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Arrhythmogenic Right Ventricular DysplasiaAka: Arrhythmogenic Right Ventricular Cardiomyopathy, Right Ventricular Dysplasia, ARVD
- See Also
- Epidemiology
- More common in Mediterranean descent
- Accounts for 5% of Sudden Cardiac Death in under age 65
- Gender predominance: Male by 3:1 margin
- Age range (typical): 20 to 47 years old
- Pathophysiology
- Inherited cardiomyopathy with variable genetics
- Right ventricular wall infiltrated with fat
- Localized to triangle of dysplasia
- Superior landmark: Anterior infundibulum
- Inferior right landmark: Inferior right ventricle
- Inferior left landmark: Right ventricular apex
- Associated with Ventricular Tachycardia predisposition
- Symptoms
- Palpitations
- Fatigue
- Decreased Exercise tolerance
- Dyspnea on exertion
- Presentations
- May present as sudden death in young athletes
- Syncope
- Refractory rapid tachycardia
- Cardiac arrest
- Diagnostics: Specific findings in ARVD
- Electrocardiogram (changes present in leads V1-V3)
- T Wave inversion in V1-V3 (not with right BBB)
- Epsilon Waves
- Ventricular Tachycardia with Left Bundle Branch Block
- Echocardiogram
- Right ventricular myocardium with fatty infiltration
- Secondary right ventricular wall motion abnormality
- Dilatation and reduced right ventricular EF
- Localized aneurysms or segmental dilatation
- Electrophysiologic studies
- Endomyocardial biopsy
- Myocardium replaced by fibrofatty tissue
- Gold standard for diagnosis
- Test Sensitivity: 20%
- Test Specificity: 92%
- Electrocardiogram (changes present in leads V1-V3)
- Diagnostics: Other testing to differentiate other causes
- Holter Monitor
- Exercise Stress Test
- MR Angiogram or CT Angiogram
- Management: General
- Avoid physical exertion until evaluation completed
- Cardiology or electrophysiology consultation
- 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
- Management: Prevent recurrent Ventricular Tachycardia
- 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
- References
Arrhythmogenic Right Ventricular Dysplasia (C0349788) | |
|---|---|
| Definition (MSH) | A congenital cardiomyopathy that is characterized by infiltration of adipose and fibrous tissue into the RIGHT VENTRICLE wall and loss of myocardial cells. Primary injuries usually are at the free wall of right ventricular and right ATRIA resulting in ventricular and supraventricular arrhythmias. |
| Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
| English | Arrhythmogenic Right Ventricular Cardiomyopathy, Arrhythmogenic Right Ventricular Dysplasia, Arrythmogenic right ventricular dysplasia, ARVD |
| Spanish | displasia arritmogenica del ventriculo derecho, displasia ventricular derecha arritmogenica, miocardiopatia ventricular derecha arritmogenica |
| Parent Concepts | Congenital Heart Defects (C0018798), Cardiomyopathies (C0878544), Tachycardia, Ventricular (C0042514), Congenital heart disease (C0152021), Right ventricular abnormality (C0344887), Disorder of right cardiac ventricle (C1562299) |
| Sources | MSH, NDFRT, OMIM, SCTSPA, SNOMEDCT Derived from the NIH UMLS (Unified Medical Language System) |
