Arrhythmogenic right ventricular dysplasia

Revision as of 17:39, 11 March 2021 by Rossdonaldson1 (talk | contribs) (→‎Imaging)
(diff) ← Older revision | Latest revision (diff) | Newer revision → (diff)


In vitro MRI and corresponding cross section of the heart in ARVD show RV dilatation with anterior and posterior aneurysms (17-year-old asymptomatic male athlete who died suddenly during a soccer game).
  • Second most common cause of sudden cardiac death in young patients [1]
    • Up to 20% of sudden cardiac deaths in young people
  • Usually occurs in people of Greek or Italian descent
  • Male:Female = 3:1
  • 1:1000-10,000 in the US
  • Fibro-fatty replacement of right ventricular myocardium
  • Two forms:
    • Autosomal dominant
      • Variable penetrance
    • Autosomal recessive
      • Naxos disease (Palmoplantar keratoderma) [2]

Clinical Features

Differential Diagnosis


Syncope Causes

T Wave Inversions



Epsilon Wave in Lead V1
  • Epsilon wave
    • Seen in 30-50% of cases
    • Most specific finding
    • Small positive deflection at the end of the QRS complex
  • V1-V3 T wave inversions
    • Seen in 85% of cases
    • Especially in patients >14 yrs old)
  • Localised QRS widening
    • 110 ms in V1-V3
  • Sudden VT episodes with LBBB morphology
  • Prolonged S-wave upstroke
    • 55 ms in V1-3
    • Seen in 95% of cases


MRI in a patient affected by ARVC/D (long axis view of the right ventricle): note the transmural diffuse bright signal in the RV free wall on spin echo T1 (a) due to massive myocardial atrophy with fatty replacement (b).

Major and minor criteria rely on echo and cardiac MRI

  • Echocardiography
    • Hypokinetic and dilated right ventricle
    • Dilation of RVOT
  • Cardiac MRI [3]
    • Fibro-fatty infiltration
    • Thinning of right ventricular myocardium
    • RV aneurysm
    • RV dilatation
    • Regional wall motion abnormalities
    • Global systolic dysfunction
  • Histological studies provide post-mortem diagnosis
    • May prompt testing in family members



  • Admission under cardiology if symptomatic
  • Admission under cardiology if high-risk features[4]:
    • Syncope due to cardiac arrest
    • Recurrent dysrhythmias not suppressed by drug therapy
    • Cardiac arrest in first-degree relative
  • Incidental finding: Cardiology follow-up for further risk assessment and possible ICD placement or ablation


  2. Protonotarios, N., and Tsatsopoulou, A. (2006). Naxos disease: Cardiocutaneous syndrome due to cell adhesion defect. Orphanet Journal of Rare Diseases, 1(4).
  • Anderson EL. Arrhythmogenic right ventricular dysplasia. Am Fam Physician. 2006 Apr 15;73(8):1391-8.
  • Perez Diez D, Brugada J. Diagnosis and Management of Arrhythmogenic Right Ventricular Dysplasia. E-Journal of the ESC Council for Cardiology Practice, European Society of Cardiology 2008.