Key result
Systemic sclerosis patients with a persistently positive exercise echocardiography are at an increased risk of developing resting pulmonary arterial hypertension over a mean 4-year follow-up.
Why the study?
Does a persistently positive exercise echocardiography predict the development of resting pulmonary arterial hypertension in patients with systemic sclerosis?
Does a persistently positive exercise echocardiography predict the development of resting pulmonary arterial hypertension in patients with systemic sclerosis?
While exercise echocardiography shows potential as a screening tool to identify systemic sclerosis patients at risk for developing resting pulmonary arterial hypertension, methodological limitations and lack of standardized definitions currently limit its clinical application.
In this issue of The Journal , Quinn, et al describe the utility of exercise echocardiography (EE) in the identification of patients with systemic sclerosis (SSc) deemed high risk for the development of pulmonary arterial hypertension (PAH)1. In a single-center observational study, the authors demonstrated that a significantly higher percentage of SSc patients with a persistently positive EE, as defined by an increase in right ventricular systolic pressure (RVSP) ≥ 20 mmHg with exercise, subsequently developed pulmonary hypertension (PH) compared with those who had persistently negative EE. The majority of patients with a baseline positive EE were found to have a persistently positive EE over time. However, interestingly, a proportion of those patients did not develop resting PAH. Similarly, 3 patients with baseline negative EE developed PAH shortly after initial testing, possibly representing a false-negative result. Because invasive hemodynamics with right heart catheterization (RHC) was not performed unless clinically indicated and was also performed up to 6 months following EE, it is difficult to ascertain the meaning of the present findings. Certainly, other SSc-specific features or biomarkers of increased risk in combination with positive EE need to be identified; however, lack of standardization in annual testing limits identification in this present study. In contrast, a negative EE may be helpful in identifying a group protected from developing PH in the future. This finding, if confirmed in larger studies, could lead to improved early detection strategies for patients with SSc at highest risk for development of PAH, and potentially other at-risk populations with connective tissue disease. The current study is an extension of an original 2008 manuscript, also by Steen, et al , in which SSc patients at risk for PAH underwent EE to determine whether … Address correspondence to Dr. M. Mukherjee, Johns Hopkins University Division of Cardiology, 301 Mason Lord Drive, Suite 2400, Baltimore, Maryland 21224, USA. E-mail: mmukher2{at}jhmi.edu
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Mukherjee et al. (2020) conducted an editorial in Systemic Sclerosis (SSc). Persistently positive exercise echocardiography vs. Persistently negative exercise echocardiography was evaluated on Development of resting pulmonary arterial hypertension (PAH). Systemic sclerosis patients with a persistently positive exercise echocardiography are at an increased risk of developing resting pulmonary arterial hypertension over a mean 4-year follow-up.
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