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Improved survival in limited scleroderma-related pulmonary artery hypertension.
MedLine Citation:
PMID:  23361526     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Carlo Marini; Bruno Formichi; Carolina Bauleo; Claudio Michelassi; Roberta Pancani; Renato Prediletto; Massimo Miniati; Giosuè Catapano; Simonetta Monti; Francesca Mannucci; Antonio Tavoni; Anna D'Ascanio; Luigi Emilio Pastormerlo; Alberto Giannoni; Carlo Giuntini
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-30
Journal Detail:
Title:  Internal and emergency medicine     Volume:  -     ISSN:  1970-9366     ISO Abbreviation:  Intern Emerg Med     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101263418     Medline TA:  Intern Emerg Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Fondazione Regione Toscana "G. Monasterio", Via G. Moruzzi 1, Ghezzano, 56124, Pisa, Italy, marini@ifc.cnr.it.
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