Arrhythmogenic Right Ventricular Dysplasia (ARVD)

 

 

History: 19 Year-old male is referred for cardiac MRI to assess for ARVD. Patient presented to ER with syncope while playing sofball. He had to prior near-syncopal episodes during exercise. He has no family history of syncope or sudden death. The ECG was suggestive of ARVD (reported to have abnormal repolarization). The echocardiogram performed at the referring hospital showed severe RV enlargement and abnormal wall motion.

 

Technique: Axial FIESTA, Axial and sagittal DIR, TIR, pre-contrast myocardial enhancement, post-contrast MDE, short- and long-axis FIESTA series.

 

Findings:

  • Diffuse RV hypokinesis with focal areas of dyskinesis (top row). The RV EF is severely decreased (measured as 17%).
  • Severe RV enlargement.
  • There is bright signal intensity in the region of the RV free wall (middle row) in the DIR images.
  • Post-contrast images show diffuse hyperenhancement of the RV wall (bottom row).

The above findings are consistent with fibrofatty infiltration of the RV suggesting ARVD.

Additional findings (not shown): Normal LV function with an EF of 50%, no abnormal enhancement in the LV wall.

 

Comment: Diagnostic criteria for ARVD would be fulfilled by the presence of:

  • 2 major criteria or
    • 1 major plus 2 minor criteria or
    • 4 minor criteria

The criteria must come from different categories (I-VI).

 

  1. Global and/or Regional Dysfunction and Structural Alterations (Detected by echocardiography, angiography, magnetic imaging, or radionuclide scinitigraphy).

 

MAJOR:

  • Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment.
  • Localized right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulging.)
  • Severe segmental dilatation of the right ventricle.

MINOR:

  • Mild global right ventricular dilatation and/or ejection fraction reduction with normal left ventricle.
  • Mild segmental dilatation of the right ventricle.
  • Regional right ventricular hypokinesia.

 

  1. Tissue Characterization of Walls

MAJOR:

  • Fibrofatty replacement of myocardium on endomyocardial biopsy.

 

III. Repolarisation Abnormalities

MINOR:

  • Inverted T waves in right precordial leads (V2 and V3) (people aged>12 years, in absence of right bundle branch block.)

 

  1. Depolarisation/Conduction Abnormalities

MAJOR:

  • Epsilon waves or localised prolongation (>110ms) of the QRS complex in right precordial leads (V1-V3.)

MINOR:

  • Late potentials (signal averaged ECG.)

 

  1. Arrhythmias

MINOR:
Left bundle branch block type ventricular tachycardia (sustained and nonsustained) ECG, Holter, exercise testing.
Frequent ventricular extrasystoles (>1000/24 hours) (Holter.)

 

  1. Family History

MAJOR:

  • Familial disease confirmed at necropsy or surgery.

MINOR:

  • Familial history of premature sudden death (<35 years) due to suspected right ventricular dysplasia.
  • Familial history (clinical diagnosis based on present criteria.)

 

McKenna WJ, Thiene G, Nava A, Fontaliran F. Blomstrom-Lundqvist C, Fontaine G, Camerini on behalf of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology, supported by the Schoepfer Association. Diagnosis of Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy. Br Heart J 1994;71:215-218.