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Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic Right Ventricular Dysplasia (Cardiomyopathy)

Stages of ARVD

• Stage 1 (asymptomatic)
• The patient is asymptomatic, with no arrhythmias or structural changes in the heart
• There are minimal changes on the ECG and imaging studies (MRI, echocardiography)
• At this stage, ectopic foci and ventricular tachycardia may already develop in the ventricle
• Stage 2 (symptomatic)
• The patient has structural changes (fibrotic and fatty) in the right ventricle
• There are recurrent ventricular extrasystoles and tachycardias
• It appears in young people < 35 years old
• Stage 3 (heart failure)
• ARVD causes dilation of the right ventricle (dilated cardiomyopathy) and subsequently heart failure
  
  
• In every stage, ventricular tachycardia and sudden cardiac death can occur

Idiopathic Ventricular Tachycardia

• Idiopathic Ventricular Tachycardia
• Occurs in a structurally normal heart (without electrolyte disturbances or QT interval changes)
• 10% of VT cases are idiopathic
• 90% of VT arises in a structurally abnormal heart
• In idiopathic ventricular tachycardia, an ectopic focus originates in the area of:
• Ventricular outflow tracts
• Fascicles of the left bundle branch
• We recognize 3 types of idiopathic ventricular tachycardia
• Ventricular Tachycardia from the Right Ventricular Outflow Tract
• Ventricular Tachycardia from the Left Ventricular Outflow Tract
• Fascicular Ventricular Tachycardia
• ECG characteristics of VT are similar in:
• Idiopathic VT from the right ventricular outflow tract
• VT from the right ventricular outflow tract in ARVD
  
  
• Idiopathic VT from the right ventricular outflow tract
• The heart is not structurally changed
• Mechanism is triggered activity
• Tachycardia responds to adenosine
• No changes are seen on ECG during sinus rhythm
  
  
• VT from the right ventricular outflow tract in ARVD
• The heart is structurally changed
• Mechanism is re-entry
• Tachycardia does not respond to adenosine
• Changes are seen on ECG during sinus rhythm

ECG and Epsilon Wave (ε)

• Epsilon wave is a small positive deflection at the end of the QRS complex.
• It is observed in leads that "view" the right ventricle (V1-V3)
• The epsilon wave is present in 30% of patients with ARVD

T Wave Inversion

• 85% of patients with ADPK have negative T waves (V1-V3)

Arrhythmogenic Right Ventricular Dysplasia

• Epsilon wave in all leads
• Negative T waves (V1-V3), also V4-6
• Hypertrophy and overload of the right ventricle
• R/S ratio (V1) ≥ 1
• Descending ST segment and Negative T waves (V1-4) (II, III, aVF)

Arrhythmogenic Right Ventricular Dysplasia

• On the ECG, you can see 6 QRS complexes (from leads V1-V3)
• Each QRS complex shows prolonged S wave upstroke > 55ms (> 1.5 squares)
• (one square is 40ms)

Arrhythmogenic Right Ventricular Dysplasia

• You see 6 QRS complexes on the ECG (from leads V1-V3)
• Each QRS complex has a prolonged S-wave upstroke > 55ms (> 1.5 small squares)
• Each QRS complex is >110ms (>2.5 small squares)

Ventricular Tachycardia from the Right Ventricular Outflow Tract

• Wide QRS complexes (>0.12s)
• Heart rate 160/min.
• Transition zone (V3)
• The location of the transition zone distinguishes VT from the right and left outflow tract
• Appearance of left bundle branch block
• Deep S (V1)
• Dominant R (V6)
• Wide QRS complexes
• Vertical axis (+ 90°)
• Positive QRS (II, III, aVF)
• AV dissociation
• P waves are visible in continuous lead II, independent of QRS complexes
• AV dissociation is a fundamental feature of ventricular tachycardia
  
  
• It could be idiopathic VT from the right ventricular outflow tract
• However, the patient had ECG signs of ARVD during sinus rhythm
• The ECG shows VT from the outflow tract with ARVD, not idiopathic VT from the right ventricular outflow tract