Key result
3D left ventricular global longitudinal strain was significantly associated with long-term prognosis in patients with Fabry disease (adjusted HR 0.85; 95% CI 0.75-0.95; P=0.004).
Why the study?
Fabry disease manifests as a phenocopy of hypertrophic cardiomyopathy, but the relationship of echocardiographic 3D LV strain to heart failure severity, myocardial scar, and long-term prognosis was unclear.
Population
75 patients with Fabry disease (out of 99 evaluated)
Comparison
Echocardiographic 3D LV strain vs heart failure severity, CMR late gadolinium enhancement scar, and prognosis
Design
Cohort study
Follow-up
Median 3.1 years
Authors
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3D LV-GLS may refine FD risk stratification; leaves open whether it adds prognostic value beyond NT-proBNP or CMR in prospective cohorts.
Cohort (n=75)
Hazard Ratio: 0.85 (95% CI 0.75–0.95)
p-value: p=0.004
In patients with Fabry disease, 3D left ventricular global longitudinal strain is associated with heart failure severity and predicts long-term adverse cardiovascular outcomes.
Marek et al. (2023) conducted a cohort in Fabry disease (n=75). 3D left ventricular global longitudinal strain was evaluated on Long-term prognosis (death, heart failure decompensation, or cardiovascular hospitalization) (adjusted HR 0.85, 95% CI 0.75-0.95, p=0.004). 3D left ventricular global longitudinal strain was significantly associated with long-term prognosis in patients with Fabry disease (adjusted HR 0.85; 95% CI 0.75-0.95; P=0.004).
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