Arrhythmogenic right ventricular cardiomyopathy

Case contributed by Dr Azza Elgendy

Presentation

Adult male with a long history of dyspnoea and exercise intolerance. ECG revealed a bundle branch block

Patient Data

Age: 35 years
Gender: Male

Multiple cine sequences show an enlarged right ventricle, abnormal right ventricle wall motion including dyskinesia and paradoxical movement most notable at the segment connecting the RVOT to the right ventricular apex. There is also abnormal corrugation of the right ventricle apex. No abnormal enhancement on the late gadolinium sequence. The 4 CH cine is compromised by the patients' arrhythmia. 

Case Discussion

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by progressive replacement of normal myocardium in the right ventricle by fibrofatty tissue, this finding can be depicted on MRI, however, the histopathology is the most reliable tool. The most common location for the fibrofatty  changes are the RVOT, right ventricle apex and the inferior surface of the right ventricle 1.

The diagnosis of ARVC is based on the presence of major and minor criteria according to the revised Task Force Criteria 2010. The diagnostic criteria include MRI, echocardiography, ECG, and histology. 

The rule of MRI is to study the structure and function of the RV. In our case there are two major criteria include decreased RVEF < 40 %, and increased  RV index ( EDV/ BSA ) is 116. In addition to abnormal right ventricular wall motion, in the form of paradoxical movement of the segment connecting the RVOT to the right apex.

Revised Task Force MRI Criteria 2010

MAJOR Criteria

  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • AND 1 of the following

                RVEDV index > 110 mL/m2 (male)

                RVEDV index > 100 mL/m2 (female)

                RVEF < 40%

MINOR Criteria

  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • AND ONE of the following

                RVEDV index > 100 to <110 mL/m2 (male)

                RVEDV index > 90 to < 100 mL/m2 (female)

                RVEF > 40%  to  < 45%

Definite: 2 Major, or 1 Major + 2 Minor, or 4 Minor 

Borderline : 1 Major + 1 Minor, or 3 Minor

Possible : Major or 2 Minor

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Case information

rID: 57972
Published: 2nd Feb 2018
Last edited: 14th Aug 2019
System: Cardiac
Inclusion in quiz mode: Included

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