Rare form of arrhythmogenic cardiomyopathy in a 5-year-old child

Abstract

Arrhythmogenic cardiomyopathy is a genetically determined disease of the heart muscle, characterized by fibrofat replacement of the ventricular myocardium, which predisposes to ventricular arrhythmias and a high risk of SCD. Initially, it was believed that this disease is characterized by an exclusive or predominant lesion of the right ventricle. However, fibro-fatty replacement can also be localized in the left ventricle without involvement of the right chambers. This article presents a rare clinical case of a child with Carvajal syndrome with the classic triad of signs (left-dominant form of AСM, keratoderma, and woolly-curly hair).