Abstract
Drug therapy for pulmonary hypertension associated to chronic lung disease is currently discouraged for the negative effects on gas exchange.We retrospectively evaluated blood gases and 6MWT in patients with severe PH associated with IPF or COPD during 12 months of treatment with Bosentan or Sildenafil. 22 pts with diagnosis of severe pre-capillary PH at right heart catheterization were evaluated including 10 IPF and 12 pts with COPD. Haemodynamical and long term respiratory function data were available in 20 pts: 5 pts with COPD treated with Bosentan, 5pts COPD with Sildenafil, 5 pts IPF treated with ERA and 5 pts with PDE5-inh. Blood gases measuring paO2 and paCO2, PFTs and 6MWT were analyzed at baseline, T6 and T12 months. A-aO2 gradient was calculated using alveolar gas equation. Baseline there were not any difference between haemodinamics and gas exchange profile between IPF and COPD (PAPm = 40mmHg vs 47mmHg), PaO2 58 vs 56 mm Hg, PaCO2 36 vs 37 mm Hg. In 5 IPF pts treated with ETRA A-aO2 gradient increased by 28% and 30% at T6 and T12; in 5 IPF pts exposed to PDE5-inib by 4% and by 6% at the same time points. The distance at 6MWT decreased in IPF-ERA group (- 41 mt T12) and it increased (+90 mt T12) in IPF- PDE5-inhib. In 5 COPD treated with ERA A-aO2 gradient increased by 13% and 22% at T6 and T12 respectively while in 5 COPD pts exposed to PDE5-inib did non significantly change. At T12 the distance at 6MWT increased + 36 mt and + 88 mt in COPD-ERA treated and in COPD-PDE5inib treated respectively.Long term treatment with sildenafil is not associated to detrimental effect on gas exchange both in IPF or in COPD with severe PH.
- © 2011 ERS