Two Faces of the Right Ventricle in Fabry Cardiomyopathy: Septal-Coupled and Atrial-Coupled Strain Patterns and Their Differential Associations with Enzyme Replacement Therapy

Figures

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  Figure 1. Differential associations of RVGLS and RVFWLS in Fabry cardiomyopathy. (A) RVGLS correlated with LVGLS. (B) RVGLS correlated with interventricular septal thickness (IVSd). (C) RVFWLS correlated inversely with right atrial (RA) reservoir strain. (D) RVFWLS correlated inversely with left atrial (LA) reservoir strain. (E) RVGLS and RVFWLS were not significantly correlated. (F) In multivariable models adjusted for age, sex, IVSd, LVGLS, E/e′ mean, and RA reservoir strain, with identical covariates entered into both models, RVGLS was independently associated with LVGLS and IVSd, whereas RVFWLS was independently associated with RA strain. Shaded bands represent 95% confidence intervals; points represent individual patients. In (F), squares indicate standardized regression coefficients and horizontal lines indicate 95% confidence intervals. RVGLS, right ventricular global longitudinal strain; RVFWLS, right ventricular free-wall longitudinal strain; LVGLS, left ventricular global longitudinal strain; IVSd, interventricular septal thickness at end-diastole; RA, right atrium; LA, left atrium.

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  Figure 2. Longitudinal changes in RVGLS, RVFWLS, LVGLS, and RA strain according to ERT status. (A) Longitudinal RVGLS change differed between untreated and ERT-treated patients. (B) RVFWLS change did not differ significantly between groups. (C) LVGLS change differed between groups. (D) RA strain change did not differ significantly between groups. Negative Δ values indicate more negative myocardial strain at follow-up. Violin plots show distributions, box plots show medians and interquartile ranges, dots represent patients, and black squares show mean ± standard error. ERT, enzyme replacement therapy; RVGLS, right ventricular global longitudinal strain; RVFWLS, right ventricular free-wall longitudinal strain; LVGLS, left ventricular global longitudinal strain; RA, right atrial; Δ, follow-up minus baseline.

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  Figure 3. Septal hypertrophy and the difference between RVGLS and RVFWLS. (A) ΔRV strain (RVFWLS−RVGLS) differed across IVSd tertiles (≤9.3, 9.3−13.0, and >13.0 mm; ANOVA p = 0.018). (B) RVGLS was more impaired in LVH-positive patients, whereas RVFWLS and RA strain did not differ significantly by LVH status. (C) Association between ERT and ΔRVGLS in the primary model adjusted for age, sex, and genotype and in the sequential model additionally adjusted for baseline RVGLS and concurrent ΔLVGLS. Squares indicate regression coefficients and horizontal lines indicate 95% confidence intervals. ΔRV strain, RVFWLS−RVGLS; RVGLS, right ventricular global longitudinal strain; RVFWLS, right ventricular free-wall longitudinal strain; RA, right atrial; IVSd, interventricular septal thickness at end-diastole; LVH, left ventricular hypertrophy; ERT, enzyme replacement therapy; ΔLVGLS, longitudinal change in left ventricular global longitudinal strain; CI, confidence interval.