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Arrhythmogenic right ventricular cardiomyopathy

Case contributed by Igor Yarmola

Presentation

Arrhythmia, exercise intolerance, dyspnea. Tricuspid annuloplasty at age of 9 months. Family history of sudden cardiac death.

Patient Data

Age: 14 years
Gender: Female
  • RV-EDVI: 189 mL/m2
  • RV-EF: 13.9%

The right ventricle (RV) is severely dilated. There are dyskinesia and akinesia of the middle and basal parts of the right ventricle with associated late gadolinium enhancement which refer to a structural abnormality of the myocardium.

There is also a susceptibility artifact of the tricuspid valve after valve repair in early childhood.

Case Discussion

This is a case of ARVC based on the presence of two major criteria according to the revised Padua criteria 1:

In this case, there are two major criteria (according to the Padua criteria 1):

  1. severe right ventricular dilation (RV-EDVI > 100 mL/m2) with akinetic and dyskinetic bulging segments of the right ventricular free wall
  2. transmural late gadolinium enhancement in ≥1 right ventricular region

 

The case is presented together with my colleagues: Anikin A., Barskiy V. and Basargina E.

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