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ABSTRACT
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads. Subsequent work up showed fatty infiltration of RV myocardium on cardiac magnetic resonance imaging and inducible ventricular tachycardia from the right ventricle during electrophysiologic study. Those findings confirmed the diagnosis of ARVC and warranted treatment with implantable cardioverter defibrillator. It is always exciting to encounter rare pathological entities with classic clinical findings, especially when they present as a diagnostic challenge.We were able to provide correct diagnosis and management, thereby preventing the potentially lethal consequences. Therefore, it is important to recognize the possible EKG findings of ARVC and to know when to pursue further investigations and to implement therapies.
ARTICLE HISTORY
Received 22 December 2016
Accepted 27 February 2017
KEYWORDS
T wave inversion; epsilon waves; monomorphic ventricular tachycardia; arrhythmogenic right ventricular cardiomyopathy; sudden cardiac death; implantable cardioverter defibrillator
1.Case report
A healthy 33-year-old athletic male was evaluated in cardiology clinic for worsening exertional palpitations and non-specific chest discomfort. About five years ago, he had similar complaints and prior electrocardiogram (EKG) from the initial encounter showed diffuse symmetric T wave inversions in right precordial leads. Subsequently, he had extensive work up consisting of cardiac monitor, echocardiogram (ECHO), exercise stress test and coronary angiogram, which were all unremarkable. There was no personal or family history of unexplained syncope or cardiac arrest. At this visit, the patient's EKG showed symmetrically inverted T waves and possible epsilon waves in the right precordial leads (Figures 1 and 2). ECHO was grossly unremarkable. Holter monitor showed frequent premature ventricular complexes of right ventricular (RV) origin and episodes of non-sustained ventricular tachycardia (NSVT) with left bundle branch block pattern (LBBB) (Figure 3). Cardiac magnetic resonance imaging (CMRI) showed dyskinetic right ventricle with RV ejection fraction (EF)...