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,NVDhad 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), whereascardiac 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 (SvO2) 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 SvO2\63.8 %, the HR was 3.7 (p\0.01).NVD have beneficial effects in patients with SSc- PAH. Both DLco andSvO2 are predictors of survival andmay assist in planning treatment.