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A 43 year old man with known arrhythmogenic right ventricular cardiomyopathy (ARVC) was referred for a cardiovascular magnetic resonance (CMR) scan to assess disease progression. The diagnosis had been established 20 years earlier by right ventricular angiography. There was no evidence of coronary artery disease. He had previously suffered two cardiac arrests and declined intervention; recurrent ventricular tachycardia was eventually controlled by amiodarone treatment.
Characteristic pathological findings in ARVC include global and local dilation of the right ventricle, aneurysms, loss of myocardium, and replacement with adipose and fibrous tissue. Focal inflammatory infiltrates are also reported. Left ventricular involvement is common in advanced cases. Although CMR aims to identify these abnormalities in vivo, concomitant presence of all the hallmark morphological features in a single case is a rarity. Equally remarkable is the detection of fibrosis in both ventricles and myocardial oedema.
A video sequence accompanies this article.
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- [view video] - 1.2MB - Left ventricular outflow tract view. A dyskinetic area near the LV apex that bulges during diastole is highlighted