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1 Jin-Seok Kim, MD Department of Cardiology, Sejong Heart Institute, Sejong General Hospital. Bucheon, Gyeonggi-do, Korea Arrhythmogenic right ventricular dysplasia/cardiomyopathy ABSTRACT Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a clinical entity characterized by ventricular arrhythmias and a specific ventricular pathology showing fibrofatty infiltration of the right ventricular (RV) free wall. ARVD/C is known as an important cause of sudden cardiac death in young adults. We reported a case of a 38-year-old female who visited us for palpitation and a history of syncope, and was diagnosed with ARVD/C. Ventricular arrhythmias such as frequent ventricular premature beats and ventricular tachycardia were documented. Also, RV enlargement and dysfunction were noted on imaging. Finally, she received an ICD implantation. Key words: ventricular tachycardia arrhythmogenic right ventricular dysplasia cardiomyopathy Received: June 28, 2010 Revision Received: August 11, 2010 Accepted: Correspondence: Jin-Seok Kim, MD, Division of Cardiology, Department of Cardiology, Sejong Heart Institute, Sejong General Hospital Sosabon-2-dong, Sosa-gu, Bucheon , Korea Tel: , Fax: heartmania@nate.com 39

T wave inversion is noted at precordial leads. B.

2 A B Figure 1. Baseline ECG on admission (A) and follow-up (B). A. Frequent ventricular ectopies with LBBB configuration are shown. T wave inversion is noted at precordial leads. B. On follow-up, ECG evolution was noted. It shows deep T wave inversions in V1 to V3. ECG; electrocardiogram 40 Journal of Cardiac Arrhythmia

Figure 2. Documented monomorphic ventricular tachycardia with left bundle branch block configuration on 24 hours Holter recordings. A B Figure 3.

3 Figure 2. Documented monomorphic ventricular tachycardia with left bundle branch block configuration on 24 hours Holter recordings. A B Figure 3. Cardiac MRI showed bulging of RV apex with dyskinetic wall motion and focal aneurysm (bold white arrow) on systolic phase (A), and suspected linear enhancement (thin white arrow) along the RV myocardium, especially at apical portion (B). RV; right ventricles 41

4 References 1. Gamayel C, Pelliccia A, Thompson PD. Arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2001;38: Sen-Chowdhry S,Lowe MD, Sporton SC, McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med. 2004;117: Corrado D, Fontaine G, Marcus FI, McKenna WJ, Nava A, Thiene G, Wichter T. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation. Circulation. 2000;101:e Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med. 1998;339: Dalal D, Nasir K, Bomma C, Prakasa K, Tandri H, Piccini J, Roguin A, Tichnell C, James C, Russell SD, Judge DP, Abraham T, Spevak PJ, Bluemke DA, Calkins H. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation. 2005;112: Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C, Daliento L, Buja G, Corrado D, Danieli GA, Thiene G. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2000;36: Tabib A, Loire R, Chalabreysse L, Meyronnet D, Miras A, Malicier D, Thivolet F, Chevalier P, Bouvagnet P. 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: Angelini A, Basso C, Nava A, Thiene G, Endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy. Am Heart J. 1996;132: McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom- Lundqvist C, Fontaine G, Camerini F. 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: Journal of Cardiac Arrhythmia

5 10. European Heart Rhythm Association; Heart Rhythm Society, Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL; American College of Cardiology; American Heart Association Task Force; European Society of Cardiology Committee for Practice Guidelines. 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 Practical 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:e Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA 3rd, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Buller CE, Creager MA, Ettinger SM, Faxon DP, Halperin JL, Hiratzka LF, Hunt SA, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Ornato JP, Page RL, Riegel B, Tarkington LG, Yancy CW; American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices); American Association for Thoracic Surgery; Society of Thoracic Surgeons. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities; a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation. 2008;117:e

L A P R RAA LMCA RVOT RV Free wall RV Septum Main Topic Reviews A RA L N R P RCA LA Figure 1. Anatomy of the outflow tract. Anatomy of the typical rig

L A P R RAA LMCA RVOT RV Free wall RV Septum Main Topic Reviews A RA L N R P RCA LA Figure 1. Anatomy of the outflow tract. Anatomy of the typical rig Main Topic Reviews 특발성심실빈맥 차의과학대학교내과학교실성정훈 Jung-Hoon Sung, MD, PhD Division of Cardiology, Department of Internal Medicine, CHA Bundang Medical Center, CHA University, Gyeonggi-do, Korea Idiopathic ventricular