II. Epidemiology
- More common in Mediterranean descent
- Prevalence: 1 in 1,000 to 5000
- Accounts for 5% of Sudden Cardiac Death in under age 65
- Gender predominance: Male by 3:1 margin
- Age range (typically 4th decade of life): 20 to 47 years old
- Familial in 50% of cases
III. Pathophysiology
- Rare inherited Cardiomyopathy with variable Genetics
- Common effect is on desmosomal Proteins
- Right ventricular wall infiltrated with fat and fibrous tissue (fibrofatty infiltration)
- Localized to triangle of dysplasia
- Superior landmark: Anterior infundibulum
- Inferior right landmark: Inferior right ventricle
- Inferior left landmark: Right ventricular apex
- Results in thinning and ballooning of the right ventricular wall
- Localized to triangle of dysplasia
- Associated with Ventricular Tachycardia predisposition
IV. Symptoms
- Palpitations
- Fatigue
- Decreased Exercise tolerance
- Dyspnea on exertion
V. Signs: Cutaneous manifestations in Genetic Syndromes
- Naxos Disease: Wooly hair and palmoplantar keratoderma
VI. Presentations
- May present as sudden death in young athletes
- Syncope
- Refractory rapid Tachycardia
- Sudden Cardiac Death (Cardiac Arrest)
VII. Diagnostics: Specific findings in ARVD
-
Electrocardiogram (changes present in leads V1-V3)
- Right precordial, septal T Wave Inversion in V1-V3 (not with right BBB)
- QRS Complex duration > 110 ms in leads V1-V3
- Epsilon Waves (small amplitude notch at end of QRS Complex)
- Right Bundle Branch Block
-
Echocardiogram
- Right ventricular Myocardium with fatty infiltration
- Secondary right ventricular wall motion abnormality
- Dilatation and reduced right ventricular ejection Fracture
- Localized aneurysms or segmental dilatation
- Electrophysiologic studies
- Endomyocardial biopsy
- Myocardium replaced by fibrofatty tissue
- Gold standard for diagnosis
- Test Sensitivity: 20%
- Test Specificity: 92%
VIII. Diagnostics: Other testing to differentiate other causes
- Holter Monitor
- Exercise Stress Test
- MR Angiogram or CT Angiogram
IX. Management: General
- Avoid physical exertion until evaluation completed
- Cardiology or electrophysiology Consultation
X. Management: Acute termination of Ventricular Tachycardia
- See Ventricular Tachycardia
- Medications (no single drug is universally effective)
- First-line agents
- Other agents if refractory to above
XI. Management: Prevent recurrent Ventricular Tachycardia
- Therapy goals
- Decrease Arrhythmia
- Prevent Sudden Cardiac Death
- Medications
- Radiofrequency ablation indications
- Medication refractory Ventricular Tachycardia
- Frequent Ventricular Tachycardia post-AICD placement
- Localized source of Arrhythmia
-
Implantable Defibrillator (AICD) indications
- Cardiac Arrest
- Ventricular Tachycardia episodes refractory to above
- Age <35 years
- Left ventricle involved
- Surgery in refractory cases
- Right ventriculotomy
- Heart Transplantation