Arrhythmogenic right ventricular dysplasia: Difference between revisions

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==Background==
==Background==
*ARVD is a significant contributor to sudden cardiac death in young patients
*Second most common cause of sudden cardiac death in young patients <ref>https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/</ref>
*More common in males and those of Mediterranean descent
**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
*Two forms:
**Autosomal dominant
***Variable penetrance
**Autosomal recessive
***Naxos disease (Palmoplantar keratoderma) <ref>Protonotarios, N., and Tsatsopoulou, A. (2006). Naxos disease: Cardiocutaneous syndrome due to cell adhesion defect. Orphanet Journal of Rare Diseases, 1(4).</ref>


==Clinical Features==
==Clinical Features==
*Syncope
*[[Palpitations]] (27%)
*Ventricular dysrhythmia/cardiac arrest
*[[Syncope]] (26%)
*[[Ventricular dysrhythmias]]/[[cardiac arrest]] (23%)
**Especially occurring during exercise
*Family history of unexplained syncope or sudden death
*Family history of unexplained syncope or sudden death
*Dysrhythmias refractory to anti-dysrhythmic meds
*[[Dysrhythmias]] refractory to antidysrhythmic meds
*Asymptomatic (40%)
**Usually these patients are identified through genetic testing of an affected or symptomatic family member
*[[CHF|Right ventricular failure]] (6%)
*[[Dilated cardiomyopathy]]
*[[Dyspnea]]
*Atypical [[chest pain]] (6%)


==Differential Diagnosis==
==Differential Diagnosis==
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{{T-wave inversion DDX}}
{{T-wave inversion DDX}}


==Diagnosis==
==Evaluation==
===ECG===
===[[ECG]]===
[[File:ARVD.png|250px]]
[[File:ARVD.png|250px]]
*Epsilon wave, 30-50%
*Epsilon wave
*V1-V3 TWI (especially in pts >14 yrs old), 85%
**Seen in 30-50% of cases
*V1-V3 QRS widening
**Most specific finding
*Sudden VT episodes with a LBBB morphology
**Small positive deflection at the end of the QRS complex
*V1-V3 T wave inversions
**Seen in 85% of cases
**Especially in patients >14 yrs old)
*Localised QRS widening
**110 ms in V1-V3
*Sudden VT episodes with LBBB morphology
*Prolonged S-wave upstroke
**55 ms in V1-3
**Seen in 95% of cases
 
===Imaging===
===Imaging===
Major and minor criteria rely on echo and cardiac MRI
Major and minor criteria rely on echo and cardiac MRI
*Echo - hypokinetic and dilated RV, dilation of RVOT
*[[Echocardiography]]
*Cardiac MRI - fibro-fatty change with RV myocardial thinning, RV aneurysms, RV dilatation
**Hypokinetic and dilated right ventricle
**Dilation of RVOT
*Cardiac MRI <ref>https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/</ref>
**Fibro-fatty infiltration
**Thinning of right ventricular myocardium
**RV aneurysm
**RV dilatation
**Regional wall motion abnormalities
**Global systolic dysfunction
*Histological studies provide post-mortem diagnosis
**May prompt testing in family members


==Management==
==Management==
*Sotalol is the preferred anti-dysrhythmic
*[[Beta blockers]] or [[amiodarone]] to suppress ventricular dysrhythmias
*Manage heart failure in the usual manner
**[[Sotalol]] is preferred
*ICD implantation if high-risk features
*Ablation of conduction pathways
*Treat [[heart failure]] with [[diuretics]], [[ACE inhibitors]]
*Heart transplant


==Disposition==
==Disposition==
*Symptomatic presentation: Admission to cardiology
*Admission under cardiology if symptomatic
*Admission under cardiology if high-risk features<ref>https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/</ref>:
**Syncope due to cardiac arrest
**Recurrent dysrhythmias not suppressed by drug therapy
**Cardiac arrest in first-degree relative
*Incidental finding: Cardiology follow-up for further risk assessment and possible ICD placement or ablation
*Incidental finding: Cardiology follow-up for further risk assessment and possible ICD placement or ablation


Revision as of 14:42, 31 August 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
  • Two forms:
    • Autosomal dominant
      • Variable penetrance
    • Autosomal recessive
      • Naxos disease (Palmoplantar keratoderma) [2]

Clinical Features

Differential Diagnosis

Cardiomyopathy

Syncope Causes

T Wave Inversions

Evaluation

ECG

ARVD.png

  • Epsilon wave
    • Seen in 30-50% of cases
    • Most specific finding
    • Small positive deflection at the end of the QRS complex
  • V1-V3 T wave inversions
    • Seen in 85% of cases
    • Especially in patients >14 yrs old)
  • Localised QRS widening
    • 110 ms in V1-V3
  • Sudden VT episodes with LBBB morphology
  • Prolonged S-wave upstroke
    • 55 ms in V1-3
    • Seen in 95% of cases

Imaging

Major and minor criteria rely on echo and cardiac MRI

  • Echocardiography
    • Hypokinetic and dilated right ventricle
    • Dilation of RVOT
  • Cardiac MRI [3]
    • Fibro-fatty infiltration
    • Thinning of right ventricular myocardium
    • RV aneurysm
    • RV dilatation
    • Regional wall motion abnormalities
    • Global systolic dysfunction
  • Histological studies provide post-mortem diagnosis
    • May prompt testing in family members

Management

Disposition

  • Admission under cardiology if symptomatic
  • Admission under cardiology if high-risk features[4]:
    • Syncope due to cardiac arrest
    • Recurrent dysrhythmias not suppressed by drug therapy
    • Cardiac arrest in first-degree relative
  • Incidental finding: Cardiology follow-up for further risk assessment and possible ICD placement or ablation

References

  1. ↑ https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/
  2. ↑ Protonotarios, N., and Tsatsopoulou, A. (2006). Naxos disease: Cardiocutaneous syndrome due to cell adhesion defect. Orphanet Journal of Rare Diseases, 1(4).
  3. ↑ https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/
  4. ↑ https://litfl.com/arrhythmogenic-right-ventricular-cardiomyopathy-arvc/
  • 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.
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