Changes in Estimated Right Ventricular Systolic Pressure Predict Mortality and Pulmonary Hypertension in a Cohort of Scleroderma Patients
Shah AA, Chung SE, Wigley M, Wise RA, Hummers LK
Ann Rheum Dis. 2013;72:1136-1140
Study Summary
Pulmonary hypertension (PAH) is a feared complication of scleroderma and a leading cause of mortality in this disease. In this study, Shah and colleagues evaluated the factors that could predict PAH in a cohort of 613 patients with scleroderma. They found that the rate of increase in right ventricular systolic pressure (RVSP) as measured by annual echocardiograms was strongly associated with PAH and increased mortality. Specifically, patients who had increases ≥ 3 mm Hg on annual echocardiography had a 6-fold increased risk for PAH compared with patients whose RVSP remained stable. Furthermore, patients who had increases ≥ 3 mm Hg on annual echocardiography had approximately a 5-fold increased risk for mortality.
These associations persisted even after adjustment for other factors, including clinical characteristics and pulmonary function test results. The investigators concluded that the rate of increase in RVSP was a strong risk factor for PAH and mortality in patients with scleroderma.
Viewpoint
Although most rheumatologists treating patients with scleroderma are aware of the complication of PAH, these findings highlight that a single echocardiographic measurement of RVSP may be inadequate to assess risk for PAH. What remains to be seen is how these findings could guide additional diagnostic strategies, and ultimately therapy.
Shah and colleagues acknowledge that echocardiography is not a precise measure of RVSP and that right-heart catheterization is needed to formally diagnose PAH. Should patients who have an increasing RVSP on echocardiography undergo catheterization even if the RVSP is still within a normal range? And what treatments should be initiated if a patient has an increasing RVSP, with or without clear PAH? These questions will need to be answered in additional studies, but a take-home point from the current study is that serial echocardiograms may provide prognostic information beyond that possible with single measurements.
Abstract