RVGLS and RVFWLS represent distinct myocardial components with different clinical correlates in Fabry cardiomyopathy, and only RVGLS—reflecting septal involvement—showed significant longitudinal change associated with enzyme replacement therapy.
RVGLS and RVFWLS were not significantly correlated with each other in patients with Fabry disease.
RVGLS was independently associated with left ventricular global longitudinal strain and interventricular septal thickness, consistent with a septal-coupled pattern.
RVFWLS was independently associated with right atrial reservoir strain, consistent with an atrial-coupled pattern.
Longitudinal RVGLS change differed significantly between untreated patients and those receiving enzyme replacement therapy.
Longitudinal RVFWLS change did not differ significantly between untreated and ERT-treated patients.
The study enrolled 100 patients with genetically confirmed Fabry disease, of whom 69 had serial echocardiography for longitudinal analysis.
This research suggests that two commonly used measures of right heart function—right ventricular global longitudinal strain (RVGLS) and right ventricular free-wall longitudinal strain (RVFWLS)—actually capture different aspects of heart muscle behavior in patients with Fabry disease, a rare inherited condition that causes a fatty substance to accumulate in organs including the heart. The study found that RVGLS is closely linked to the function of the wall separating the left and right ventricles (the septum) and mirrors left heart dysfunction, while RVFWLS is more closely tied to how well the right atrium (the upper right chamber) functions. Importantly, these two measures were not correlated with each other, suggesting they should not be used interchangeably. When the researchers tracked 69 patients over time, they found that enzyme replacement therapy (ERT)—the standard treatment for Fabry disease—was associated with improvement in RVGLS but not in RVFWLS. Untreated patients showed worsening RVGLS over time, while treated patients showed improvement. This difference held up even after accounting for factors like age, sex, and genetic subtype. This research suggests that RVGLS may be a more sensitive marker for detecting treatment response in Fabry cardiomyopathy than RVFWLS. These findings have potential practical implications for how doctors monitor heart involvement in Fabry disease patients. Using RVGLS rather than—or in addition to—RVFWLS may provide better insight into whether a patient's right heart is responding to ERT. However, the authors note that these findings are preliminary and require prospective validation in larger, controlled studies before they can be used to guide clinical decisions.
Sung K, Niu D, Hsu S, Liu M, Lin P, Lai Y, et al.. (2026). Two Faces of the Right Ventricle in Fabry Cardiomyopathy: Septal-Coupled and Atrial-Coupled Strain Patterns and Their Differential Associations with Enzyme Replacement Therapy.. Biomolecules. https://doi.org/10.3390/biom16081196