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Arrhythmogenic Right Ventricular Cardiomyopathyin Athletes: Diagnosis, Management,and Recommendations for Sport ActivityCristina Basso, MD, PhD a , Domenico Corrado, MD, PhD b ,Gaetano Thiene, MD, FRCP Hon a, *a Department of Medical-Diagnostic Sciences and Special Therapies, University of Padua Medical School,Via A. Gabelli 61, 35121 Padova, Italyb Department of Cardio-Thoracic and Vascular Sciences, University of Padua Medical School,Via N. Giustiniani 2, 35128 Padova, ItalyArrhythmogenic right ventricular cardio-myopathy/dysplasia (ARVC/D) is an inheritedheart muscle disease that predominantly af f ectsthe right ventricle and is characterized pathologi-cally by progressive replacement of right ven-tricular myocardium with f i brofatty tissue [1–10].Clinically, the disease presents with myocardialelectrical instability leading to ventricular tachy-cardia or ventricular f i brillation which may precip-itate cardiac arrest, particularly during physicalexercise [11].This article examines the role of ARVC/D incausingsuddendeathinyoungcompetitiveathletesand suggests a prevention strategy based on iden-tif i cation of af f ected athletes at preparticipationscreening. Systematic cardiovascular screening(including 12-lead ECG) of all subjects embarkingin sports activity has the potential to identify thoseathletes at risk and to reduce mortality [12,13].The advent of molecular genetics has providednew insights in understanding the etiopathogenesisof ARVC/D, showing that it is a desmosomaldisease resulting from defective cell adhesionproteins such as desmoplakin, plakoglobin, plako-philin-2, desmoglein-2, and desmocollin-2 [14–18].It has been postulated that the lack of the proteinortheincorporationofmutantproteinintocardiacdesmosomesmayprovokeremodelingwithdetach-ment of myocytes at the intercalated disks, parti-cularly under the condition of mechanical stressduring training and competitive sports activity[19,20]. As a consequence, there is progressivemyocytedeathwithsubsequentrepairbyf i brofattyreplacement. Life-threatening ventricular arrhy-thmias such as ventricular tachycardia and f i brilla-tion may occur during the ‘‘hot phase’’ of myocytedeath or later in the form of a scar-related macro-reentrant mechanism [21].Arrhythmogenic right ventricularcardiomyopathy/dysplasia: a major causeof sudden death in young athletesSystematic monitoring and pathologic investi-gationofsuddendeathinyoungpeopleandathletesof the Veneto region of Italy has shown thatARVC/D is the most common pathologic sub-strate, accounting for nearly one fourth of fataleventsontheathletic f i eld[11,12,22].Theincidenceof sudden death from ARVC/D in athletes is esti-mated to be 0.5 cases per 100,000 persons per year(Fig. 1). In the authors’ experience, sudden deathvictims with ARVC/D have all been male witha mean age of 22.6 ? 4 years [22].This work was supported by the European Commis-sion ARVC/D Project, Brussels, Veneto Region,Venice, and Telethon, Rome; Cariparo Foundation,Pedova; Ministry of Health, Rome.* Corresponding author.E-mail address: gaetano.thiene@unipd.it(G. Thiene).0733-8651/07/$ - see front matter ? 2007 Elsevier Inc. All rights reserved.doi:10.1016/j.ccl.2007.08.009 cardiology.theclinics.comCardiol Clin 25 (2007) 415–422