In conclusion, there is an association between diffuse interstitial LV myocardial fibrosis and genetic severity in FRDA, with this effect being independent of the FRDA-associated LV changes in LVEDVI and LVMI. Localised replacement fibrosis was found in a minority of subjects with FRDA, this minority including both children and adults, and subjects with and without a reduction of LVEF. In contrast to T1 mapping variables, LGE in subjects with a normal LVEF was not associated with genetic severity, and was also not associated with LVM or LVEDV, consistent with the development of LGE in FRDA having an idiosyncratic element. There is the potential for roles of both T1 mapping and LGE in the assessment and monitoring of the cardiomyopathy of FRDA, but information will be required about their prognostic significance, and more data will also be required regarding the reproducibility of T1 mapping variables.
Friday, May 31, 2024
Insights into the effects of Friedreich ataxia on the left ventricle using T1 mapping and late gadolinium enhancement
Peverill RE, Lin KY, Fogel MA, Cheung MMH, Moir WS, Corben LA, et al. (2024) Insights into the effects of Friedreich ataxia on the left ventricle using T1 mapping and late gadolinium enhancement. PLoS ONE 19(5): e0303969. https://doi.org/10.1371/journal.pone.0303969
