ConclusionHealth-related quality of life is severely impaired in patients with pulmonary arterial hypertension and is associated with measures of functional status. Specific associations with impaired health-related quality of life suggest potential areas for targeted intervention.
Highlights
Pulmonary arterial hypertension (PAH) is a devastating disease, characterized by progressive dyspnea and exercise limitation
No differences in summary physical or mental component health-related quality of life (HRQOL) scores were found between patients who were being treated with calcium channel antagonists, intravenous epoprostenol or bosentan at the time of HRQOL assessment (Figure 4)
We found HRQOL to be best for patients with idiopathic PAH and worst for those with systemic sclerosis, unrelated to the type of vasodilator therapy used, and to be correlated with functional but not hemodynamic assessments
Summary
Pulmonary arterial hypertension (PAH) is a devastating disease, characterized by progressive dyspnea and exercise limitation. Improved outcomes with expanding treatment options present the opportunity to consider additional end-points in choosing approaches to therapy, including factors influencing health-related quality of life. Little is known about the determinants of health-related quality of life (HRQOL) in patients with PAH. An improved understanding of these determinants will be essential as HRQOL becomes an important outcome in research on therapeutics for PAH [5,6].We studied health-related quality of life in a large population of PAH patients and performed an initial assessment of the clinical factors associated with better (or worse) HRQOL. 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. Comparatively little is known about health-related quality of life and its determinants in patients with pulmonary arterial hypertension
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