BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-known complication of systemic sclerosis (SSc). Doppler echocardiographic screening for the detection of PAH (by measuring right ventricular systolic pressure [RVSP]) is therefore recommended for all patients with SSc. However, the validity of RVSP as a predictor of mortality in patients with SSc is not well established. HYPOTHESIS: Doppler-determined PAH identifies a high-risk subset of patients with SSc with decreased survival. METHODS: We performed echocardiography in 155 consecutive patients with SSc between May 2005 and July 2006 and tested the value of an RVSP level of ≥36 mm Hg to predict mortality. Cox proportional hazards model was used to examine the individual relationship between each variable and the mortality rate. RESULTS: Tricuspid regurgitant jets for RVSP determination were quantified in 129 patients (82.6%), of which 47 (36.4%) had RVSP ≥36 mm Hg. The median follow-up time was 34 months. The 1-, 2-, and 3-year survival rates were significantly lower among SSc patients with RVSP ≥36 vs ≤36 mm Hg (82%, 78%, and 67% vs 98%, 90%, and 86%, respectively, P < 0.01 by Wilcoxon test). In a multivariate analysis including echocardiographic and clinical variables, only an RVSP ≥36 mm Hg and a New York Heart Association III/IV class were associated with increased mortality; the respective Cox hazard ratios were 2.22 (95% confidence interval [CI]: 1.01-4.89, P = 0.048) and 4.77 (95% CI: 2.09-10.90, P = 0.000). CONCLUSIONS: Our results indicate that Doppler RVSP identifies a high-risk subset and supports the use of Doppler RVSP as a screening test in patients with SSc who may warrant early treatment of their PAH.
BACKGROUND:Pulmonary arterial hypertension (PAH) is a well-known complication of systemic sclerosis (SSc). Doppler echocardiographic screening for the detection of PAH (by measuring right ventricular systolic pressure [RVSP]) is therefore recommended for all patients with SSc. However, the validity of RVSP as a predictor of mortality in patients with SSc is not well established. HYPOTHESIS: Doppler-determined PAH identifies a high-risk subset of patients with SSc with decreased survival. METHODS: We performed echocardiography in 155 consecutive patients with SSc between May 2005 and July 2006 and tested the value of an RVSP level of ≥36 mm Hg to predict mortality. Cox proportional hazards model was used to examine the individual relationship between each variable and the mortality rate. RESULTS: Tricuspid regurgitant jets for RVSP determination were quantified in 129 patients (82.6%), of which 47 (36.4%) had RVSP ≥36 mm Hg. The median follow-up time was 34 months. The 1-, 2-, and 3-year survival rates were significantly lower among SSc patients with RVSP ≥36 vs ≤36 mm Hg (82%, 78%, and 67% vs 98%, 90%, and 86%, respectively, P < 0.01 by Wilcoxon test). In a multivariate analysis including echocardiographic and clinical variables, only an RVSP ≥36 mm Hg and a New York Heart Association III/IV class were associated with increased mortality; the respective Cox hazard ratios were 2.22 (95% confidence interval [CI]: 1.01-4.89, P = 0.048) and 4.77 (95% CI: 2.09-10.90, P = 0.000). CONCLUSIONS: Our results indicate that Doppler RVSP identifies a high-risk subset and supports the use of Doppler RVSP as a screening test in patients with SSc who may warrant early treatment of their PAH.
Authors: A Tabarroki; D J Lindner; V Visconte; L Zhang; H J Rogers; Y Parker; H K Duong; A Lichtin; M E Kalaycio; M A Sekeres; S E Mountantonakis; G A Heresi; R V Tiu Journal: Leukemia Date: 2014-01-10 Impact factor: 11.528
Authors: Eric Hachulla; Pierre Clerson; Paolo Airò; Giovanna Cuomo; Yannick Allanore; Paola Caramaschi; Edoardo Rosato; Patricia E Carreira; Valeria Riccieri; Marta Sarraco; Christopher P Denton; Gabriela Riemekasten; Maria Rosa Pozzi; Silvana Zeni; Carmen Marina Mihai; Susanne Ullman; Oliver Distler; Simona Rednic; Vanessa Smith; Ulrich A Walker; Marco Matucci-Cerinic; Ulf Müller-Ladner; David Launay Journal: Rheumatology (Oxford) Date: 2015-01-17 Impact factor: 7.580
Authors: Sebastian Greiner; Andreas Jud; Matthias Aurich; Christoph Geisenberger; Lorenz Uhlmann; Thomas Hilbel; Meinhard Kieser; Hugo A Katus; Derliz Mereles Journal: PLoS One Date: 2018-01-19 Impact factor: 3.240