Relation of novel echocardiographic measures to invasive hemodynamic assessment in scleroderma-associated pulmonary arterial hypertension.

OBJECTIVE: Systemic sclerosis (SSC; scleroderma)-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-sided heart hemodynamics and gold standard right-sided heart catheterization (RHC) measurements in a scleroderma population and to investigate whether this relationship is modified by a subset of pulmonary hypertension.

METHODS: We performed RHC and echocardiography on the same day, with pulmonary function testing in 21 consecutive subjects with scleroderma and precapillary pulmonary hypertension (mean ± SD age 57 ± 10 years, 81% women).

RESULTS: RHC measures, including pulmonary arterial systolic and mean pressure and pulmonary vascular resistance (PVR), correlated strongly with echocardiogram-derived data. RHC-derived PVR was negatively associated with right ventricular (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 forced vital capacity %/diffusing capacity of the lung for carbon monoxide % ≥1.6 was used to identify pure PAH phenotypes in SSc. Bland-Altman analyses demonstrated strong agreement between RHC and echocardiogram-derived hemodynamic measures.

CONCLUSION: Our findings suggest that echocardiography may play a clinical role in identifying pulmonary hypertension and RV dysfunction noninvasively, 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.