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(Circulation. 2000;101:e101.)
© 2000 American Heart Association, Inc.
Circulation Electronic Pages |
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Need for an International Registry
From the Departments of Cardiology (D.C., A.N.) and Pathology (G.T.), University of Padova, Italy; Department of Cardiology (G.F.), Hôpital Jean Rostand, Ivry-sur-Seine, Paris, France; Section of Cardiology (F.I.M.), University of Arizona, Tucson, Az; Department of Cardiological Sciences (W.J.M.), St George’s Hospital Medical School, London, UK; and Department of Cardiology and Angiology (T.W), T. Medizinische Klinik und Poliklinik, Innere Medizin, Westfälische Wilhelms-Universität, Münster, Germany.
Correspondence to Domenico Corrado, MD, Department of Cardiology, University of Padua Medical School, Via N. Giustiniani 2—35121 Padova, Italy. E-mail cardpath{at}ux.1.unipd.it
Abstract—Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar RV involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group on ARVD/C of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since it first was described in 1977. The present article focuses on important but still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed and thus answer pending questions. The registry also will facilitate pathological, molecular, and genetics research on the causes and pathogenesis of the ARVD/C. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.
Key Words: cardiomyopathy • electrophysiology • genetics • death, sudden • tachyarrhythmias
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