Papillary muscle inflammation in Takayasu's arteritis revealed by FDG-PET
1 Department of Nuclear Medicine, Free University of Brussels (ULB), Academic Erasme Hospital, Route de Lennik 808, Brussels 1070, Belgium
2 Department of Vascular Diseases, Free University of Brussels (ULB), Academic Erasme Hospital, Route de Lennik 808, Brussels 1070, Belgium
3 Department of Radiology, Free University of Brussels (ULB), Academic Erasme Hospital, Route de Lennik 808, Brussels 1070, Belgium
4 Department of Cardiology, Free University of Brussels (ULB), Academic Erasme Hospital, Route de Lennik 808, Brussels 1070, Belgium
5 Department of Nephrology, Free University of Brussels (ULB), Academic Erasme Hospital, Route de Lennik 808, Brussels 1070, Belgium
* Corresponding author. Tel: +32 25553300; fax: +32 5556800. E-mail address: ndumarey{at}ulb.ac.be
An 18-year-old woman with a recent history of Takayasu's arteritis and Crohn's disease was admitted with severe hypertension, haemodynamic pulmonary oedema, fever, and increased serum C-reactive protein. Corticosteroid treatment had been discontinued 4 months prior to admission. Transthoracic echocardiography showed restrictive closure of a mildly thickened mitral valve (Panel A) with severe mitral regurgitation (Panel B) and an elevated pulmonary arterial pressure. Angiography demonstrated stenosing lesions in the superior mesenteric and left renal arteries and in the infra-renal aorta. Subsequently, a whole body FDG-PET was performed in order to seek metabolic signs suggestive of an active Takayasu's disease. FDG uptake in the wall of the aorta and its major branches was normal, but focally increased uptake was detected within the heart (Panel C). These PET findings were further investigated with a cardiovascular MR (CMR). Fusion of FDG-PET with CMR images revealed that the intracardiac hypermetabolic foci corresponded to the posterior papillary muscle of the mitral valve (Panel D). In addition, the myocardial wall surrounding the annulus fibrosus of the aortic valve was also hypermetabolic (Panel E). CMR showed abnormal kinetics of the mitral valve with a club-shaped aspect of its posterior papillary muscle (Panel F). Transesophageal echocardiography showed a mildly thickened mitral valve without any vegetation. Bacterial blood cultures remained negative.
Heart involvement by Takayasu's disease was diagnosed, and corticosteroids were successfully re-introduced. Two months later, the formerly observed cardiac foci of increased FDG uptake completely disappeared on a follow-up FDG-PET. A pattern of FDG uptake limited to cardiac valvular structures should raise the suspicion of a local inflammatory process.
Panels A and B. Systolic freeze-frame of parasternal echocardiography showing restrictive mitral valve closure (A) and severe mitral regurgitation (B). LA, left atrium; LV, left ventricle.
Panel C. Cardiac foci with increased uptake visualized on whole body FDG-PET.
Panel D. FDG-PET/CMR fusion: foci with increased FDG uptake in the left ventricle are located in the posterior papillary muscle (arrows).
Panel E. Other foci with increased FDG uptake are located in the myocardial wall surrounding the annulus fibrosus of the aortic valve on FDG-PET/CMR fusion images (arrows).
Panel F. CMR: the posterior papillary muscle has a club-shaped aspect (arrow).
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