Abstract
Naxos disease is a recessively inherited stereotype association of arrhythmogenic cardiomyopathy with a cutaneous phenotype, characterized by peculiar woolly hair and palmoplantar keratoderma. The cardiomyopathy clinically manifests by adolescence and the symptomatic presentation is usually with syncope and/or sustained ventricular tachycardia of left bundle branch block configuration. We report the case of a 43-year-old man without any history of heart disease who was admitted to the hospital because of an episode of sustained ventricular tachycardia and troponin I elevation, in the absence of coronary artery disease. Diagnostic workup, including genetic assessment, revealed Naxos disease as the underlying cause. In this case, acute myocarditis seems to be the most plausible explanation for the nonischemic myocardial injury.
Similar content being viewed by others
References
McKoy G, Protonotarios N, Crosby A, Tsatsopoulou A, Anastasakis A, Coonar A, Norman M, Baboonian C, Jeffery S, McKenna WJ (2000) Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease). Lancet 355:2119–2124
Protonotarios N, Tsatsopoulou A, Anastasakis A, Sevdalis E, McKoy G, Stratos K, Gatzoulis K, Tentolouris K, Spiliopoulou C, Panagiotakos D, McKenna W, Toutouzas P (2001) Genotypephenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. J Am Coll Cardiol 38:1477–1484
Protonotarios NI, Tsatsopoulou AA, Gatzoulis KA (2002) Arrhythmogenic right ventricular cardiomyopathy caused by a deletion in plakoglobin (Naxos disease). Card Electrophysiol Rev 6:72–80
Protonotarios N, Tsatsopoulou A (2004) Arrhythmogenic right ventricular cardiomyopathy/dysplasia and Naxos disease. Hellenic J Cardiol 45:370–378
McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, Camerini F (1994) Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 71:215–218
Basso C, Thiene G, Corrado D, Angelini A, Nava A, Valente M (1996) Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy or myocarditis? Circulation 94:983–991
Thiene G, Corrado D, Nava A, Rossi L, Poletti A, Boffa GM, Daliento L, Pennelli N (1991) Right ventricular cardiomyopathy: is there evidence of an inflammatory aetiology? Eur Heart J Suppl D:22–25
Valente M, Calabrese F, Thiene G, Angelini A, Basso C, Nava A, Rossi L (1998) In vivo evidence of apoptosis in arrhythmogenic right ventricular cardiomyopathy. Am J Pathol 15:479–484
Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C, Daliento L, Buja G, Corrado D, Danieli GA, Thiene G (2000) Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy J Am Coll Cardiol 36:2226–2233
Fontaine G, Fontaliran F, Rosas Andrade F, Velasquez E, Tonet J, Jouven X, Fujioka Y, Frank R (1995) The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy. Heart Vessels 10:227–235
American College of Cardiology; American Heart Association Task Force; European Society of Cardiology Committee for Practice Guidelines; European Heart Rhythm Association; Heart Rhythm Society (2006) ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death — executive summary: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines. Eur Heart J 27:2099–2140
Corrado D, Leoni L, Link MS, Della Bella P, Gaita F, Curnis A, Salerno JU, Igidbashian D, Raviele A, Disertori M, Zanotto G, Verlato R, Vergara G, Delise P, Turrini P, Basso C, Naccarella F, Maddalena F, Estes NA 3rd, Buja G, Thiene G (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108:3084–3091
Anastasakis A, McKenna W, Stefanadis C (2006) Prevention of sudden cardiac death in the young: targeted evaluation of those at risk. Hellenic J Cardiol 47:251–254
Boriani G, Artale P, Biffi M, Martignani C, Frabetti L, Valzania C, Diemberger I, Ziacchi M, Bertini M, Rapezzi C, Parlapiano M, Branzi A (2007) Outcome of cardioverter-defibrillator implant in patients with arrhythmogenic right ventricular cardiomyopathy. Heart Vessels 22:184–192
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Lazaros, G., Anastasakis, A., Tsiachris, D. et al. Naxos disease presenting with ventricular tachycardia and troponin elevation. Heart Vessels 24, 63–65 (2009). https://doi.org/10.1007/s00380-008-1082-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00380-008-1082-5