Relation of Novel Echocardiographic Measures to Invasive Hemodynamic Assessment in Scleroderma‐Associated Pulmonary Arterial Hypertension

Abstract

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.

Pulmonary Arterial Hypertension is Normalized Following Six Years of Inhaled Iloprost Treatment in a Patient with Systemic Sclerosis

“…According to the study by Denton et al [4], echocardiographic measures correlate significantly with invasive hemodynamic assessment. Several newer echocardiographic variables have also been closely associated with RHC measurements [8]. Therefore, echocardiography can play a clinical role in detecting PAH and right ventricular dysfunction noninvasively.…”

Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

“…PH was not diagnosed in current practical recommendation, most of patients were diagnosed PH by echocardiography without confirmation with RHC. The reports demonstrated the estimated pulmonary pressure by echocardiography was correlated with that measured by RHC and associated with mortality [13,19,20]. The small sample size of each CTD made it impossible to identify more characteristics according to each disease.…”

“…In Korean registry report, a number of patients received a diagnosis of PAH on the basis of only echocardiography because the frequency of RHC was low [18,19]. Moreover, pulmonary vascular resistance estimated by echocardiography was shown to closely correlate with that evaluated by RHC in patients with a forced vital capacity (FVC)/lung diffusion capacity for carbon monoxide (DLCO) ratio ＞1.6, which means that echocardiography can be a reliable marker in identifying PH noninvasively [20]. Moreover, PASP measured by using echocardiography was revealed to be associated with mortality regardless of the results of RHC [21].…”

Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

“…58 Other novel TTE measures of RV systolic performance, including tissue Doppler systolic velocity and fractional area change, have also been shown to correlate negatively with PVR in patients with SSc-PAH. 59 …”