Constrictive Pericarditis Versus Restrictive Cardiomyopathy?

Mario J. Garcia, Journal of the American College of Cardiology, Volume 67, Issue 17, 3 May 2016, Pages 2061-2076, ISSN 0735-1097, doi:10.1016/j.jacc.2016.01.076.


Friedreich’s ataxia is an autosomal recessive neurodegenerative disorder. Ventricular arrhythmias and SCD are common. Early in the course of the disease, ECG and echocardiographic findings resemble those of hypertrophic cardiomyopathy, including symmetric LV hypertrophy, abnormal myocardial relaxation, and LV outflow obstruction. Over time, the restrictive phenotype evolves into a dilated phenotype. There is no specific treatment for this condition other than standard HF drugs. Implantable cardioverter-defibrillators are used for prevention of SCD, but a survival benefit has not been demonstrated.