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Case Report Cardiomyopathy Left Dominant Arrhythmogenic Cardiomyopathy Causing Sustained Ventricular Tachycardia – A Case Report Francesco Guglielmi, 1 Sergio Cannas, 1 Rocco Arancio, 1 Nicolò Martini 2 and Bortolo Martini 1 1.Cardiac Unit, Alto Vicentino Hospital, Santorso (VI), Italy; 2. Ferrara Medical School, Ferrara University, Ferrara, Italy 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. No abnormalities of right ventricular size, function or tissue characterisation were detected. A diagnosis of atypical left dominant arrhythmogenic cardiomyopathy was made, based on clinical and images basis, as gene testing was not available. Keywords Ventricular tachycardia, left dominant arrhythmogenic cardiomyopathy, desmoplakin Disclosure: Francesco Guglielmi, Sergio Cannas, Rocco Arancio, Nicolò Martini, and Bortolo Martini have no conflicts of interest to declare in relation to this article. No funding was received in the publication of the article. Compliance with Ethical Guidance: All procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki Declaration of 1975 and subsequent revisions, and informed consent was received from the patient involved in this case study. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution, adaptation and reproduction provided the original author(s) and source are given appropriate credit. Received: 26 January 2016 Accepted: 24 February 2016 Citation: European Journal of Arrhythmia & Electrophysiology, 2016;2(1):37–9 Correspondence: Bortolo Martini, MD, Via Gioberti 9, 36016 Thiene (VI), Italy. E: bortolo.martini@gmail.com The purpose of this report is to describe an unusual case of arrhythmogenic cardiomyopathy, involving the left ventricle (LV), with an atypical standard electrocardiogram (ECG) pattern. Sustained ventricular arrhythmias in a middle-aged subject are usually related to ischemic heart disease. A latent or overt cardiomyopathy is probably the second cause of a rhythm disturbance especially. Idiopathic forms are rare at this age, but only a detailed investigation can clarify the organic substrate, that was so unusual in the following report. A final diagnosis of left dominant arrhythmogenic cardiomyopathy (LDAC) was reached and the patient was discharged on bisoprololo 10 mg twice per day with indication for very close follow-up and restriction of physical activity. The choice of not implanting an implantable cardioverter-defibrillator or to perform an ablation, was decided after a discussion with the patient, who refused an invasive procedure. After a 4-year follow-up, the patient is asymptomatic, and no repetitive ventricular arrhythmias have been detected during Holter monitoring and exercise stress test. Case report Limitations 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. 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. Genetic studies have not yet been performed because of high cost/ effectiveness ratio. TOU CH MED ICA L MEDIA Discussion After 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, 37