title: Guidelines for the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy.
abstract: BACKGROUND
  Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon inherited myocardial disorder characterised by fibro-fatty inflammation affecting the right and left ventricles. It most commonly presents with palpitations or syncope but sudden death may occur, especially in young males.
  
  
  METHODS
  Diagnosis is not possible with a single test and may be difficult. Task Force criteria agreed in 1994 comprise major and minor criteria spanning structural abnormalities, ECG appearances, arrhythmias, family history of premature death and myocardial histology. Modified criteria were introduced in 2010 to improve sensitivity.
  
  
  RESULTS
  Arrhythmogenic right ventricular cardiomyopathy is a desmosomal disease. Mutations have been detected in five desmosomal genes, most frequently in plakophilin-2 (PKP2) and multiple mutations are also reported. Antiarrhythmic drugs such as sotalol and amiodarone may improve symptoms but are unproven to increase survival. An implantable defibrillator is appropriate in individuals surviving cardiac arrest or sustained ventricular tachycardia, but there is not yet consensus about prophylactic treatment of Task Force positive but asymptomatic individuals.
  
  
  CONCLUSIONS
  Arrhythmogenic right ventricular cardiomyopathy is more common than previously believed. Preliminary evidence supports improved sensitivity without loss of specificity using the revised Task Force criteria. The genetics of the disease are complex but should ultimately advance diagnosis and management.
authors: Green Lane Cardiovascular Service, Cardiology Department, Auckland City Hospital, Private Bag 92024, Auckland, New Zealand. warrens@adhb.govt.nz
authors: Warren
authors: W
authors: Smith
mesh_terms: D000638:Amiodarone / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D017147:Defibrillators, Implantable; D003896:Desmosomes / Q000235:genetics; D004562:Electrocardiography / Q000379:methods; D005260:Female; D030342:Genetic Diseases, Inborn / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D006323:Heart Arrest / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D006801:Humans; D008297:Male; D009154:Mutation; D010375:Pedigree; D051191:Plakophilins / Q000235:genetics; D017410:Practice Guidelines as Topic; D012737:Sex Factors; D013015:Sotalol / Q000627:therapeutic use; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000235:genetics / Q000628:therapy
chemical_list: D000889:Anti-Arrhythmia Agents; C495337:PKP2 protein, human; D051191:Plakophilins; D013015:Sotalol; D000638:Amiodarone