Arrhythmogenic Right Ventricular Dysplasia

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EKG

EKG of a patient with ARVD. The Epsilon wave is marked with an arrow.

Characteristics

Arrhythmogenic Right Ventricular Dysplasia, also known as arrhythmogenic right ventricular cardiomyopathy, is characterized by replacement of the right ventricular muscle by fatty and fibrous tissue. Patients usually present with arrhythmias of right ventricular origin that range from isolated premature ventricular beats to nonsustained or sustained VT and ventricular fibrillation. Other clinical manifestations include global or regional right ventricular dysfunction, ECG changes, and late evolution to right or biventricular heart failure. Tachycardia is due to reentry and has a LBBB morphology

EKG Characteristics

• Incomplete or complete RBBB
• Inverted T waves in the anterior precordial leads
• Localized prolongation of the QRS complex in leads V1 and V2
• Epsilon waves visible as sharp discrete deflections at the terminal portion of the QRS complex in the anterior precordial leads
• Use QRS width in Lead I which is always <120ms
• Lead III R>S
• S wave upstroke in V1 - V3 >55ms was found in 95 percent of ARVD

Imaging Characteristics

• Abnormal Signal Averaged ECG which is present in 50-80% of cases.
• Echo shows Right Ventricular dysfunction
• Workup with MRI

Criteria for Diagnosis

Family history

• Major
  • Familial disease confirmed at necropsy or surgery
• Minor
  • Family history of premature sudden death (<35 years of age) due to suspected ARVD
  • Family history {clinical diagnosis based on present criteria)

ECG depolarization/conduction abnormalities

• Major
  • Epsilon waves or localized prolongation (>110 ms) of QRS complex in right precordial leads (V1-V3)
• Minor
  • Late potentials on signal-averaged ECG

ECG repolarization abnormalities

• Minor
  • Inverted T waves in right precordial leads (V2 and V3) in people >12 years of age and in absence of right bundle branch block

Arrhythmias

• Minor
  • Sustained or nonsustaincd left bundle branch block-type VT documented on ECG, Holter monitoring or during exercise testing
  • Frequent ventricutar extrasystoles (> 1000/24 h on Holter monitoring}

Global or regional dysfunction and structural alterations

• Major
  • Severe dilatation and reduction ol RV ejection fraction with no or mild LV involvement
  • Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulgings)
  • Severe segmental dilatation of RV
• Minor
  • Mild global RV dilatation or ejection fraction reduction with normal LV
  • Mild segmental dilatation of RV
  • regional RV hypokincsia

Tissue characteristics of walls

• Major
  • Fibrofatty replacement of myocardium on endomyocardial biopsy

References

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 71:215, 1994