Objectives Scleroderma (SSc)-associated pulmonary arterial hypertension (PAH) is a major cause of mortality in SSc patients and represents an important diagnostic and therapeutic target. Our aims were to evaluate the relationship between echocardiogram-derived right heart hemodynamics and “gold standard” right heart catheterization (RHC) measurements in a scleroderma population, and investigate whether this relationship is modified by a subset of pulmonary hypertension. Methods We performed right heart catheterization and echocardiography on the same day, with pulmonary function testing in 21 consecutive subjects (age 57 ± 10, 81% female) with scleroderma and pre-capillary pulmonary hypertension. Results RHC measures including PA systolic and mean pressure, and pulmonary vascular resistance (PVR) correlated strongly with echo-derived data. RHC-derived pulmonary vascular resistance was negatively associated with RV systolic performance as measured by tricuspid annular plane systolic excursion (TAPSE, rho −0.70, p < 0.001), tissue Doppler tricuspid s’ velocity (rho −0.68, p 0.002), and RV fractional area change (rho −0.78, p < 0.001). Correlations with TAPSE and s’ velocity were strengthened when FVC%/DLCO% ≥ 1.6 used to identify pure PAH phenotypes in SSc. Bland-Altman analyses demonstrated strong agreement between RHC and echo-derived hemodynamic measures. Conclusions Our findings suggest that echocardiography may play a clinical role in identifying pulmonary hypertension and RV dysfunction non-invasively, particularly in a subset of SSc patients stratified by pulmonary function testing. This method may establish specific disease phenotypes with differential cardiovascular impact and prove useful as a marker of disease progression/risk stratification in SSC patients that warrants further investigation in larger cohorts.
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