Les tumeurs de l'aorte (à propos d'un cas d'angiosarcome de révélation cutanée, revue de la littérature)

LIMOGES-BU Médecine pharmacie (870852108) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

A rare right ventricular involvement of eosinophilic granulomatosis demonstrated by cardiac MRI

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis with a prevalence rate of seven per million. Cardiac involvement was reported in 20-50%, yet with improved diagnostic methods, the frequency of cardiac involvement is expected to be even higher. It can result in significant morbidity and mortality, accounting for about 50% of death. Cardiac magnetic resonance (CMR) imaging is used to evaluate the myocardium, valves, coronary arteries, pericardium, also to assess cardiac structure and function. Perfusion study allows tissue characterisation with a suggestive pattern of late gadolinium enhancement. CASE REPORT: We report a rare case of EGPA in a 54-year-old male patient who presented with fever, sore throat and dizziness. Echocardiography showed a filling defect at the apex of the right ventricle (RV). CMR findings suggested the diagnosis of EGPA by demonstrating an impressive lesion at RV apex with the typical 3-layer appearance and thrombus formation. Post-gadolinium subendocardial hyperenhancement suggested focal involvement at the inferolateral wall of the left ventricle. Computed Tomography (CT) was done to rule out calcific or soft plaques of the coronary arteries, small vessel vasculitis and small aneurysm. CT scan showed a low-attenuation lesion at the inner wall of the right ventricle. In the lungs, bilateral interstitial changes and bilateral cystic bronchiectases were found. Under appropriate treatment, the patient improved clinically. CONCLUSIONS: It is of crucial importance to perform full cardiac imaging that includes CMR even in asymptomatic patients with suspected EGPA, since early identification of cardiac involvement may allow to apply appropriate therapy and full recovery of the patient