Key result
Risk tree of LV wall thickness, LAVImax, and GLS predicts Fabry major events with ~0.91 AUC.
Why the study?
Speckle tracking echocardiography analysis may be more sensitive for assessing myocardial impairment in Anderson-Fabry disease and have prognostic value.
Does an echocardiographic prognostic risk decision tree using LV wall thickness, LAVImax, and LV GLS predict adverse cardiovascular events in patients with Anderson-Fabry disease?
Observational (n=112)
Does an echocardiographic prognostic risk decision tree using LV wall thickness, LAVImax, and LV GLS predict adverse cardiovascular events in patients with Anderson-Fabry disease?
Effect estimate: AUC 0.910
An echocardiographic decision tree incorporating LV wall thickness, LAVImax, and LV GLS effectively stratifies risk for adverse cardiovascular events in patients with Anderson-Fabry disease.
Supports risk stratification in Anderson-Fabry disease; leaves open prospective validation before clinical use.
Aims Anderson–Fabry disease (AFD) is an X-linked disease, with cardiac involvement resulting in increased left ventricular (LV) wall thickness. Speckle tracking echocardiography analysis may be more sensitive in the assessment of myocardial impairment in AFD patients and have prognostic value. Our aim was to evaluate LV and left atrial (LA) dysfunction by traditional and strain parameters in AFD patients and evaluate prognostic utility. Methods and results Fifty-six AFD patients were age- and sex-matched to 56 healthy controls. LV global longitudinal strain (GLS) and LA reservoir strain (LASR) were significantly lower in male (GLS: 19.38[3.21] vs. 17.8[7.0], P = 0.009; LASR: 38.07 ± 6.67 vs. 31.12 ± 6.76, P = 0.003) and female (GLS: 20.58 ± 1.63 vs. 19.29 ± 1.67, P = 0.003; LASR: 38.77 ± 7.43 vs. 33.13 ± 6.06, P < 0.001) AFD patients compared with controls. Reduced strain parameters were also seen in female AFD patients with normal wall thickness (GLS: 20.88 ± 1.74 vs. 19.72 ± 1.53, P = 0.037; LASR: 40.09 ± 7.15 vs. 34.79 ± 6.20, P = 0.004). 53/56 AFD patients had a median follow-up of 43[81] months; 11/53 experienced an adverse cardiovascular event (i.e. cardiac death, myocardial infarction, arrhythmias, stroke. and heart failure). LV wall thickness, LAVImax, and LV GLS displayed good sensitivity and specificity for adverse cardiac events. A prognostic risk decision tree comprising of these parameters demonstrated good predictive value for adverse events (AUC = 0.910). Conclusion We demonstrate differences in LV and LA echocardiographic parameters in AFD patients compared with healthy controls, including female AFD patients with normal LV wall thickness. A prognostic risk decision tree stratified AFD patients into three groups with the highest risk group demonstrating more AFD-related adverse events.
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Stefani et al. (2025) conducted an observational in Anderson-Fabry disease (n=112). Echocardiographic prognostic risk stratification decision tree vs. Healthy controls was evaluated on Adverse cardiovascular events (cardiac death, myocardial infarction, arrhythmias, stroke, and heart failure) (AUC 0.910). A prognostic risk decision tree comprising LV wall thickness, LAVImax, and LV GLS demonstrated good predictive value for adverse cardiovascular events in Anderson-Fabry disease patients (AUC = 0.910).
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