Observational studyHeart (British Cardiac Society)2025
Changes in peak oxygen consumption in Fabry disease and associations with cardiomyopathy severity.
Observational study in Heart (British Cardiac Society), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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
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Who cites it
5 citing papers in PubMed.
- Physical Fitness and Physical Function in Patients With Fabry Disease: A Cross-Sectional Multicentre Study.Journal of cachexia, sarcopenia and muscle · 2026Article
- Left ventricular myocardial work combined with stress echocardiography assessment of cardiac function in patients with Fabry disease.Scientific reports · 2025Article
- Unveiling Functional Impairment in Fabry Disease: The Role of Peripheral vs. Cardiac Mechanisms.Biomedicines · 2025Article
- Review
- Cardiopulmonary exercise testing in Fabry disease: is it an early marker of cardiomyopathy?Heart (British Cardiac Society) · 2025Article
Corrections and comments
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Authors and funding
13 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundFabry disease (FD) causes multiorgan sphingolipid accumulation, with cardiac involvement responsible for the largest burden of morbidity and mortality. Exercise intolerance in FD is prevalent, yet the mechanisms of this are poorly understood. The aim of this study was to assess exercise intolerance in FD and identify whether this correlates with the phase of cardiomyopathy.
methodsThis was a retrospective observational study of adults with FD undergoing cardiopulmonary exercise testing (CPEX) between September 2011 and September 2023 at a national referral centre in the UK. The primary outcome measure was peak oxygen uptake (V̇O
resultsCPEX was completed by 42 patients, with a median age of 54 years and of whom 62% were male. Patients were approximately equally distributed across the four cardiomyopathy phases. At phase I, the mean (±SD) V̇O
conclusionsThis study identifies significantly impaired aerobic capacity in FD, even in those without phenotypic cardiomyopathy. No corresponding changes in respiratory impairment were observed, suggesting that exercise intolerance may be due to early cardiac sphingolipid accumulation and subsequent atrial and ventricular dysfunction, which increases as cardiomyopathy progresses. As such, peak V̇O
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