ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA. CLINICAL CASE
Abstract:
Arrhythmogenic right ventricular dysplasia (ARVD) is a rare form of spontaneous or familyinherited
cardiomyopathy, autosomal dominant or recessive, characterized by progressive loss of
myocyte mass and its replacement with fibroadipose tissue, which leads to ventricular dysfunction,
ventricular arrhythmias and sudden death. Studies have shown that ARVD is present in 0.08-9% of cases
of sudden death. ARVD main feature is the tendency to ventricular arrhythmias and sudden death even
in the absence of clinical ventricular dysfunction. Physical exertion produces catecholamines release
and hyperextension of myocardial fibers, which can lead to stimulation of ventricular displasic areas,
producing arrhythmias. Clinical case: Young man, 32 years old, smoker without no pathological
personal history suffered at home a cardiac arrest; he is resuscitated, stabilized. ECG: rare ESV of
normal aspect of arrhythmic episodes, crochet discrete terminal portion of QRS in V1 (epsilon wave).
MRI: morphologically, there has been highlighted a small area located about 2 cm at anterior apical
ventricular septal level with increased signal; paradoxical motion in right septal anterior ventricular
wall with little area “buldging” in systole. There has been decided to implant a cardiac defibrillator.
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