Letter to the Editor Arrhythmogenic biventricular dysplasia/cardiomyopathy masquerading as dilated cardiomyopathy with typical electrocardiographic features V. Davutoglu a, * , S. Kervancioglu b , S. Soydinc a , H. Dinckal a , A. Sirikcioglu b , I. Akdemir a , M. Aksoy a a Gaziantep University, Department of Cardiology, Sahinbey Medical Centre, Gaziantep, Turkey b Gaziantep University, Department of Radiology, Sahinbey Medical Centre, Gaziantep, Turkey Received 2 April 2003; accepted 6 May 2003 Arrhythmogenic right ventricular (RV) dysplasia/cardio- myopathy (ARVC) is a myocardial disease affecting pri- marily the RV and characterized by the gradual replacement of myocytes by adipose and fibrous tissue that lead to structural and functional abnormalities of the RV [1]. The ARVC has been found to be genetic in 30–50% of indi- viduals and is transmitted from one affected parent to a child as a dominant with incomplete inheritance [2]. The charac- teristic clinical findings include a variety of RV arrhythmias, global or regional RV and left ventricular (LV) involvement that may culminate in biventricular heart failure and elec- trocardiographic (ECG) evidence of depolarization or repo- larization abnormalities [1]. We report a case with ARVC with LV involvement in one family that masquerading as dilated cardiomyopathy with highly typical ECG features. A 16-year-old girl who developed fatigue, palpitation and prominent ascites 5 months ago. Her older sibling, who was previously considered healthy, died suddenly 19 years ago and the younger sibling developed congestive heart failure and died 9 years ago. In our case, there were prominent ascites and peripheral edema with an irregular rhythm. There was no audible murmur and lungs were clear to auscultation. ECG showed a very low voltage QRS pattern with paroxysmal atrial fibrillation and frequent ventricular extrasystoles with left bundle branch block (LBBB) morphology. Complete RBBB pattern and T-wave inversion in leads V1–V6 were observed (Fig. 1A). When we calibrate her ECG to 50 mm/s, Epsilon wave in leads V2 and V3 were become prominent (Fig. 1B) and selective prolongation of the QRS in leads V1–V3 compared with lead V6 were observed. Echocardiography showed severely hypokinetic and dilated right and left ventricle (Fig. 1) with aneurysm of the RV outflow tract (Fig. 1). A 24 h monito- rization revealed very frequent ventricular extrasystoles with some episodes of nonsustained ventricular tachycardia, with LBBB morphology, suggesting a RV origin. Magnetic resonance imaging (MRI) showed fatty replacement of right and LV myocardium. In addition, there was massive dilata- tion of the right and left heart chambers (Fig. 2C, D). The patient was diagnosed as having ARVC with LV involve- ment. The patient was initially treated with diuretics, ACE inhibitors, digoxine and spironolactone. For frequent ven- tricular extrasystoles, sotalol 80 mg 2Â1 p.o. was initiated. During follow-up, her symptoms were diminished signifi- cantly. After 2 weeks, efficacy of sotalol was determined by suppression of ventricular arrhythmias during repeated Hol- ter monitoring and exercise testing. She is currently on anti congestive heart failure and sotalol medication without complications and is doing well. The mechanism for the heart failure is dilation, thinning of the wall and progressive loss of contractile function because of myocardial fibrofatty infiltration. Clinician should be considered the possibility of ARVC if the patient has an apparent dilated cardiomyopathy with resting ECG showing right precordial T-wave changes. Along with repolarization abnormalities and conduction delays, there may also be low voltage in QRS related to the loss of RV myocardium. Selective prolongation of the QRS in leads V1–V3 compared with lead V6 is an additional major criterion [3]. Epsilon wave are a major diagnostic criterion that are found in up to 30% of cases of ARVC [4]. Epsilon wave is postexcitation electrical potentials of small ampli- tude that occur at the end of the QRS complex and at the beginning of the ST segment. They are highly specific for ARVC and reflected delayed RV activation. The most prevalent finding in TTE is a severely hypokinetic and dilated RV, although the spectrum of abnormalities may 0167-5273/$ - see front matter D 2003 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2003.05.043 * Corresponding author. Guneykent Mah., Besyuzevler Sitesi, 7 Blok. Daire No: 10, Sahinbey, 27310 Gaziantep, Turkey. Tel.: +90-342-360-6060; fax: +90-342-360-3928. E-mail address: vedatdavutoglu@hotmail.com (V. Davutoglu). www.elsevier.com/locate/ijcard International Journal of Cardiology 97 (2004) 147 – 149