American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: http://www.sciepub.com/journal/ajmcr Editor-in-chief: Samy, I. McFarlane
Open Access
Journal Browser
Go
American Journal of Medical Case Reports. 2016, 4(3), 83-86
DOI: 10.12691/ajmcr-4-3-3
Open AccessCase Report

Breaking It Gently: A Rare Case of Arrhythmogenic Right Ventricular Cardiomyopathy Presenting as Sustained Ventricular Tachycardia

Glenmore Lasam1, , Roberto Roberti2 and Roberto Ramirez1

1Department of Medicine, Overlook Medical Center, Summit, NJ 07901 USA

2Section of Cardiology, Overlook Medical Center, Summit, NJ 07901 USA

Pub. Date: March 25, 2016

Cite this paper:
Glenmore Lasam, Roberto Roberti and Roberto Ramirez. Breaking It Gently: A Rare Case of Arrhythmogenic Right Ventricular Cardiomyopathy Presenting as Sustained Ventricular Tachycardia. American Journal of Medical Case Reports. 2016; 4(3):83-86. doi: 10.12691/ajmcr-4-3-3

Abstract

We report a case of a 42-year-old female with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) who presented with recurrent episodes of palpitations, dizziness and lightheadedness. On evaluation, she developed a new onset sustained ventricular tachycardia (VT) and the diagnosis was confirmed through genetic testing, cardiac imaging, and electrophysiological study. Insertion of automatic implantable cardioverter defibrillator (AICD), antiarrhythmic medication, and ventricular ectopy radiofrequency ablation afforded improvement of symptoms. In conclusion, combination of these treatment modalities abated ventricular tachycardia from ARVC.

Keywords:
arrhythmogenic right ventricular cardiomyopathy ventricular tachycardia arrhythmia anti-arrhythmic medications automatic implantable cardioverter defibrillator radiofrequency ablation

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References:

[1]  Fontaine G, Frank R, Vedel J, et al. Stimulation studies and epicardial mapping in ventricular tachycardia: study of mechanisms and selection for surgery. In: Kulbertus HE, editor. Reentrant arrhythmias. Lancaster: MTP; 1977. p. 334-50.
 
[2]  Dalal D, James C, Devanagondi R, et al. Penetrance of mutations in plakophilin-2 among families with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2006 Oct 3. 48(7):1416-24.
 
[3]  Moric-Janiszewska E, Markiewicz-Loskot G. Review on the genetics of arrhythmogenic right ventricular dysplasia. Europace. 2007 May. 9(5):259-66.
 
[4]  Rampazzo A, Nava A, Danieli GA, et al. The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23-q24. Hum Mol Genet. 1994;3:959-962.
 
[5]  Domenico Corrado, MD, PhD; Gaetano Thiene, MD. Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Clinical Impact of Molecular Genetic Studies. Circulation. 2006;113:1634-1637.
 
[6]  McKoy G, Protonotarios N, Crosby A, et al. Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease). Lancet. 2000;355:2119-24.
 
[7]  Rampazzo A, Nava A, Malacrida S, et al. Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet. 2002;71:1200 -1206.
 
[8]  van Tintelen JP, Hauer RNW. Cardiomyopathies: New test for arrhythmogenic right ventricular cardiomyopathy. Nature Reviews Cardiology. 2009; 6: 450-451.
 
[9]  Munkholm J, Andersen CB, Ottesen GL. Plakoglobin: A diagnostic marker of arrhythmogenic right ventricular cardiomyopathy in forensic pathology? Forensic Sci Med Pathol. 2015; 11:47-52.
 
[10]  Peters S, Trümmel M, Meyners W. Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital. Int J Cardiol. 2004 Dec. 97(3):499-501.
 
[11]  Dalal D, Nasir K, Bomma C, et al. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation. 2005 Dec 20. 112(25):3823-32.
 
[12]  McKenna WJ, Thiene G, Nava A, et al. 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 Society and Federation of Cardiology. Br Heart J. 1994;71:215-8.
 
[13]  Wei YJ, Huang YX, Shen Y et al. Proteomic analysis reveals significant elevation of heat shock protein 70 in patients with chronic heart failure due to arrhythmogenic right ventricular cardiomyopathy. Molecular and Cellular Biochemistry, 2009, Volume 332, Number 1-2, Page 103.
 
[14]  Kostis WJ, Tedford RJ, Miller D et al. Troponin-I elevation in a young man with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of Interventional Cardiac Electrophysiology, 2008, Volume 22, Number 1, Page 49.
 
[15]  Sommariva E, D’Alessandra Y, Brambilla S et al. MiR-320 as Potential Biomarker of Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation. 2014;130:A13136.
 
[16]  Hulot JS, Jouven X, Empana JP, et al. Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation. 2004;110(14):1879.
 
[17]  Nava A, Bauce B, Basso C, et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2000;36(7):2226.
 
[18]  Maron BJ, Carney KP, Lever HM, et al. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2003;41(6):974.
 
[19]  Thiene G, Nava A, Corrado D, et al. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318(3):129.
 
[20]  Tabib A, Loire R, Chalabreysse L, et al. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia. Circulation. 2003;108(24):3000.
 
[21]  McKenna WJ, Cheng A, Downey BC. Clinical manifestations and diagnosis of arrhythmogenic right ventricular cardiomyopathy. UpToDate, Waltham, MA. (Accessed on September 13, 2015.)
 
[22]  Gemayel C, Pelliccia A, MD, Thompson PD. Arrhythmogenic Right Ventricular Cardiomyopathy. Journal of the American College of Cardiology. 2001; 38(7):1773-81.
 
[23]  Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy: an analysis using 123I metiodobenzylguanidine scintigraphy. Circulation 1994;89:667-83.
 
[24]  Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol. 2006;48(5):e247.
 
[25]  Dalal D, Jain R, Tandri H, et al. Long-term efficacy of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2007;50(5):432.
 
[26]  Verma A, Kilicaslan F, Schweikert RA, et al. Short- and long-term success of substrate-based mapping and ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia. Circulation. 2005;111(24):3209.
 
[27]  Marchlinski FE, Zado E, Dixit S, et al. Electroanatomic substrate and outcome of catheter ablative therapy for ventricular tachycardia in setting of right ventricular cardiomyopathy. Circulation. 2004;110(16):2293.
 
[28]  Epstein AE, DiMarco JP, Ellenbogen KA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2013 Jan;61(3):e6-75. Epub 2012 Dec 19.
 
[29]  Schinkel AF. Implantable cardioverter defibrillators in arrhythmogenic right ventricular dysplasia/cardiomyopathy: patient outcomes, incidence of appropriate and inappropriate interventions, and complications.Circ Arrhythm Electrophysiol. 2013 Jun; 6(3):562-8. Epub 2013 May 14.