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

Abstract

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.

Trial registration: ClinicalTrials.gov NCT00706082.

Copyright © 2014 by the American College of Rheumatology.

Figures

Figure 1. Relationship between RHC and echo-derived PASP

Panel A. Linear regression demonstrates significant correlation in this study between cath and echo PASP for all subjects (rho 0.92, p < 0.001). TTE= Transthoracic echocardiogram Panel B. Bland-Altman plot for echo-derived PASP and RHC PASP.

Figure 2. Relationship between RHC PVR and echo TAPSE measures

Linear regression demonstrates significant correlation between TAPSE and RHC PVR for all subjects (rho −0.70, p CO% ≥ 1.6, (open circle symbol, rho −0.90, p < 0.001). Closed circle symbol represents FVC%/DLCO% < 1.6.

Figure 3. Relationship between RHC and echo-derived PVR

Panel A. Linear regression demonstrates significant correlation between cath and echo PVR for all subjects (rho 0.75, p < 0.001). The correlation strengthens when stratified by FVC%/DLCO% ≥ 1.6, (open circle symbol, rho −0.87, p 0.003). Closed circle symbol represents FVC%/DLCO% < 1.6. Panel B. Bland-Altman plot for echo-derived PVR and RHC PVR.