Jonathan F. Plehn, Keren Hasbani, Inez Ernst, Kenneth D. Horton, Bart E. Drinkard, Nicholas A. Di Prospero, Journal of Cardiac Failure, Available online 3 October 2017, ISSN 1071-9164, doi:10.1016/j.cardfail.2017.09.012.
A subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and CH is associated with both diastolic and systolic dysfunction.
In our pediatric cohort, GAA repeat length on either allele failed to correlate with LVMI or with measures of systolic or diastolic LV function. These findings are consistent with those of others, two or which were based on robust cMRI criteria. We did note a non-significant trend in GAA repeat length on the long allele with increasing categorical severity of LV remodeling. Despite the high prevalence of ventricular remodeling and functional associations described in this population, there is no evidence to date that early pharmacologic intervention can prevent or impede development of ventricular and/or clinical deterioration.
Thursday, October 5, 2017
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