Friedreich's ataxia-associated childhood hypertrophic cardiomyopathy: a national cohort study.
Gabrielle NorrishThomas RanceElena MontanesElla FieldElspeth BrownVinay BholeA Graham StuartOrhan UzunKaren A McLeodMaria IlinaSatish AdwaniPiers DaubeneyGrazia Delle DonneKatie LinterCaroline B JonesTara BharuchaElena CerviJuan Pablo Pablo KaskiPublished in: Archives of disease in childhood (2021)
This is the largest cohort of childhood FA-HCM reported to date and describes a high prevalence of atrial arrhythmias and impaired systolic function in childhood, suggesting early progression to end-stage disease. Overall mortality is similar to that reported in non-syndromic childhood HCM, but no patients died suddenly.