ArticleEuropean heart journal. Cardiovascular Imaging2023
Comparative analysis of right ventricular strain in Fabry cardiomyopathy and sarcomeric hypertrophic cardiomyopathy.
Article in European heart journal. Cardiovascular Imaging, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 19 citations in OpenAlex.
- Article
- Cardiological aspects of Fabry disease: from diagnosis to therapeutic efficacy assessment.Orphanet journal of rare diseases · 2026Review
- Assessment of right ventricular systolic function in patients with hypertrophic cardiomyopathy by layer-specific speckle tracking echocardiography.Quantitative imaging in medicine and surgery · 2026Article
- The role of quantitative cardiovascular MRI and late gadolinium enhancement patterns in differentiating late-onset Anderson-Fabry disease (c.640-801G > A) from hypertrophic cardiomyopathy: a case-control study.International journal of cardiology. Heart & vasculature · 2026Article
- Evolution of Cardiac Damage Staged With Echocardiography in Fabry Disease.Journal of the American Heart Association · 2025Observational
- Subclinical Cardiac Involvement in Asymptomatic ATTR Mutation Carriers: Insights from Cardiac MRI, Myocardial Strain, and Mapping Techniques.Journal of cardiovascular development and disease · 2025Article
- Right atrial strain in Anderson-Fabry disease.Frontiers in cardiovascular medicine · 2025Article
- Right heart and left atrial strain to differentiate cardiac amyloidosis and Fabry disease.Scientific reports · 2024Article
- Fabry Disease: More than a Phenocopy of Hypertrophic Cardiomyopathy.Journal of clinical medicine · 2023Review
- Right Ventricular Myocardial Involvement in Anderson-Fabry Disease at Diagnosis: Evaluation with Three-Dimensional Strain Imaging.Life (Basel, Switzerland) · 2023Article
- Multimodality Imaging in Sarcomeric Hypertrophic Cardiomyopathy: Get It Right…on Time.Life (Basel, Switzerland) · 2023Review
- Anderson-Fabry disease cardiomyopathy: an update on epidemiology, diagnostic approach, management and monitoring strategies.Frontiers in cardiovascular medicine · 2023Review
- Fabry disease: Definition, Incidence, Clinical presentations and TreatmentPakistan journal of medical sciencesReview
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Authors and funding
12 authors at 5 institutions in 4 countries.
Funding
Abstract
aimsTo perform a comparative analysis of right ventricle (RV) myocardial mechanics, assessed by 2D speckle-tracking echocardiography (2D-STE), between patients with Fabry disease and patients with sarcomeric disease. METHODS AND
resultsPatients with Fabry cardiomyopathy (FC) (n = 28) were compared with patients with sarcomeric hypertrophic cardiomyopathy (HCM), matched for degree of left ventricle hypertrophy (LVH) and demographic characteristics (n = 112). In addition, patients with Fabry disease and no LVH [phenotype-negative carriers of pathogenic α-galactosidase gene mutations (GLA LVH-)] (n = 28) were compared with age and sex-matched carriers of sarcomeric gene mutations without LVH [Phenotype-negative carriers of pathogenic sarcomeric gene mutations (Sarc LVH-)] (n = 56). Standard echocardiography and 2D-STE were performed in all participants. Despite a subtle impairment of RV global longitudinal strain (RV-GLS) was common in both groups, patients with FC showed a more prominent reduction of RV free wall longitudinal strain (RV-FWS) and lower values of difference between RV-FWS and RV-GLS (ΔRV strain), in comparison to individuals with HCM (P < 0.001 and P = 0.002, respectively). RV-FWS and ΔRV strain demonstrated an independent and additive value in discriminating FC from HCM, over the presence of symmetric LVH, systolic anterior motion of the mitral valve and RV hypertrophy. Similar results were found in GLA LVH- patients: they had worse RV-FWS and lower values of ΔRV strain as compared to Sarc LVH- patients (both P < 0.001).
conclusionPatients 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.
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