Heart failure & cardiomyopathies
Three-dimensional echocardiographic left ventricular strain analysis in Fabry disease: correlation with heart failure severity, myocardial scar, and impact on long-term prognosis
Authors' conclusion
3D LV-GLS is associated with both heart failure severity measured by natriuretic peptide levels and long-term prognosis. Decreased posterolateral 3D CS reflects typical posterolateral scarring in FD.
Study context
- Population
- Patients with Fabry disease (mean age 47 ± 14 years, 44% male, LVEF 65 ± 6%; 51% with left ventricular (LV) hypertrophy or concentric remodelling)3D echo feasible in 75
- Participants
- 99 patients
- Outcome
- Correlation with NT-proBNP and association with long-term prognosis (death, HF decompensation or CV hospitalization)
- Follow-up
- Median 3.1 years
Main finding
As reported in the abstract
median follow-up 3.1 years
- NT-proBNP vs 3D LV-GLS correlation
- r = −0.49, P < 0.0001
- NT-proBNP vs 3D LV-GCS correlation
- r = −0.38, P < 0.001
- NT-proBNP vs 3D LVEF correlation
- r = −0.25, P = 0.036
- 3D LV-GLS and long-term prognosis, adjusted HR
- 0.85, P = 0.004 (CI 0.75-0.95)
- 3D LV-GCS and long-term prognosis, not associated
- P = 0.284
- 3D LVEF and long-term prognosis, not associated
- P = 0.324
Observational data: associations, not effects.
Further findings
Posterolateral 3D circumferential strain with vs without posterolateral cardiovascular magnetic resonance (CMR) scar
- Lower posterolateral 3D circumferential strain (CS) with posterolateral scar on CMR
- P = 0.009
Original publication
Three-dimensional echocardiographic left ventricular strain analysis in Fabry disease: correlation with heart failure severity, myocardial scar, and impact on long-term prognosis. Eur Heart J Cardiovasc Imaging. Published 13 June 2023. doi:10.
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