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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.
True FISP image. Four chamber view shows severe global enlargement of the right ventricle (RV) with localised dilation at the inflow tract (black arrows). The left ventricle (LV) is also moderately dilated. Regional thinning of the posterolateral LV wall is observed (black arrows). Normal LV wall thickness is highlighted by white arrows for comparison. LA, left atrium; RA, right atrium. To view video clip of left ventricular outflow tract, visit the Heart website—http://www.heartjnl.com/supplemental
T2 weighted spin echo image in the four chamber view with fat suppression. The bright areas within the grey myocardium (white arrows) are likely to represent oedema, suggesting the presence of patchy inflammatory infiltrates.
Following intravenous administration of gadolinium-DTPA, late enhancement was observed in both the right ventricle (RV) (black arrows) and the septum (white arrows). Since fat was absent from these regions on corresponding T1 weighted turbospin images, the appearances are consistent with myocardial fibrosis. The mid-myocardial distribution is typical of fibrosis secondary to cardiomyopathy.
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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