Heart failure & cardiomyopathies
Comparative analysis of right ventricular strain in Fabry cardiomyopathy and sarcomeric hypertrophic cardiomyopathy
Authors' conclusion
Patients with FC show a specific pattern of RV myocardial mechanics, characterized by a larger impairment of RV-FWS and lower ΔRV strain in comparison to patients with HCM, which may be helpful in the differential diagnosis between these two diseases.
Study context
- Population
- Patients with Fabry disease vs patients with sarcomeric disease, with and without left ventricle hypertrophy (LVH)
- Participants
- 28 / 112 / 28 / 56
- Exposure
- Fabry cardiomyopathy vs sarcomeric hypertrophic cardiomyopathy (HCM)
- Fabry cardiomyopathy (FC)n = 28
- HCMn = 112reference
- GLA LVH-n = 28
- Sarc LVH-n = 56reference
- Outcome
- RV myocardial mechanics by 2D speckle-tracking echocardiography
Methodological detailsmatched
- Matched
Main finding
As reported in the abstract
RV strain, FC vs HCM
- Subtle impairment of RV global longitudinal strain (RV-GLS) common in both groups
- RV-FWS and ΔRV strain had independent and additive value in discriminating FC from HCM over symmetric LVH, systolic anterior motion of the mitral valve and right ventricle (RV) hypertrophy
- FC vs HCM: P < 0.001
- FC vs HCM: P = 0.002
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| RV strain, GLA LVH- vs Sarc LVH- | P < 0.001 |
- RV strain, GLA LVH- vs Sarc LVH-: worse RV-FWS and lower ΔRV strain in GLA LVH- (both P < 0.001)
Original publication
Comparative analysis of right ventricular strain in Fabry cardiomyopathy and sarcomeric hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging. Published 28 July 2022. doi:10.
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