Left Dominant Arrhythmogenic Cardiomyopathy Causing Sustained Ventricular Tachycardia – A Case Report

Abstract

A 56-year-old white man was admitted to hospital because of palpitations and dyspnoea. He had no family history of cardiomyopathy or sudden death, and the electrocardiograms (ECG) of his son and parents were normal. He had an history of palpitations, and in the emergency department ECG showed sustained ventricular tachycardia-flutter with inferior axis right bundle branch block (RBBB) in V1 and heart rate 250 beats per minute. His standard ECG showed a complete RBBB pattern with minor ST-T segment changes. 2D echo and coronary angiography were normal, while magnetic resonance imaging (MRI) revealed a reduction in left mid-lateral wall thickening with hypokinetic movement and mild reduction of ejection fraction. An Extensive sub-epicardial area of conspicuous left ventricular intra-myocardial fat and sub-epicardial left ventricular late enhancement in lateral wall, was also seen at MRI. Case reportA 56-year-old white man was admitted to hospital because of palpitations and dyspnoea. He had no family history of cardiomyopathy or sudden death. He complained of palpitations starting in 2002, but had not been to see a doctor. In the emergency department, the ECG showed sustained ventricular tachycardia-flutter with apparently inferior axis right bundle branch block (RBBB) in V1 and heart rate 250 beats per minute (Figure 1). The ventricular tachycardia was associated with initial signs and symptoms of left heart failure and the patient was referred to the Coronary Care Unit where sinus rhythm was restored by direct current electric shock. Symptoms promptly restored after the procedure. The results of the investigation performed are listed in Table 1. LimitationsGenetic studies have not yet been performed because of high cost/ effectiveness ratio. DiscussionAfter the description of Markus and Fontaine, 1 the arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has crossed the borders of the right ventricle. LDAC has been recognised as an associated or isolated entity also characterised pathologically by fibro-adipose replacement of the LV, often occurring as a circumferential band in the outer third of the myocardium and the right side of the inter-ventricular septum.2-5 First described at post-mortem examination in sudden cardiac death (SCD) victims,