Background Whether pulmonary hemodynamic parameters and functional capacity are associated with quality of life in patients with chronic thromboembolic pulmonary hypertension remains unknown. This study aimed to evaluate disease-specific quality of life using the emPHasis-10 questionnaire and assess its determinants in patients with chronic thromboembolic pulmonary hypertension with normalized pulmonary hemodynamics. Methods and Results This cross-sectional study included 187 health status assessments of 143 patients with chronic thromboembolic pulmonary hypertension (median age, 68 [58-75] years; men/women, 51/136; use of home oxygen therapy, 51 patients [27%]) after balloon pulmonary angioplasty with normalized mean pulmonary artery pressure <25 mm Hg at rest. Right heart catheterization was performed, followed by assessment of 6-minute walk distance and the emPHasis-10 questionnaire. The median pulmonary artery pressure and pulmonary vascular resistance were 18 (15-21) mm Hg and 2.2 (1.7-2.9) wood units, respectively. The median emPHasis-10 score was 14 (8-24), whereas the median 6-minute walk distance was 447 (385-517) m. Univariate linear regression analysis showed that the emPHasis-10 score was associated with 6-minute walk distance (β=-0.476 [95% CI -0.604, -0.348], P<0.001) and home oxygen therapy (β=0.214 [95% CI, 0.072, 0.356], P=0.003) but not with hemodynamic parameters. Multiple regression analysis revealed that a higher emPHasis-10 score was associated with lower 6-minute walk distance (β=-0.475 [95% CI, -0.631 to -0.319], P<0.001). Conclusions Health-related quality of life was associated with exercise capacity and the use of home oxygen therapy, but not with hemodynamic parameters, in patients with chronic thromboembolic pulmonary hypertension and normalized hemodynamics after balloon pulmonary angioplasty. Improvements in exercise capacity may lead to further improvements in quality of life.
Keywords: angioplasty; exercise tolerance; hemodynamics; hypertension; pulmonary; pulmonary artery; quality of life.