48years old man, with 2 syncopal episodes and frequent ventricular extrasystolia.
What do you think about the diagnosis?
A – Myocarditis B – Amyloidosis C – Arrhythmogenic dysplasia D – Dilated cardiomyopathy
Correct answer is C) : Arrhythmogenic dysplasia
The answer is arrhythmogenic dysplasia that involves both ventricles in this case. CMR showes biventricular dilation (VTD-LV 247ml/ 111ml/m2, VTD-RV 302ml/ 135ml/m2) with mild reduced EF (LV-EF=53%, RV-EF= 47%) and regional wall motion abnormalities of the right ventricle. LGE sequences show the presence of irregular subepicardic hyper-enhancement of the left ventricle and hyper-enhancement of the right ventricle walls. The reduced T1 value suggests myocardial adipose infiltration. All the findings are included in the diagnostic criteria of arrhythmogenic dysplasia, which has been confirmed by genetic investigation.
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