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      Treatment of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia : An International Task Force Consensus Statement

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          Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.

          W. J. McKenna, G. Thiene, A. Nava … (1994)
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            Arrhythmogenic Right Ventricular Cardiomyopathy: Dysplasia, Dystrophy, or Myocarditis?

            Cristina Basso, Gaetano Thiene, Domenico Corrado … (1996)
            Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a frequent cause of sudden death in young individuals and athletes. Although familial occurrence has been documented and a gene defect was recently localized on chromosome 14q23-q24 the etiopathogenesis of the disease is still obscure. A pathological study was conducted in 30 hearts with ARVC (age range, 15 to 65 years; mean, 28 years). In the 27 autopsy cases, the mode of death was sudden in 24 and congestive heart failure in 3. ECG, available in 19 cases, showed inverted T waves in the right precordial leads in 15 cases (79%) and ventricular arrhythmias in 15 (79%). Right ventricular aneurysms were present in 15 hearts (50%) and located in the inferior wall in 12. Left ventricle and ventricular septum were involved in 14 (47%) and 6 (20%) cases, respectively. Scattered foci of lymphocytes with myocardial death were observed in 20 cases (67%). Electron microscopy studies, although confirming the myocardial death and lymphocyte infiltrates, did not show any specific ultrastructural substrate. Two pathological patterns, fatty (40%) and fibrofatty (60%), were identified. The fibrofatty pattern was associated with a thinner right ventricular wall (P < .0001) and a higher occurrence of focal myocarditis (P < .001). In sections of right ventricular free wall with maximal fatty infiltration, the mean percentage area of fatty tissue was 35.9 +/- 11.1% in control versus 80.4 +/- 9.6% in the ARVC, fatty variety (P < .00001). Involvement of the left ventricle and/or ventricular septum, right ventricular aneurysms, and inflammation were found almost exclusively in the fibrofatty variety. In the fibrofatty variety of ARVC, the myocardial atrophy appears to be the consequence of acquired injury (myocyte death) and repair (fibrofatty replacement), mediated by patchy myocarditis. Whether the inflammation is a primary event or a reaction to spontaneous cell death remains unclear.
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              Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy.

              Andrea Nava, Barbara Bauce, Cristina Basso … (2000)
              We sought to define the clinical picture and natural history of familial arrhythmogenic right ventricular cardiomyopathy (ARVC). Arrhythmogenic right ventricular cardiomyopathy is a myocardial disease, often familial, clinically characterized by the impending risk of ventricular arrhythmias and sudden death. Thirty-seven ARVC families of northeast Italy were studied. Probands had a histologic diagnosis of ARVC, either at autopsy (19 families) or endomyocardial biopsy (18 families). Protocol of the investigation included basal electrocardiogram (ECG), 24-hour ECG, signal-averaged ECG, stress test and two-dimensional Doppler echocardiography. Invasive evaluation was performed when deemed necessary. Of the 365 subjects, 151 (41%) were affected, 157 (43%) were unaffected, 17 (5%) were healthy carriers, and 40 (11%) were uncertain. Mean age at diagnosis was 31+/-13 years. By echocardiography, 64% had mild, 30% had moderate, and 6% had severe form. Forty percent had ventricular arrhythmias, 49 were treated with antiarrhythmic drugs, and two were treated with implantable cardioverter defibrillators. Sport activity was restricted in all. Of the 28 families who underwent linkage analysis, 6 mapped to chromosome 14q23-q24, 4 to 1q42-q43, and 4 to 2q32.1-q32.3. No linkage with known loci was found in four families and 10 had uninformative results. During a follow-up of 8.5+/-4.6 years, one patient died (0.08 patient/year mortality), and 15 developed an overt form of ARVC. Arrhythmogenic right ventricular cardiomyopathy is a progressive disease appearing during adolescence and early adulthood. Systematic evaluation of family members leads to early identification of ARVC, characterized by a broad clinical spectrum with a favorable outcome. In the setting of positive family history, even minor ECG and echocardiographic abnormalities are diagnostic.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Circulation
                Journal ID (iso-abbrev): Circulation
                Journal ID (publisher-id): CIR
                Title: Circulation
                Publisher: Lippincott Williams & Wilkins
                ISSN (Print): 0009-7322
                ISSN (Electronic): 1524-4539
                Publication date (Print): 4 August 2015
                Publication date (Electronic): 03 August 2015
                Volume: 132
                Issue: 5
                Pages: 441-453
                Affiliations
                From the Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy (D.C., B.B., C.Basso, G.T.); Heart Center Osnabrück-Bad Rothenfelde, Marienhospital Osnabrück, Osnabrück, Germany (T.W.); New England Cardiac Arrhythmia Center, Tufts University School of Medicine, Boston, MA (M.S.L., N.A.M.E.); ICIN-Netherlands Heart Institute, Utrecht, The Netherlands (R.N.W.H.); University of Pennsylvania Health System, Philadelphia, PA (F.M.); First Cardiology Department, University of Athens, Medical School, Athens, Greece (A.A.); Cardiovascular Center, University Hospital Zurich, Zurich, Switzerland (C. Brunckhorst, C.S., F.D.); Yannis Protonotarios Medical Centre, Hora Naxos, Greece (A.T., N.P.); Johns Hopkins Hospital, Baltimore, MD (H.T., H.C.); University Hospital of Münster, Münster, Germany (M.P.); Center of Sports Sciences, Rome, Italy (A.P.); The Heart Hospital, London, UK (W.J.M.); and University of Arizona, Tucson (F.I.M.).
                Author notes
                Correspondence to Domenico Corrado, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Via N. Giustiniani 2, Padova 35121, Italy. E-mail domenico.corrado@ 123456unipd.it
                Article
                Accession ID: 00011
                DOI: 10.1161/CIRCULATIONAHA.115.017944
                PMC ID: 4521905
                PubMed ID: 26216213
                SO-VID: 696f85eb-ba5b-4449-bad4-62a571a2c25e
                Copyright © © 2015 The Authors.
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                Circulation is published on behalf of the American Heart Association, Inc., by Wolters Kluwer. This is an open access article under the terms of the Creative Commons Attribution Non-Commercial-NoDervis License, which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited.

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