Service de rythmologie, hôpital Pitié-Salpêtrière, 47-83 bd de l’Hôpital, 75013 Paris
Recent advances in molecular biology have led to the proposal of the descriptive term of “right ventricle cardiomopathies” to define a group of diseases affecting the right ventricle. “Arrhythmogenic right ventricular dysplasia”, Brugada syndrome and right ventricular outflow tract tachycardia are the most frequent forms of presentation. Several rare variants, recently identified, have also improved our understanding of these cardiomyopathies. This is important for the clinician, because of the therapeutic consequences. Treatments include antiarrhythmic drugs, surgery, ablative techniques and implantable defibrillators.