ReviewReviews in cardiovascular medicine2022
The Heart in Fabry Disease: Mechanisms Beyond Storage and Forthcoming Therapies.
Review in Reviews in cardiovascular medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Imaging predictors of adverse prognosis in Fabry disease cardiomyopathy: A systematic review and meta-analysis.European journal of clinical investigation · 2025Pooled it
- Genetic Modifiers of Cardiac Remodeling Severity in IVS4+919G>A-Associated Fabry Cardiomyopathy.International journal of molecular sciences · 2026Article
- Supranormal Ejection Fraction as a Clinical Red Flag: Differentiating Hemodynamic Stress From Intrinsic Cardiomyopathy in Heart Failure.Mayo Clinic proceedings. Innovations, quality & outcomes · 2026Article
- Cardiological aspects of Fabry disease: from diagnosis to therapeutic efficacy assessment.Orphanet journal of rare diseases · 2026Review
- Enhancing specialist competence in clinical pathways for left ventricular hypertrophy: design and evaluation of a competency-based curriculum.BMC health services research · 2026Article
- Exploring a plasma proteomic biosignature associated with cardiac involvement in Fabry disease.Journal of molecular medicine (Berlin, Germany) · 2026Article
- Longitudinal Adipokine and Lipid Profiles in Fabry Disease.Journal of clinical medicine · 2026Article
- Pathogenic mechanisms in Fabry disease.Frontiers in medicine · 2026Review
- Early diagnosis of cardiac involvement in Anderson-Fabry disease using cardiac MRI parameters.Future cardiology · 2025Review
- Article
- Evolution of Cardiac Damage Staged With Echocardiography in Fabry Disease.Journal of the American Heart Association · 2025Observational
- Classification, Diagnosis, and Prognosis of Cardiomyopathy: A Comprehensive Narrative Review.Reviews in cardiovascular medicine · 2025Review
- Enzyme Replacement and Immunosuppression in Heart Transplant Recipients with Fabry Cardiomyopathy: A 7-Year Case Study.The American journal of case reports · 2025Article
- Cardiac involvement in Fabry disease: Recent advances, unresolved issues, and unmet needs.European heart journal supplements : journal of the European Society of Cardiology · 2025Article
- Right atrial strain in Anderson-Fabry disease.Frontiers in cardiovascular medicine · 2025Article
- Fabry Disease: More than a Phenocopy of Hypertrophic Cardiomyopathy.Journal of clinical medicine · 2023Review
- Sex Differences in Anderson-Fabry Cardiomyopathy: Clinical, Genetic, and Imaging Analysis in Women.Genes · 2023Article
- Inflammation across the spectrum of hypertrophic cardiac phenotypes.Heart failure reviews · 2023Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In patients with Fabry disease (FD), cardiovascular involvement is the main cause of death and reduction of quality of life. Left ventricular hypertrophy mimicking hypertrophic cardiomyopathy is the main feature of FD cardiac involvement although glycolipid storage occurs in all cardiac cellular types. Accumulation of lysosomal globotriasylceramide represents the main mechanism of cardiac damage in early stages, but secondary pathways of cellular and tissue damage, triggered by lysosomal storage, and including altered energy production, inflammation and cell death, contribute to cardiac damage and disease progression. These mechanisms appear prominent in more advanced stages, hampering and reducing the efficacy of FD-specific treatments. Therefore, additional cardiovascular therapies are important to manage cardiovascular symptoms and reduce cardiovascular events. Although new therapies targeting lysosomal storage are in development, a better definition and comprehension of the complex pathophysiology of cardiac damage in FD, may lead to identify new therapeutic targets beyond storage and new therapeutic strategies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.