An ECG changed the life of a young boy: a case of arrhythmogenic right ventricular dysplasia

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a progressive condition with the right ventricular myocardium being replaced by fibrofatty tissue. It is a hereditary disorder mostly caused by desmosome gene mutations. The prevalence of arrhythmogenic right ventricular cardiomyopathy is about 1/1000-5000. Clinical presentation is usually related to ventricular tachycardias, syncope, presyncope or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. We report a case of a 17-year-old boy from Turkey, who was referred to our cardiology department for an ECG, required of him prior to joining a football team.

Figure 1

ECG of frequent ventricular extrasystoles in left bundle branch block pattern and non-sustained ventricular tachycardia with inferior axis.

Figure 2

(A) Transthoracic echocardiographic images of the right ventricular (RV) dilation with depressed thinning of the RV free wall. (B) Cardiac MRI of the RV dilation with aneurysmatic ectasia of the RV lateral wall. (C and D) Cardiac MRI: extensive fat infiltration of the enlarged RV free wall during T1-weighted (C) and conventional spin-echo images (D).