Electrical Storm or Naxos Syndrome in an Adult Causing Recurrent Syncope.

Abstract

Among the rare and well-known causes of sudden cardiac death by malignant arrthymias is a condition called arrhythmogenic right ventricular cardiomyopathy. It commonly presents with right ventricular dilatation, dysfunction and ventricular tachycardia of left bundle branch morphology due to fibro-fatty infiltration of right ventricle in second to fifth decade of life, making it an unrecognized and important cause of sudden cardiac death. Two rare variants of arrhythmogenic right ventricular cardiomyopathy are Carvajal syndrome and Naxos syndrome. Both variants have systemic manifestations. Being a rare variant of arrhythmogenic right ventricular dysplasia, Naxos syndrome was initially described in the families of the Greek island of Naxos. It is a recessive disorder with cardio-cutaneous manifestations characterized by arrhythmogenic right ventricular cardiomyopathy, palmoplantar keratoderma and wooly hair. We report a rare case of Naxos syndrome in an adult patient presented with recurrent episodes of palpitation and syncope.