Carvajal Syndrome-A Rare Variant of Naxos Disease in Two Saudi Siblings

Yusuf Parvez *,Abdul Aziz Al-H

Abstract

Naxos disease is a recessively inherited condition characterised by woolly hair, palmoplantar keratosis and arrhythmogenic right ventricular cardiomyopathy (ARVC). A rare syndrome with similar clinical picture and predominantly involving left ventricle has been termed as Carvajal Syndrome. We report two cases of a Saudi family admitted in our PICU.