Something you better know if your patient speaks Italian!
Episode 147

June 23, 2014


ECG Differentials for Syncope:

  • Ischemia
  • Dysrhythmias & AV-blocks
  • Intervals
    • WPW / Pre-excitation
    • Long / Short QT syndromes
  • Hypertrophic cardiomyopathy
  • Brugada syndrome
  • Arrhythmogenic RV dysplasia​ (ARVD)

ARVD - Epsilon waves

Background

  • Not common except in certain populations
    • ​General population: 1/1,000-1/10,000
    • Italy: 40/10,000
      • Most common cause of sudden death in young
  • Familial (autosomal dominant)
  • Male > Female
  • Caused by progressive replacement of RV myocardium by fibrofatty tissue that interferes with impulse conduction

Clinical 

  • Common cause of ventricular arrhythmias and sudden death in adolescents and young adults, athletes
  • Usually manifests between puberty - 60 years of age
  • Often have FHx of premature sudden death in family members < 35 years old
  • ECG findings are imperfect
  • ECHO, MRI, MDCT, and endomyocardial biopsy used for diagnosis

ECG findings

  • Leads V1-V3
    • ​Epsilon waves
    • TWI’s 
    • Slight prolongation of QRS
  • LBBB-type VT or many PVC’s

Management

  • Consult electrophysiology
  • Active ventricular dysrhythmias
    • Stable: Beta blockers, Class III antiarrhythmics
    • ICD placement in patients with cardiac arrest, syncope, VT
  • ​​Heightened concern if
    • Young, especially males
    • Family Hx of early sudden death
    • Many PVC’s
    • LBBB-type VT
    • Epsilon Waves with TWI in V1-V3

Reference:

Basso C, Corrado D, Marcus FL, et al. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009 Aril 11;373(9671):1289-300. PMID:19362677

Something you better know if your patient speaks Italian!
Episode 147

June 23, 2014


ECG Differentials for Syncope:

  • Ischemia
  • Dysrhythmias & AV-blocks
  • Intervals
    • WPW / Pre-excitation
    • Long / Short QT syndromes
  • Hypertrophic cardiomyopathy
  • Brugada syndrome
  • Arrhythmogenic RV dysplasia​ (ARVD)

ARVD - Epsilon waves

Background

  • Not common except in certain populations
    • ​General population: 1/1,000-1/10,000
    • Italy: 40/10,000
      • Most common cause of sudden death in young
  • Familial (autosomal dominant)
  • Male > Female
  • Caused by progressive replacement of RV myocardium by fibrofatty tissue that interferes with impulse conduction

Clinical 

  • Common cause of ventricular arrhythmias and sudden death in adolescents and young adults, athletes
  • Usually manifests between puberty - 60 years of age
  • Often have FHx of premature sudden death in family members < 35 years old
  • ECG findings are imperfect
  • ECHO, MRI, MDCT, and endomyocardial biopsy used for diagnosis

ECG findings

  • Leads V1-V3
    • ​Epsilon waves
    • TWI’s 
    • Slight prolongation of QRS
  • LBBB-type VT or many PVC’s

Management

  • Consult electrophysiology
  • Active ventricular dysrhythmias
    • Stable: Beta blockers, Class III antiarrhythmics
    • ICD placement in patients with cardiac arrest, syncope, VT
  • ​​Heightened concern if
    • Young, especially males
    • Family Hx of early sudden death
    • Many PVC’s
    • LBBB-type VT
    • Epsilon Waves with TWI in V1-V3

Reference:

Basso C, Corrado D, Marcus FL, et al. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009 Aril 11;373(9671):1289-300. PMID:19362677

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