Arrhythmogene rechtsventrikuläre Kardiomyopathie

Abstract

Arrhythmogenic right ventricular cardiomyopathy (arrhythmogenic right ventricular dysplasia) is a rare syndrome that affects mostly adolescents and young adults. It is characterized by right ventricular atrophy and dilatation. Arrhythmias and syncopes as well as signs of right heart failure can be seen. Some patients report an increased frequency of sudden cardiac deaths in their families. Diagnostic options are for instance ECG, 24 hour ECG and cardiac imaging (Echocardiography, Cardiac MRI). The diagnosis can be confirmed by myocardial biopsy. In the therapy besides other options cardioverter defibrillators or antiarrhythmics like sotalol, amiodarone or beta blockers play a role.

Although there are similar morphologic changes, Uhl's disease seems to be an independent disease. It is associated with a very thin right ventricular wall and therefore with an increased risk of right heart failure.