The current revised task force criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) includes only morphological criteria for right ventricle with no consideration for left ventricle criteria.1 However, accumulating evidence suggests increasing left ventricle involvement in ACM. One such phenotype is Carvajal syndrome, which is a primary left ventricle ACM that is inherited as a familial cardiocutaneous syndrome consisting of woolly hair, palmoplantar keratoderma, and cardiac involvement.
