Abstract

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.

© 2011 Wiley Periodicals, Inc.