Naxos Disease Presenting with Ventricular Tachycardia and Troponin Elevation

Overview

Journal Heart Vessels

Specialty Cardiology & Vascular Diseases

Date 2009 Jan 24

PMID 19165571

Citations 2

Authors

George Lazaros

Aris Anastasakis

Dimitris Tsiachris

Polychronis Dilaveris

Nikos Protonotarios

Christodoulos Stefanadis

Affiliations

Soon will be listed here.

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.

Citing Articles

Naxos Disease and Related Cardio-Cutaneous Syndromes.

Protonotarios A, Asimaki A, Basso C, Xylouri Z, Monda E, Protonotarios I JACC Adv. 2025; 4(2):101547.

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Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review.

Asatryan B, Asimaki A, Landstrom A, Khanji M, Odening K, Cooper L Circulation. 2021; 144(20):1646-1655.

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Electrical storm originating from a left ventricular epicardial scar in a patient with completely normal endocardial voltage.

Sternick E, Piorkowski C, Hindricks G, Dagres N, Sommer P Heart Vessels. 2011; 26(6):663-6.

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References

Anastasakis A, Mckenna W, Stefanadis C . Prevention of sudden cardiac death in the young: targeted evaluation of those at risk. Hellenic J Cardiol. 2006; 47(5):251-4. View

Zipes D, Camm A, Borggrefe M, Buxton A, Chaitman B, Fromer M . 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.... Eur Heart J. 2006; 27(17):2099-140. DOI: 10.1093/eurheartj/ehl199. View

Corrado D, Leoni L, Link M, Della Bella P, Gaita F, Curnis A . Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation. 2003; 108(25):3084-91. DOI: 10.1161/01.CIR.0000103130.33451.D2. View

Boriani G, Artale P, Biffi M, Martignani C, Frabetti L, Valzania C . Outcome of cardioverter-defibrillator implant in patients with arrhythmogenic right ventricular cardiomyopathy. Heart Vessels. 2007; 22(3):184-92. DOI: 10.1007/s00380-006-0963-8. View

Valente M, Calabrese F, Thiene G, Angelini A, Basso C, Nava A . In vivo evidence of apoptosis in arrhythmogenic right ventricular cardiomyopathy. Am J Pathol. 1998; 152(2):479-84. PMC: 1857974. View

Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C . Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2000; 36(7):2226-33. DOI: 10.1016/s0735-1097(00)00997-9. View

Protonotarios N, Tsatsopoulou A, Anastasakis A, Sevdalis E, Mckoy G, Stratos K . Genotype-phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. J Am Coll Cardiol. 2001; 38(5):1477-84. DOI: 10.1016/s0735-1097(01)01568-6. View

Mckoy G, Protonotarios N, Crosby A, Tsatsopoulou A, Anastasakis A, Coonar A . Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease). Lancet. 2000; 355(9221):2119-24. DOI: 10.1016/S0140-6736(00)02379-5. View

Protonotarios N, Tsatsopoulou A, Gatzoulis K . Arrhythmogenic right ventricular cardiomyopathy caused by a deletion in plakoglobin (Naxos disease). Card Electrophysiol Rev. 2002; 6(1-2):72-80. DOI: 10.1023/a:1017943323473. View

10.

Fontaine G, Fontaliran F, Andrade F, Velasquez E, Tonet J, Jouven X . The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy. Heart Vessels. 1995; 10(5):227-35. DOI: 10.1007/BF01744901. View

11.

Thiene G, Corrado D, Nava A, Rossi L, Poletti A, Boffa G . Right ventricular cardiomyopathy: is there evidence of an inflammatory aetiology?. Eur Heart J. 1991; 12 Suppl D:22-5. DOI: 10.1093/eurheartj/12.suppl_d.22. View

12.

Basso C, Thiene G, Corrado D, Angelini A, Nava A, Valente M . Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis?. Circulation. 1996; 94(5):983-91. DOI: 10.1161/01.cir.94.5.983. View

13.

McKenna W, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G . Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International.... Br Heart J. 1994; 71(3):215-8. PMC: 483655. DOI: 10.1136/hrt.71.3.215. View