This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Gemayel, C. Articles by Thompson, P. D. Search for Related Content PubMed PubMed Citation Articles by Gemayel, C. Articles by Thompson, P. D.

REVIEW ARTICLE

Arrhythmogenic right ventricular cardiomyopathy

Carol Gemayel, MD^*, Antonio Pelliccia, MD and Paul D. Thompson, MD, FACC^*,*

^* Hartford Hospital, Division of Cardiology, Hartford, Connecticut, USA
Institute of Sports Science, Rome, Italy

Manuscript received February 21, 2001; revised manuscript received August 21, 2001, accepted August 31, 2001.

^* Reprint requests and correspondence: Dr. Paul D. Thompson, Division of Cardiology, Hartford Hospital, 80 Seymour Street, Hartford, Connecticut 06102 USA
pthomps{at}harthosp.org

Arrythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a cardiomyopathy characterized pathologically by fibrofatty replacement primarily of the RV and clinically by life-threatening ventricular arrhythmias in apparently healthy young people. The prevalence of the disease has been estimated at 1 in 5,000 individuals, although this estimate will likely increase as awareness of the condition increases among physicians. Arrythmogenic RV cardiomyopathy is recognized as a cause of sudden death during athletic activity because of its association with ventricular arrhythmias that are provoked by exercise-induced catecholamine discharge. Diagnosis may be difficult because many of the electrocardiographic abnormalities mimic patterns seen in normal children, and the disease often involves only patchy areas of the RV. For this reason, international diagnostic criteria for ARVC were proposed by an expert consensus panel in 1996. Treatment is directed to preventing life-threatening cardiac arrhythmias with medications and the use of implantable defibrillators. This article will present in detail the etiology, clinical presentation, diagnosis and management of this condition.

Abbreviations and Acronyms   ARVC = arrhythmogenic right ventricular cardiomyopathy   CHF = congestive heart failure   CT = computerized tomography   ECG = electrocardiogram   ICD = implantable cardioverter defibrillator   LV = left ventricle or left ventricular   MRI = magnetic resonance imaging   NSVT = nonsustained ventricular tachycardia   PVC = premature ventricular complex   RBBB = right bundle branch block   RV = right ventricle or right ventricular   RVOT = right ventricular outflow tachycardia   VT = ventricular tachycardia

This article has been cited by other articles:

				F. Djouadi, Y. Lecarpentier, J.-L. Hebert, P. Charron, J. Bastin, and C. Coirault A potential link between peroxisome proliferator-activated receptor signalling and the pathogenesis of arrhythmogenic right ventricular cardiomyopathy Cardiovasc Res, July 1, 2009; (2009) cvp183v2. [Abstract] [Full Text] [PDF]

				B. A. Koplan and W. G. Stevenson Ventricular Tachycardia and Sudden Cardiac Death Mayo Clin. Proc., March 1, 2009; 84(3): 289 - 297. [Abstract] [Full Text] [PDF]

				T. Gilljam and C.-H. Bergh Right ventricular cardiomyopathy: timing of heart transplantation in Uhl's anomaly and arrythmogenic right ventricular cardiomyopathy Eur J Heart Fail, January 1, 2009; 11(1): 106 - 109. [Abstract] [Full Text] [PDF]

				R. F. Padera Jr. and F. J. Schoen Pathology of Cardiac Surgery Card. Surg. Adult, January 1, 2008; 3(2008): 111 - 178. [Full Text]

				J.G. Coghlan and J. Davar How should we assess right ventricular function in 2008? Eur. Heart J. Suppl., December 1, 2007; 9(suppl_H): H22 - H28. [Abstract] [Full Text] [PDF]

				R. J. Myerburg and V. L. Vetter Electrocardiograms Should Be Included in Preparticipation Screening of Athletes Circulation, November 27, 2007; 116(22): 2616 - 2626. [Full Text] [PDF]

				E. A. Stephenson and C. I. Berul Electrophysiological Interventions for Inherited Arrhythmia Syndromes Circulation, August 28, 2007; 116(9): 1062 - 1080. [Full Text] [PDF]

				R. Nijveldt, A. M. Beek, T. Germans, O. Bondarenko, and A. C. van Rossum Arrhythmogenic right ventricular cardiomyopathy with evidence of biventricular involvement Can. Med. Assoc. J., June 19, 2007; 176(13): 1819 - 1821. [Full Text] [PDF]

				J Marietta Clewing, B. C Antalfy, T. Lucke, B. Najafian, K. M Marwedel, A. Hori, R. M Powel, A F Safo Do, L. Najera, K. SantaCruz, et al. Schimke immuno-osseous dysplasia: a clinicopathological correlation J. Med. Genet., February 1, 2007; 44(2): 122 - 130. [Abstract] [Full Text] [PDF]

				P. D. Thompson and B. D. Levine Protecting Athletes From Sudden Cardiac Death JAMA, October 4, 2006; 296(13): 1648 - 1650. [Full Text] [PDF]

				Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, 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., September 5, 2006; 48(5): e247 - e346. [Full Text] [PDF]

				Writing Committee Members, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, 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) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society Europace, September 1, 2006; 8(9): 746 - 837. [Full Text] [PDF]

				A. Nemes, W. B. Vletter, M. F. Scholten, and F. J. ten Cate Contrast echocardiography for perfusion in right ventricular cardiomyopathy Eur J Echocardiogr, December 1, 2005; 6(6): 470 - 472. [Abstract] [Full Text] [PDF]

				C. Chimenti, M. Pieroni, A. Maseri, and A. Frustaci Histologic findings in patients with clinical and instrumental diagnosis of sporadic arrhythmogenic right ventricular dysplasia J. Am. Coll. Cardiol., June 16, 2004; 43(12): 2305 - 2313. [Abstract] [Full Text] [PDF]

				P. Motta, E. Mossad, and R. Savage Right Ventricular Exclusion Surgery for Arrhythmogenic Right Ventricular Dysplasia with Cardiomyopathy Anesth. Analg., June 1, 2003; 96(6): 1598 - 1602. [Abstract] [Full Text] [PDF]

				H. Kottkamp and G. Hindricks Right ventricular tachycardia--arrhythmogenic right ventricular cardiomyopathy or idiopathic? Eur. Heart J., May 1, 2003; 24(9): 787 - 788. [Full Text] [PDF]

				G. Aviram, J. E. Fishman, M.-L. Young, E. Redha, G. Biliciler-Denktas, and M. M. Rodriguez MR Evaluation of Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients Am. J. Roentgenol., April 1, 2003; 180(4): 1135 - 1141. [Abstract] [Full Text] [PDF]

				F. J. Schoen and R. F. Padera Jr. Cardiac Surgical Pathology Card. Surg. Adult, January 1, 2003; 2(2003): 119 - 185. [Full Text]

				M. Scoote and A. J Williams The cardiac ryanodine receptor (calcium release channel): Emerging role in heart failure and arrhythmia pathogenesis Cardiovasc Res, December 1, 2002; 56(3): 359 - 372. [Abstract] [Full Text] [PDF]