Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

April 29, 2010 by  
Filed under Acute Med, All Updates, Resus

This disease may result in sudden cardiac death in young people, and the assessment of patients who present with dysrhythmias or syncope should prompt a review of the ECG for suggestive features of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (as well as ischaemia, conduction deficits, WPW syndrome, Brugada syndrome, and prolonged QT interval).

A Task force has revised its diagnostic criteria for the disease, listed as major and minor criteria pertaining to family history, ECG, echo, MRI, and angiographic features. The ECG features that front line doctors need to be on the look out for include:

  • Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individual >14 years of age (in the absence of complete right bundle-branch block QR>120 ms)
  • Inverted T waves in leads V1 and V2 in individual>14 years of age (in the absence of complete right bundle-branch block) or in V4, V5, or V6
  • Inverted T waves in leads V1, V2, V3, and V4 in individual>14 years of age in the presence of complete right bundle-branch block
  • Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 to V3)

Epsilon waves

  • Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL)
  • Nonsustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis
  • >500 ventricular extrasystoles per 24 hours (Holter)

Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Proposed Modification of the Task Force Criteria
Circulation. 2010 Apr 6;121(13):1533-41

More info on this disease from the European Society of Cardiology here

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