Background The relationship between pulmonary function abnormalities and quality of life (QOL) in patients with pulmonary arterial hypertension has not been well described.
Methods We studied pulmonary function data from 58 subjects with pulmonary arterial hypertension defined by mean pulmonary artery pressure > 25 mm Hg on right heart catheterization. Airway obstruction was defined as an FEV1 < 80% predicted with a normal total lung capacity (TLC). Restriction was defined as a TLC < 80% predicted. Diffusion impairment was defined as DLCO < 60% predicted. Exercise capacity was measured by 6-minute walk distance (6MWD). Airway-specific QOL was assessed using the revised Airways Questionnaire 20 (AQ20-R).
Results Causes of pulmonary hypertension included idiopathic, 17 (29%); connective tissue disease, 22 (38%); portal hypertension, 9 (16%); and other, 10 (17%). Airway obstruction was present in 18 (31%) subjects, restriction in 15 (26%), and impaired diffusion in 21 (36%). FEV1% predicted correlated significantly with both 6MWD (r = .44, p = .001) and airway-specific QOL (r = .31, p = .03). TLC correlated with 6MWD (r = .48, p = .001) but not QOL. Diffusion capacity did not correlate with either 6MWD or QOL. In a multivariable model adjusted for the presence of restriction and diffusion impairment, FEV1 was an independent predictor of airway-specific QOL (p = .01). Further adding 6MWD to the model did not significantly alter this relationship.
Conclusion Pulmonary function abnormalities are prevalent in patients with pulmonary arterial hypertension. Reduced FEV1 is associated with worse airway-specific QOL in pulmonary arterial hypertension even after taking exercise capacity into account.
Funded by the Flight Attendant Medical Research Institute.
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