Arrhythmogenic right ventricular dysplasia: Difference between revisions
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==Background== | ==Background== | ||
* | *Second most common cause of sudden cardiac death in young patients <ref>https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/</ref> | ||
** | **Up to 20% of sudden cardiac deaths in young people | ||
* | *Usually occurs in people of Greek or Italian descent | ||
* | *Male:Female = 3:1 | ||
*1:1000-10,000 in the US | *1:1000-10,000 in the US | ||
*Fibro-fatty replacement of myocardium | *Fibro-fatty replacement of right ventricular myocardium | ||
==Clinical Features== | ==Clinical Features== | ||
Revision as of 00:39, 20 March 2019
Background
- Second most common cause of sudden cardiac death in young patients [1]
- Up to 20% of sudden cardiac deaths in young people
- Usually occurs in people of Greek or Italian descent
- Male:Female = 3:1
- 1:1000-10,000 in the US
- Fibro-fatty replacement of right ventricular myocardium
Clinical Features
- Palpitations (27%)
- Syncope (26%)
- Ventricular dysrhythmia/cardiac arrest (23%)
- Family history of unexplained syncope or sudden death
- Dysrhythmias refractory to anti-dysrhythmic meds
- Asymptomatic (40%)
- Usually these patients are identified through genetic testing of an affected or symptomatic family member
- Right ventricular failure (6%)
- Dyspnea
- Atypical chest pain (6%)
Differential Diagnosis
Cardiomyopathy
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy
- Peripartum cardiomyopathy
- Takotsubo cardiomyopathy
- Arrhythmogenic right ventricular dysplasia
Syncope Causes
- Cardiovascular-mediated syncope
- Dysrhythmias:
- Cardiovascular disease
- Neurally mediated syncope
- Vasovagal:
- Fear, pain, emotion, valsalva, breath-holding spell
- Situational (associated with):
- Vasovagal:
- Orthostatic hypotension-mediated syncope:
- Volume depletion:
- Autonomic Dysreflexia
- Autonomic failure due to meds
- Other serious causes
- Stroke
- SAH
- TIA
- Vertebrobasilar Insufficiency
- Subclavian steal
- Heat syncope
- Hypoglycemia
- Hyperventilation
- Asphyxiation
- Seizure
- Narcolepsy
- Psychogenic (anxiety, conversion disorder, somatic symptom disorder)
- Toxic (drugs, carbon monoxide, etc.)
T Wave Inversions
- Normal in pediatrics
- Myocardial infarct (NSTEMI)
- Myocardial ischemia (Wellen's) - T waves go up, then down
- Hypokalemia - T waves go down, then up (or camel humped, one upright TW and upright U-wave in severe hypokalemia)
- Hyperkalemia
- Pulmonary embolism (RV strain)
- Pulmonary hypertension, acute or chronic
- Pulmonary disease - hyperventilation, pneumothorax, pneumonia
- LVH with strain pattern
- RVH
- Bundle branch block (both left and right)
- WPW
- Pericarditis (stage 3)
- CNS T waves (diffuse, deep)
- Arrhythmogenic right ventricular dysplasia (may also have epsilon wave)
- HOCM
- Paced rhythm
- Elevated intracranial pressure
Evaluation
ECG
- Epsilon wave (30-50%): positive deflection at the end of the QRS complex
- V1-V3 TWI (especially in patients >14 yrs old) (85%)
- V1-V3 QRS widening
- Sudden VT episodes with a LBBB morphology
- Prolonged S-wave upstroke of 55 ms in V1-3 (95%)
Imaging
Major and minor criteria rely on echo and cardiac MRI
- Echo - hypokinetic and dilated RV, dilation of RVOT
- Cardiac MRI - fibro-fatty change with RV myocardial thinning, RV aneurysms, RV dilatation
Management
- Sotalol is the preferred anti-dysrhythmic
- Manage heart failure in the usual manner
Disposition
- Symptomatic presentation: Admission to cardiology
- Incidental finding: Cardiology follow-up for further risk assessment and possible ICD placement or ablation
References
- Anderson EL. Arrhythmogenic right ventricular dysplasia. Am Fam Physician. 2006 Apr 15;73(8):1391-8.
- Perez Diez D, Brugada J. Diagnosis and Management of Arrhythmogenic Right Ventricular Dysplasia. E-Journal of the ESC Council for Cardiology Practice, European Society of Cardiology 2008.