Arrhythmogenic RV dysplasia

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by fibrofatty replacement of the Anatomy of the right ventricle myocardium.

In the early stage of disease, areas of structural changes are typically confined to the “triangle of dysplasia”: inflow tract, outflow tract, or apex of the right ventricle. Diffuse right ventricular involvement may occur with disease progression, and in approximately a third of patients, left ventricular myocardium (typically posterior wall) is also affected. These changes may result in right ventricular dysfunction and electrical instability leading to ventricular arrhythmias and sudden cardiac death.

Contents

Diagnosing ARVD/C

According to recently revised Task Force criteria [1], major and minor diagnostic criteria are classified into 6 categories:

  1. Global or regional dysfunction and structural alterations,
  2. Tissue characterization of wall,
  3. Repolarization abnormalities,
  4. Depolarization/conduction abnormalities,
  5. Arrhythmias, and
  6. Family history.
  • A definite diagnosis of AVRD/C is made when 2 major or 1 major and 2 minor criteria or 4 minor from different categories are met.
  • A borderline diagnosis of AVRD/C involves 1 major and 1 minor or 3 minor criteria from different categories.
  • The diagnosis of AVRD/C is considered possible if 1 major or 2 minor criteria from different categories are present.

Global or regional right ventricular dysfunction and structural alterations (category I criteria) can be assessed by echocardiography, cardiac magnetic resonance imaging and right ventricular angiography.

Echocardiographic criteria

Echocardiographic assessment can provide either 1 major or 1 minor diagnostic criterion. It includes evaluation of right ventricular wall motion abnormalities/structural changes and the measurement of right ventricular outflow tract (RVOT) diameter in parasternal long-axis (PLAX) and short-axis (PSAX) view [Figure 1].


Major criterion by 2D echocardiography

● Regional RV akinesia, dyskinesia, or aneurysm
and 1 of the following (end diastole):
— PLAX RVOT ≥32 mm (corrected for body size, PLAX/BSA ≥19 mm/m2)
— PSAX RVOT ≥36 mm (corrected for body size, PSAX/BSA ≥21 mm/m2)
— or fractional area change ≥33%


Minor criterion by 2D echocardiography

● Regional RV akinesia or dyskinesia
and 1 of the following (end diastole):
— PLAX RVOT ≥29 to <32 mm (corrected for body size _PLAX/BSA ≥16 to <19 mm/m2)
— PSAX RVOT ≥32 to <36 mm (corrected for body size _PSAX/BSA ≥18 to <21 mm/m2)
— or fractional area change ≥33% to <40%


Echocardiographic assessment of right ventricular function is challenging due to the complex geometry of this chamber and its unfavorable retrosternal position [2]. Both atypical (off axis) 2D echocardiographic views and 3D echocardiography may sometimes be useful. Besides visual assessment, regional right ventricular function may also be quantified using tissue Doppler or speckle tracking based deformation imaging, but the added value of this approach remains to be determined [3].

References

  1. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Protonotarios N, Saffitz JE, Sanborn DM, Steinberg JS, Tandri H, Thiene G, Towbin JA, Tsatsopoulou A, Wichter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121(13):1533-41.
  2. Jurcut R, Giusca S, La Gerche A, Vasile S, Ginghina C, Voigt JU. The echocardiographic assessment of the right ventricle: what to do in 2010? Eur J Echocardiogr. 2010;11(2):81-96.
  3. Teske AJ, Cox MG, De Boeck BW, Doevendans PA, Hauer RN, Cramer MJ. Echocardiographic tissue deformation imaging quantifies abnormal regional right ventricular function in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Soc Echocardiogr. 2009;22(8):920-7.
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