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Arrhythmogenic right ventricular cardiomyopathy: a focused update on diagnosis and risk stratification
  1. Laurens P Bosman1,2,
  2. Anneline S J M te Riele1,2
  1. 1 Cardiology, UMC Utrecht, Utrecht, The Netherlands
  2. 2 ICIN-Netherlands Heart Institute, Utrecht, The Netherlands
  1. Correspondence to Dr Anneline S J M te Riele, Cardiology, UMC Utrecht, Utrecht, The Netherlands; ariele{at}


Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by fibrofatty replacement of predominantly the right ventricle and high risk of ventricular arrhythmias and sudden cardiac death (SCD). Early diagnosis and accurate risk assessment are challenging yet essential for SCD prevention. This manuscript summarises the current state of the art on ARVC diagnosis and risk stratification. Improving the 2010 diagnostic criteria is an ongoing discussion. Several studies suggest that early diagnosis may be facilitated by including deformation imaging (‘strain’) for objective assessment of wall motion abnormalities, which was shown to have high sensitivity for preclinical disease. Adding fibrofatty replacement detected by late gadolinium enhancement or T1 mapping in cardiac MRI as criterion for diagnosis is increasingly suggested but requires more supporting evidence from consecutive patient cohorts. In addition to the traditional right-dominant ARVC, standard criteria for arrhythmogenic cardiomyopathy (ACM) and arrhythmogenic left ventricular cardiomyopathy (ALVC) are on the horizon. After diagnosis confirmation, the primary management goal is SCD prevention, for which an implantable cardioverter-defibrillator is the only proven therapy. Prior studies determined that younger age, male sex, previous (non-) sustained ventricular tachycardia, syncope, extent of T-wave inversion, frequent premature ectopic beats and lower biventricular ejection fraction are risk factors for subsequent events. Previous implantable cardioverter-defibrillator indication guidelines were however limited to three expert-opinion flow charts stratifying patients in risk groups. Now, two multivariable risk prediction models ( combine the abovementioned risk factors to estimate individual risks. Of note, both the flow charts and prediction models require clinical validation studies to determine which should be recommended.

  • arrhythmogenic right ventricular dysplasia
  • risk factors
  • diagnostic imaging
  • defibrillators
  • implantable
  • genetic diseases
  • inborn

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  • Contributors LPB and ASJMtR contributed equally.

  • Funding LPB is supported by a Netherlands Heart Institute Fellowship 2020. The Dutch ACM Registry is supported by the Dutch Heart Foundation (CVON eDETECT 2015–12, CVON2018- 30 PREDICT2 and a personal grant to ASJMtR (grant number 2015T058).

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Not commissioned; internally peer reviewed.