Arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy

The disease is characterised by a replacement of cardiac muscle tissue by connective tissue and fat, particularly in the right ventricle. This may lead to dilatation (widening) of the right ventricle, dysfunction and aneurysm formation. As a result, malignant arrhythmias can occur. The disease often occurs in men between 20 and 40 years of age and is inherited as an autosomal dominant trait. The estimated prevalence in the general population is 1 in 1000. Often, the first symptoms are seen during physical exercise in the form of syncope, arrhythmias and even SCD. If affected, patients should not participate in competitive sport.