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Arrhythmogenic Right Ventricular Dysplasia

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Arrhythmogenic Right Ventricular Dysplasia, Arrhythmogenic Right Ventricular Cardiomyopathy, Right Ventricular Dysplasia, ARVD

  • See Also
  1. Sudden Cardiac Death
  2. Sudden Death in Athletes
  3. Cardiomyopathy
  • Epidemiology
  1. More common in Mediterranean descent
  2. Prevalence: 1 in 1,000 to 5000
  3. Accounts for 5% of Sudden Cardiac Death in under age 65
  4. Gender predominance: Male by 3:1 margin
  5. Age range (typically 4th decade of life): 20 to 47 years old
  6. Familial in 50% of cases
  • Pathophysiology
  1. Rare inherited Cardiomyopathy with variable Genetics
    1. Common effect is on desmosomal Proteins
  2. Right ventricular wall infiltrated with fat and fibrous tissue (fibrofatty infiltration)
    1. Localized to triangle of dysplasia
      1. Superior landmark: Anterior infundibulum
      2. Inferior right landmark: Inferior right ventricle
      3. Inferior left landmark: Right ventricular apex
    2. Results in thinning and ballooning of the right ventricular wall
  3. Associated with Ventricular Tachycardia predisposition
  • Symptoms
  1. Palpitations
  2. Fatigue
  3. Decreased Exercise tolerance
  4. Dyspnea on exertion
  1. Naxos Disease: Wooly hair and palmoplantar keratoderma
  • Presentations
  1. May present as sudden death in young athletes
  2. Syncope
  3. Refractory rapid Tachycardia
  4. Sudden Cardiac Death (Cardiac Arrest)
  • Diagnostics
  • Specific findings in ARVD
  1. Electrocardiogram (changes present in leads V1-V3)
    1. Right precordial, septal T Wave Inversion in V1-V3 (not with right BBB)
    2. QRS Complex duration > 110 ms in leads V1-V3
    3. Epsilon Waves (small amplitude notch at end of QRS Complex)
    4. Right 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 ejection Fracture
    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%
  • Diagnostics
  • Other testing to differentiate other causes
  1. Holter Monitor
  2. Exercise Stress Test
  3. MR Angiogram or CT Angiogram
  • Management
  • General
  1. Avoid physical exertion until evaluation completed
  2. Cardiology or electrophysiology Consultation
  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
  1. Therapy goals
    1. Decrease Arrhythmia
    2. Prevent Sudden Cardiac Death
  2. Medications
    1. Amiodarone
    2. Sotalol
    3. Beta Blockers
    4. Propafenone
  3. Radiofrequency ablation indications
    1. Medication refractory Ventricular Tachycardia
    2. Frequent Ventricular Tachycardia post-AICD placement
    3. Localized source of Arrhythmia
  4. Implantable Defibrillator (AICD) indications
    1. Cardiac Arrest
    2. Ventricular Tachycardia episodes refractory to above
    3. Age <35 years
    4. Left ventricle involved
  5. Surgery in refractory cases
    1. Right ventriculotomy
    2. Heart Transplantation
  • Complications
  1. Sudden Cardiac Death
  • References
  1. Brieler (2017) Am Fam Physician 96(10):640-6 [PubMed]
  2. Anderson (2006) Am Fam Physician 73:1391-1401 [PubMed]
  3. Fontaine (1998) Circulation 97:1532-5 [PubMed]
  4. Gear (2003) Circulation 107:e31-3 [PubMed]