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Arrhythmogenic right ventricular dysplasia

Last edited: 4/15/2026

Overview

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium, leading to ventricular arrhythmias and potential sudden cardiac death 1.

Diagnosis

  • Major Criteria:
  • - Ventricular tachycardia with right precordial ST-segment depression or T-wave inversion - Structural right ventricular abnormality on imaging (e.g., wall thinning, aneurysm) - Histopathological evidence of myocyte dropout and fatty replacement
  • Minor Criteria:
  • - Family history of ARVD or sudden cardiac death - Right ventricular dysfunction on echocardiography or MRI - Conduction defects (e.g., bundle branch block, right axis deviation)
  • Grading System: Use Task Force Criteria for diagnosis, typically requiring ≥3 major or ≥2 major + 2 minor criteria 1
  • Management

  • First-Line Treatments:
  • - Implantable cardioverter-defibrillator (ICD) for primary prevention in high-risk patients 1
  • Adjunctive Treatments:
  • - Antiarrhythmic drugs (e.g., sotalol, amiodarone) for symptomatic arrhythmias 1 - Beta-blockers to reduce arrhythmic triggers 1

    Special Populations

  • Pregnancy: Management focuses on risk stratification and close monitoring with ICD precautions; specific guidelines are limited 1
  • Pediatrics: Early diagnosis and close follow-up are crucial; ICD implantation may be considered in severe cases 1
  • Elderly: Tailored risk stratification and management similar to younger adults, with consideration for comorbidities 1
  • Comorbidities: Management strategies adjusted based on coexisting heart conditions; no specific guidelines provided in abstracts 1
  • Key Recommendations

  • Use Task Force Criteria for diagnosing ARVD (Evidence: Strong 1)
  • Implant ICDs in patients meeting high-risk criteria for primary prevention of sudden death (Evidence: Strong 1)
  • Consider beta-blockers as part of the management to reduce arrhythmic triggers (Evidence: Moderate 1)
  • References

    1 Berry D. Guy Fontaine and arrhythmogenic right ventricular dysplasia. European heart journal 2011. link

    Original source

    1. [1]
      Guy Fontaine and arrhythmogenic right ventricular dysplasia.Berry D European heart journal (2011)

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