Abstract
The first monograph on arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) was published 10 years ago [1]. Since then, there have been major advancements in the basic knowledge of the disease as well as a better understanding of the diagnosis and treatment. A workshop was held in Venice, Italy on October 3, 2005, where research on various aspects of this disease, both biological and clinical was presented.
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References
Nava A, Rossi L, Thiene G (1997) Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Elsevier, Amsterdam.
Corrado D, Fontaine G, Marcus FI et al (2000) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy 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. Circulation 101:E101–106
Basso C, Wichter T, Danieli GA et al (2004) Arrhythmogenic right ventricular cardiomyopathy: Clinical registry and database, evaluation of therapies, pathology registry, DNA banking. Eur Heart J 25:531–534
Marcus F, Towbin JA, Zareba W et al (2003) ARVD/C Investigators. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C): A multidisciplinary study: Design and protocol. Circulation 107:2975–2978
McKoy G, Protonotarios N, Crosby A et al (2000) Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease).Lancet 355:2119–2124
Rampazzo A, Nava A, Malacrida S et al (2002) Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet 71:1200–1206
Gerull B, Heuser A, Wichter T et al (2004) Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet 36:1162–1164
Pilichou K, Nava A, Basso C et al (2006) Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation 113:1171–1179
Syrris P, Ward D, Evans A et al (2006) Arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in the desmosomal gene desmocollin-2. Am J Hum Genet 79:978–984
Thiene G, Basso C, Corrado D (2004) Cardiomyopathies: Is it time for a molecular classification? Eur Heart J 25:1772–1775
Maron BJ, Towbin JA, Thiene G et al (2006) Contemporary definitions and classification of the cardiomyopathies: An American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 113:1807–1816
Corrado D, Thiene G (2006) Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Clinical impact of molecular genetic studies. Circulation 113:1634–1637
Basso C, Czarnowska E, Della Barbera M et al (2006) Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: An electron microscopy investigation on endomyocardial biopsies. Eur Heart J 27:1847–1854
Bauce B, Basso C, Rampazzo A et al (2005) Clinical profile of four families with arrhythmogenic right ventricular cardiomyopathy caused by dominant desmoplakin mutations. Eur Heart J 26:1666–1675
Norman M, Simpson M, Mogensen J et al (2005) Novel mutation in desmoplakin causes arrhythmogenic left ventricular cardiomyopathy. Circulation 112:636–642
Sen-Chowdry S, Prasad SK, Syrris P et al (2006) Cardiovascular magnetic resonance in arrhythmogenic right ventricular cardiomyopathy revisited: Comparison with task force criteria and genotype. J Am Coll Cardiol 48:2132–2140
Corrado D, Basso C, Leoni L et al (2005) Threedimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 111:3042–3050
Corrado D, Leoni L, Link MS et al (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108:3084–3091
Wichter T, Paul M, Wollmann C et al (2004) Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy. Single-center experience of long-term follow-up and complications in 60 patients. Circulation 109:1503–1508
Corrado D, Basso C, Pavei A et al (2006) Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA 296:1593–1601
McKenna WJ, Thiene G, Nava A et al (1994) 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. Br Heart J 71:215–218
Garcia-Gras E, Lombardi R, Giocondo MJ et al (2006) Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. J Clin Invest 116:1825–1828
Yang Z, Bowles NE, Scherer SE et al (2006) Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Res 99:646–655
Kirchhof P, Fabritz L, Zwiener M et al (2006) Age and training dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin deficient mice. Circulation 114:1799–1806
Marcus F, Towbin JA (2006) The mystery of arrhythmogenic right ventricular dysplasia/cardiomyopathy. From observation to mechanistic explanation. Circulation 114:1794–1795
Sermon K, Van Steirteghem A, Liebaers I (2004) Preimplantation genetic diagnosis. Lancet 363:133–1641
Dalla Volta S, Battaglia G, Zerbini E (1961) “Auricularization” of right ventricular pressure curve. Am Heart J 61: 25–33
Marcus FI, Fontaine GH, Guiraudon G et al (1982) Right ventricular dysplasia: A report of 24 adult cases. Circulation 65:384–398
Nava A, Thiene G, Canciani B et al (1988) Familial occurrence of right ventricular dysplasia: A study involving nine families. J Am Coll Cardiol 12:1222–1228
Thiene G, Nava A, Corrado D et al (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318:129–133
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Thiene, G., Nava, A., Marcus, F.I. (2007). Introduction: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Clarified. In: Markus, F.I., Nava, A., Thiene, G. (eds) Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer, Milano. https://doi.org/10.1007/978-88-470-0490-0_1
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DOI: https://doi.org/10.1007/978-88-470-0490-0_1
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