Inspiratory muscle weakness contributes to exertional dyspnea in chronic thromboembolic pulmonary hypertension

Determination of potentially-reversible factors contributing to exertional dyspnea remains an unmet clinical need in chronic thromboembolic pulmonary hypertension (CTEPH). Therefore, we aimed to evaluate the influence of inspiratory muscle weakness (IMW) on exercise capacity and dyspnea during effort in patients with CTEPH. We performed a prospective cross-sectional study that included thirty-nine consecutive patients with CTEPH (48 +/- 15 yrs, 61% female) confirmed by right heart catheterization that underwent an incremental cardiopulmonary exercise test, 6-minute walk test and maximum inspiratory pressure (MIP) measurement. MIP < 70%(pred) was found in 46% of patients. On a multiple linear regression analysis, MIP was independently associated with 6MWD and <(V)over dot>O-2 (PEAK). Patients with MIP < 70% presented greater Delta(V)over dot>E/Delta(V)over dot>CO2 than those with MIP >= 70%. Additionally, they also presented stronger sensations of dyspnea throughout exercise, even when adjusted for ventilation. At rest and at different levels of exercise, mean inspiratory flow (V-T/T-I) was significantly higher in patients with MIP < 70%. In conclusion, IMW is associated with a rapid increase of dyspnea, higher inspiratory load and poor exercise capacity in patients with CTEPH.