Arrhythmogenic Right Ventricular Dysplasia

Aka: Arrhythmogenic Right Ventricular Dysplasia, Arrhythmogenic Right Ventricular Cardiomyopathy, Right Ventricular Dysplasia, ARVD

1. See Also
   1. Sudden Cardiac Death
   2. Sudden Death in Athletes
   3. Cardiomyopathy
2. Epidemiology
   1. More common in Mediterranean descent
   2. Accounts for 5% of Sudden Cardiac Death in under age 65
   3. Gender predominance: Male by 3:1 margin
   4. Age range (typical): 20 to 47 years old
3. Pathophysiology
   1. Rare inherited Cardiomyopathy with variable genetics
   2. Right ventricular wall infiltrated with fat and fibrous tissue
      1. Localized to triangle of dysplasia
      2. Superior landmark: Anterior infundibulum
      3. Inferior right landmark: Inferior right ventricle
      4. Inferior left landmark: Right ventricular apex
   3. Associated with Ventricular Tachycardia predisposition
4. Symptoms
   1. Palpitations
   2. Fatigue
   3. Decreased Exercise tolerance
   4. Dyspnea on exertion
5. Signs: Cutaneous manifestations in genetic syndromes
   1. Naxos Disease: Wooly hair and palmoplantar keratoderma
6. Presentations
   1. May present as sudden death in young athletes
   2. Syncope
   3. Refractory rapid Tachycardia
   4. Cardiac Arrest
7. Diagnostics: Specific findings in ARVD
   1. Electrocardiogram (changes present in leads V1-V3)
      1. T Wave inversion in V1-V3 (not with right BBB)
      2. Epsilon Waves (small amplitude potentials at end of QRS Complex)
      3. Ventricular Tachycardia with Left Bundle Branch Block
   2. Echocardiogram
      1. Right ventricular Myocardium with fatty infiltration
      2. Secondary right ventricular wall motion abnormality
      3. Dilatation and reduced right ventricular EF
      4. Localized aneurysms or segmental dilatation
   3. Electrophysiologic studies
   4. Endomyocardial biopsy
      1. Myocardium replaced by fibrofatty tissue
      2. Gold standard for diagnosis
      3. Test Sensitivity: 20%
      4. Test Specificity: 92%
8. Diagnostics: Other testing to differentiate other causes
   1. Holter Monitor
   2. Exercise Stress Test
   3. MR Angiogram or CT Angiogram
9. Management: General
   1. Avoid physical exertion until evaluation completed
   2. Cardiology or electrophysiology consultation
10. Management: Acute termination of Ventricular Tachycardia
    1. See Ventricular Tachycardia
    2. Medications (no single drug is universally effective)
       1. First-line agents
          1. Amiodarone
          2. Sotalol
       2. Other agents if refractory to above
          1. Beta Blockers
          2. Procainamide
          3. Flecainide
          4. Propafenone
11. Management: Prevent recurrent Ventricular Tachycardia
    1. Medications
       1. Amiodarone
       2. Sotalol
       3. Beta Blockers
       4. Propafenone
    2. Radiofrequency ablation indications
       1. Medication refractory Ventricular Tachycardia
       2. Frequent Ventricular Tachycardia post-AICD placement
       3. Localized source of arrhythmia
    3. Implantable Defibrillator (AICD) indications
       1. Cardiac Arrest
       2. Ventricular Tachycardia episodes refractory to above
       3. Age <35 years
       4. Left ventricle involved
    4. Surgery in refractory cases
       1. Right ventriculotomy
       2. Heart Transplantation
12. References
    1. Anderson (2006) Am Fam Physician 73:1391-1401
    2. Fontaine (1998) Circulation 97:1532-5
    3. Gear (2003) Circulation 107:e31-3