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 (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 and SvO2 are predictors of survival and may assist in planning treatment.
Fondazione Regione Toscana "G. Monasterio", Via G. Moruzzi 1, Ghezzano, 56124, Pisa, Italy, marini@ifc.cnr.it.
Carlo
C
Marini
Bruno
B
Formichi
Carolina
C
Bauleo
Claudio
C
Michelassi
Roberta
R
Pancani
Renato
R
Prediletto
Massimo
M
Miniati
Giosuè
G
Catapano
Simonetta
S
Monti
Francesca
F
Mannucci
Antonio
A
Tavoni
Anna
A
D'Ascanio
Luigi Emilio
LE
Pastormerlo
Alberto
A
Giannoni
Carlo
C
Giuntini
D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery; D045745:Scleroderma, Limited / Q000150:complications*; D015996:Survival Rate