Changes in peak oxygen consumption in Fabry disease and associations with cardiomyopathy severity

Roy, Ashwin; Thompson, Sophie E; Hodson, James; van Vliet, Jan; Condon, Nicola; Alvior, Amor Mia; O'Shea, Christopher; Vijapurapu, Ravi; Nightingale, Tom E; Clift, Paul F; Townend, Jonathan; Geberhiwot, Tarekegn; Steeds, Richard Paul

Abstract

This 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 (V̇O2peak), with forced expiratory volume in 1 s (FEV1) used to quantify respiratory impairment. Age-normalised/sex-normalised values were additionally calculated, based on published normal ranges for subgroups of age and sex. The cardiomyopathy phase was classified on a 4-point scale by two FD experts using contemporaneous imaging and biochemistry results.

Citation

Roy A, Thompson SE, Hodson J, van Vliet J, Condon N, Alvior AM, O'Shea C, Vijapurapu R, Nightingale TE, Clift PF, Townend J, Geberhiwot T, Steeds RP. Changes in peak oxygen consumption in Fabry disease and associations with cardiomyopathy severity. Heart. 2024 Nov 25:heartjnl-2024-324553. doi: 10.1136/heartjnl-2024-324553. Epub ahead of print.

Type

Article