Double combination therapy in patients with pulmonary arterial hypertension associated with connective tissue disease

Background: Pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) is a severe and progressive condition despite the availability of 3 specific classes of drugs: prostanoids (PROST), endothelin receptor antagonists (ERA) and phosphodiesterase-5 inhibitors (PDE5-I). Combination therapy (CT) has been proposed for patients with unsatisfactory response to monotherapy. Aim: To examine the effect of double CT in patients with PAH -CTD who do not achieve an adequate clinical response on monotherapy. Methods: Between October 1999 and December 2010, 48 PAH-CTD patients in WHO functional class III treated with monotherapy were included. At baseline and after 5±5 months on CT, all patients underwent 6-minute walk test (6MWT) and right-heart catheterization. Results: Mean age was 58±14 years, 85% females. Mean time from initiation of monotherapy to initiation of CT was 18±20 months. Forty (83%) patients received ERA+PDE5-I, 5 (10%) received PDE5-I+PROST and 3 (7%) received ERA+PROST. Four (8%) patients died before CT assessment. The table shows the haemodynamic and functional changes after CT. View this table: Conclusions: CT in CTD-PAH patients improves exercise capacity and haemodynamics. However 8% of patient die after an average of 3.2±1.5 months of CT testifying the persistent severity of the condition.