Abstract
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal dominant genetic form of cardiomyopathy (CM), which primarily affects the right ventricle (RV) and results in life threatening ventricular arrhythmias and sudden cardiac death (SCD). Diagnosis is difficult due to the broad spectrum of phenoltypic variations, especially in the early stage. Clinical suspicion should be raised in the setting of refractory ventricular tachycardia originating from the RV, and the final diagnosis could be made based on the combination of electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Implantable cardioverter-defibrillator (ICD) implantation is an effective option for the treatment of ARVC.
Highlights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is one of the major causes of recurrent ventricular tachycardia (VT) and sudden cardiac death in young people
ARVC is characterized by the progressive replacement of right ventricular myocytes by fibrous or fibro-fatty tissue which results in an area of slow conduction and dispersion of refractoriness [1], leading to reentrant ventricular tachyarrhythmia
The patient firstly referred to our hospital with recurrent VT of left bundle branch block (LBBB) morphology and an inferior axis, presenting as an R wave in II, III and AVF leads; QS wave in AVR and AVR leads with precordial R/S transition in V4, which was typical features of idiopathic VT arising from the right ventricular outflow tract (RVOT)
Summary
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is one of the major causes of recurrent ventricular tachycardia (VT) and sudden cardiac death in young people. QRS morphology of VT and abnormalities of precordial leads in sinus rhythm, in association with echocardiography (UCG) and cardiac magnetic resonance imaging (CMR), play an important role in early diagnosis of ARVC. Presented is a 25-year-old male soldier who suffered from recurrent episodes of palpitation and presyncope due to VT, which resembled that of idiopathic right ventricular outflow tract (RVOT) origin, diagnosed as idiopathic RVOT VT in the presence of echocardiographic normal heart and received oral administration of metoprolol which eventually proved to be ineffective. Surface 12 leads ECG indicated persistent T wave inversion in V1-V4, which was suggestive of ARVC, and following CMR scan confirmed the diagnosis, and subsequently the patient was treated with implantable cardioverter defibrillator (ICD)
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