Arrhythmogenic right ventricular dysplasia/cardiomyopathy - Need for an international registry

Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar RV involvement and electri cal instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group on ARVD/C o f the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Card iomyopathies of the World Heart Federation is to review the considerable pr ogress in our understanding of the etiopathogenesis, morbid anatomy, and cl inical presentation of ARVD/C since it first was described in 1977. The pre sent article focuses on important but still unanswered issues, mostly regar ding risk stratification, clinical outcome, and management of affected pati ents. Because ARVD/C is relatively uncommon and any one center may have exp erience with only a few patients, an international registry is being establ ished to accumulate information and enhance the numbers of patients that ca n be analyzed and thus answer pending questions. The registry also will fac ilitate pathological, molecular, and genetics research on the causes and pa thogenesis of the ARVD/C. Furthermore, availability of an international dat abase will enhance awareness of this largely unrecognized condition among t he medical community. Physicians are encouraged to enroll patients in the I nternational Registry of ARVD/C.