PT  - JOURNAL ARTICLE
AU  - Kaitlin A.  Quinn
AU  - Stephanie R.  Wappel
AU  - Tunay Kuru
AU  - Virginia D.  Steen
TI  - Exercise Echocardiography Predicts Future Development of Pulmonary Hypertension in a High-risk Cohort of Patients with Systemic Sclerosis
AID  - 10.3899/jrheum.190226
DP  - 2019 Nov 15
TA  - The Journal of Rheumatology
PG  - jrheum.190226
4099  - http://www.jrheum.org/content/early/2019/12/09/jrheum.190226.short
4100  - http://www.jrheum.org/content/early/2019/12/09/jrheum.190226.full
AB  - Objective To evaluate whether a positive exercise echocardiogram (EE) predicts future development of pulmonary arterial hypertension (PAH) in a high-risk cohort of patients with systemic sclerosis (SSc). Methods Patients with SSc with features associated with an increased risk for PAH were recruited into a prospective, observational cohort. All patients underwent clinical assessment and EE. A positive EE was defined as an increase of ≥ 20 mmHg in the right ventricular systolic pressure with exercise. All patients with positive EE underwent right heart catheterization (RHC). Results The study included 85 patients. In the positive EE cohort, 10 of 43 patients (23%) developed resting pulmonary hypertension (PH) on RHC over a mean 4-year followup period [4 with PAH, 5 with pulmonary venous hypertension (PVH), and 1 with PH associated with interstitial lung disease]. In the persistently negative EE cohort, only 3 of 42 patients (7%) developed resting PH (1 PAH, 2 PVH; p = 0.04). Of the remaining 33 patients in the positive EE group who did not develop resting PH, 22 (67%) had a persistently positive EE over an average 5-year followup period. Conclusion In this high-risk cohort of patients with SSc, a positive EE may predict the future development of resting PH. In addition, a majority of patients may have a persistently positive EE for years without progression to resting PH. Finally, a consistently negative EE may identify patients at low risk for future PH.