Observational studyJournal of the American Heart Association2025
Evolution of Cardiac Damage Staged With Echocardiography in Fabry Disease.
Observational study in Journal of the American Heart Association, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 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.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Fabry disease cardiomyopathy: A state-of-the-art review.Progress in cardiovascular diseasesPooled it
- Article
- Cardiological aspects of Fabry disease: from diagnosis to therapeutic efficacy assessment.Orphanet journal of rare diseases · 2026Review
- Staging of Fabry cardiomyopathy in clinical practice: an algorithm proposal.International journal of cardiology. Heart & vasculature · 2026Article
- Longitudinal Adipokine and Lipid Profiles in Fabry Disease.Journal of clinical medicine · 2026Article
- Long-Term Cardiac Stability Despite Late Enzyme Replacement Therapy in Fabry Disease With Severe Renal Involvement.JACC. Case reports · 2026Article
- Article
Corrections and comments
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Authors and funding
19 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundFabry disease can be classified in 4 stages based on the extent of cardiac damage assessed with echocardiography. This staging is strongly associated with prognosis, with a doubled risk increase of cardiovascular events for each stage progression. The aims of the present study were to investigate the evolution of cardiac damage using this staging system during midterm follow-up and to identify predictors of stage worsening. METHODS AND
resultsThis is a retrospective, multicenter study, which included 168 patients with Fabry disease (38% men) categorized at baseline and follow-up in 4 stages, defined as follows: stage 0, no cardiac involvement; stage 1, left ventricular hypertrophy; stage 2, left atrial enlargement; and stage 3, ventricular impairment. After a median of 5 years, cardiac stage worsened of at least 1 class in 34 patients (24% of 143 patients in stage 0-2 at baseline), improved in 9 (12% of 73 patients in stages 1-3 at baseline), and remained stable in 125 (74% of the 168 patients enrolled). At multivariable logistic regression analysis, age (odds ratio, 1.05 [95% CI, 1.01-1.09];
conclusionsFabry disease is associated with a high rate of progression of cardiac damage during midterm follow-up, with ≈24% of patients experiencing cardiac stage worsening. Age, male sex, classic phenotype, and renal failure are independent predictors of stage progression.
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