Changes in peak oxygen consumption in Fabry disease and associations with cardiomyopathy severity
This 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<sub>2peak</sub> holds promise as a therapeutic marker of response to FD-specific therapy.
Authors
- S. Thompson (ORCID: https://orcid.org/0000-0002-8645-5529)
- Ashwin Roy (ORCID: https://orcid.org/0000-0003-4600-2733)
- Tom E. Nightingale (ORCID: https://orcid.org/0000-0003-2947-4931)
- Ravi Vijapurapu (ORCID: https://orcid.org/0000-0002-2472-5538)
- John Townend (ORCID: https://orcid.org/0000-0002-5881-5806)
- Richard P. Steeds (ORCID: https://orcid.org/0000-0001-5687-2535)
- Christopher O’Shea (ORCID: https://orcid.org/0000-0003-3030-7364)
- Paul Clift (ORCID: https://orcid.org/0000-0001-8906-7647)
- James Hodson (ORCID: https://orcid.org/0000-0003-3487-0090)
- Nicola Condon
- Tarekegn Geberhiwot
- Amor Mia Alvior (ORCID: https://orcid.org/0009-0008-7994-7614)
- Jan van der Vliet
Institutions
- University Hospitals Birmingham NHS Foundation Trust (GB)
- Queen Elizabeth Hospital Birmingham (GB)
- University of Birmingham (GB)
Publication Details
- Journal
- Heart
- Published
- 2024-11-25
- DOI
- https://doi.org/10.1136/heartjnl-2024-324553
- Citations
- 6
- Primary Topic
- Lysosomal Storage Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 1.96