Improved survival in limited scleroderma-related pulmonary artery hypertension

Reportedly, patients with scleroderma-related pulmonary hypertension (SSc-PAH) respond poorly to new vasoactive drugs (NVD). Forty-nine SSc-PAH patients underwent right heart catheterization (RHC) and, according to NVD availability, divided as follows: Group 1 ( n = 23, from 1999 to 2004, poor availability), and Group 2 ( n = 26, from 2005 to 2010, good availability). Before diagnostic RHC, NVD had been given to 30 % of the patients in Group 1, and 58 % of those in Group 2 ( p = 0.049). At diagnosis, patients in Group 1 had greater heart dilatation ( p < 0.01), higher mean pulmonary artery pressure ( p < 0.05), lower pulmonary artery capacitance ( p < 0.05), and lower carbon monoxide lung diffusing capacity (DLco, p < 0.05) than those in Group 2. At a median follow-up time of 15.5 months, DLco further decreased in Group 1 ( p < 0.05), whereas cardiac index increased in Group 2 ( p < 0.05). At 36 months of follow-up, 72.4 % of the patients in Group 2 were still alive as opposed to 30.4 % in Group 1 ( p = 0.02). In multivariate analysis, DLco and mixed venous oxygen saturation (SvO 2 ) were independent predictors of survival. A value of DLco <7.2 mL/mmHg/min was associated with a hazard ratio (HR) of 5.3 ( p < 0.001); for SvO 2 <63.8 %, the HR was 3.7 ( p < 0.01).NVD have beneficial effects in patients with SSc-PAH. Both DLco and SvO 2 are predictors of survival and may assist in planning treatment.