Letter by Peverill Regarding Article, "The Heart in Friedreich Ataxia: Definition of Cardiomyopathy, Disease Severity, and Correlation With Neurological Symptoms"

In Friedreich ataxia (FA), cardiac disease is the major cause of premature mortality¹ and a common cause of symptoms, yet our understanding of the nature of the myocardial involvement in FA remains incomplete. Weidemann et al² are therefore to be congratulated for their report on comprehensive cardiac data acquired from a large cohort of FA subjects. The main aim of the study was the development of criteria for staging of the cardiomyopathy in FA; however, there are assumptions underlying the analysis that merit further consideration.