title: Management of patients with Arrhythmogenic Right Ventricular Cardiomyopathy in the Nordic countries.
abstract: OBJECTIVE
  Diagnostics of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are complex, and based on the 2010 Task Force document including different diagnostic modalities. However, recommendations for clinical management and follow-up of patients with ARVC and their relatives are sparse. This paper aims to give a practical overview of management strategies, risk stratification, and selection of appropriate therapies for patients with ARVC and their family members.
  
  
  METHODS
  This paper summarizes follow-up and treatment strategies in ARVC patients in the Nordic countries. The author group represents cardiologists who are actively involved in the Nordic ARVC Registry which was established in 2009, and contains prospectively collected clinical data from more than 590 ARVC patients from Denmark, Norway, Sweden, and Finland.
  
  
  RESULTS
  Different approaches of management and follow-up are required in patients with definite ARVC and in genetic-mutation-positive family members. Furthermore, ARVC patients with and without implantable cardioverter defibrillators (ICDs) require different follow-up strategies.
  
  
  CONCLUSIONS
  Careful follow-up is required in patients with ARVC diagnosis to evaluate the need of anti-arrhythmic therapy and ICD implantation. Mutation-positive family members should be followed regularly for detection of early disease and risk stratification of ventricular arrhythmias.
authors.affiliation: a Dept of Cardiology, Center for Cardiological Innovation, Institute for Surgical Research, Oslo University Hospital, Rikshospitalet, Oslo, Norway and University of Oslo , Oslo , Norway.
authors.forename: Kristina H
authors.initials: KH
authors.lastname: Haugaa
authors.affiliation: b Unit for Inherited Cardiac Diseases, The Heart Center, The National University Hospital , Rigshospitalet, Copenhagen, Denmark.
authors.forename: Henning
authors.initials: H
authors.lastname: Bundgaard
authors.affiliation: a Dept of Cardiology, Center for Cardiological Innovation, Institute for Surgical Research, Oslo University Hospital, Rikshospitalet, Oslo, Norway and University of Oslo , Oslo , Norway.
authors.forename: Thor
authors.initials: T
authors.lastname: Edvardsen
authors.affiliation: c Dept. of Cardiology, Aalborg University Hospital , Denmark.
authors.forename: Ole
authors.initials: O
authors.lastname: Eschen
authors.affiliation: d Institute of Medicine at Sahlgrenska Academy, University of Gothenburg , Gothenburg , Sweden.
authors.forename: Thomas
authors.initials: T
authors.lastname: Gilljam
authors.affiliation: e Dept. of Cardiology, Gentofte Hospital, University of Copenhagen , Kildegårdsvej 28, 2900 Hellerup, Denmark.
authors.forename: Jim
authors.initials: J
authors.lastname: Hansen
authors.affiliation: f Dept. of Cardiology, Aarhus University Hospital , Palle Juul-Jensens Boulevard 99, 8200 Aarhus N, Denmark.
authors.forename: Henrik Kjærulf
authors.initials: HK
authors.lastname: Jensen
authors.affiliation: g Dept. of Cardiology, Lund University and Arrhythmia Clinic, Skåne University Hospital , Lund , Sweden.
authors.forename: Pyotr G
authors.initials: PG
authors.lastname: Platonov
authors.affiliation: h Department of Cardiology and Department of Medical and Health Sciences , Linköping University , Linköping , Sweden.
authors.forename: Anneli
authors.initials: A
authors.lastname: Svensson
authors.affiliation: i Dept. of Cardiology, The Heart Centre, Rigshospitalet, University of Copenhagen, and Danish National Research Foundation Centre for Cardiac Arrhythmia (DARC) , Copenhagen , Denmark and Department of Clinical Medicine , Faculty of Health and Medical Sciences, University of Copenhagen , Copenhagen , Denmark.
authors.forename: Jesper H
authors.initials: JH
authors.lastname: Svendsen
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy*; D002317:Cardiovascular Agents / Q000009:adverse effects / Q000627:therapeutic use*; D017115:Catheter Ablation / Q000009:adverse effects; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D004554:Electric Countershock / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods; D020022:Genetic Predisposition to Disease; D006801:Humans; D010375:Pedigree; D010641:Phenotype; D011237:Predictive Value of Tests; D018570:Risk Assessment; D012307:Risk Factors; D012537:Scandinavian and Nordic Countries / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D002317:Cardiovascular Agents
keywords: Management of ARVC; Ventricular arrhythmias; arrhythmogenic right ventricular cardiomyopathy; mutation positive family members