Arrhythmogenic right ventricular cardiomyopathy/dysplasia is a progressive
condition with right ventricular myocardium being replaced by fibro-fatty
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 or presyncopa, or ventricular fibrillation leading to
cardiac arrest, mostly in young people and athletes. It may be difficult to
make the diagnosis of arrhythmogenic right ventricular cardiomyopathy due to
several problems arising from the specificity of electrocardiograph
abnormalities, different potential etiologies of ventricular arrhythmias with
a left bundle branch morphology, the assessment of the right ventricular
structure and function, and the interpretation of endomyocardial biopsy
findings. Therefore, standardized diagnostic criteria have been proposed by
the Study Group on arrhythmogenic right ventricular cardiomyopathy of the
European Society of Cardiology. In order to make the diagnosis of
arrhythmogenic right ventricular cardiomyopathy, a number of clinical tests
are employed, including the electrocardiogram, echocardiography, myocardial
perfusion scintigraphy, myocardial biopsy, right ventricular angiography,
cardiac magnetic resonance imaging and genetic testing. The therapeutic
options include beta blockers, antiarrhythmic drugs, catheter ablation, and
implantable cardioverter defibrillator. The implantable cardioverter
defibrillator is the most effective safe-guard against arrhythmic sudden
death. Preparticipation screening for sport eligibility has been proven to be
effective in detecting asymptomatic patients and sport disqualification has
been lifesaving, substantially declining sudden death in young athletes.
Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
