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Naxos Disease

By Nikos Protonotarios and Adalena Tsatsopoulou


Since 1995, according to the World Health Organisation’s classification of cardiomyopathies, Naxos disease has been considered as the recessive form of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).1 It is a stereotype association of ARVD/C with a cutaneous phenotype, characterised by woolly hair and palmoplantar keratoderma.2 Epidemiology Naxos disease was first reported in 1986 by Protonotarios et al in patients originating from the Hellenic island of Naxos.2,3 Apart from Naxos, cases have also been reported from other Hellenic islands, as well as from Turkey, Israel and Saudi Arabia.4 The prevalence of the disease in Hellenic islands reaches 1:1000. A variety of Naxos disease presenting at a younger age with more pronounced left ventricular involvement has been described in families from India and Ecuador (Carvajal syndrome)

Topics: Indian Pacing and Electrophysiology Journal
Publisher: Indian Pacing and Electrophysiology Group
Year: 2005
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