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Myocardial Fatty Foci in Tuberous Sclerosis

2023; Radiological Society of North America; Volume: 307; Issue: 5 Linguagem: Inglês

10.1148/radiol.230156

1527-1315

Matthias Eberhard, Hatem Alkadhi,

Medical Imaging and Pathology Studies

HomeRadiologyVol. 307, No. 5 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyMyocardial Fatty Foci in Tuberous SclerosisMatthias Eberhard , Hatem AlkadhiMatthias Eberhard , Hatem AlkadhiAuthor AffiliationsFrom the Department of Radiology, Spital Interlaken, Spitäler fmi AG, Unterseen, Switzerland (M.E.); and Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, Raemistrasse 100, 8091 Zurich, Switzerland (M.E., H.A.).Address correspondence to M.E. (email: [email protected]).Matthias Eberhard Hatem AlkadhiPublished Online:Jun 27 2023https://doi.org/10.1148/radiol.230156MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In A 44-year-old man presented to the emergency department with new-onset severe back pain after having a seizure. Aortic dissection and pulmonary embolism were excluded with electrocardiogram-gated chest CT angiography, but there were fractures of the fourth through sixth thoracic vertebrae. Numerous sclerotic lesions were found in the thoracic skeleton. CT angiography also showed subepicardial and midmyocardial fat in the left ventricular myocardium and in the right ventricular papillary muscle, which were confirmed with cardiac MRI (Figs 1, 2). Subsequent brain MRI showed cortical tubers and a high-grade subcortical glioma. The patient had a history of multiple seizures during childhood. During hospitalization, the patient was diagnosed with tuberous sclerosis according to the 2012 revised diagnostic consensus criteria (1), meeting four major criteria (angiofibroma, ungual fibromas, shagreen patch, and cortical dysplasias). The patient did not undergo genetic testing.Figure 1: Images in a 44-year-old man who presented to the emergency department with new-onset severe back pain after having a seizure. (A) Four-chamber view from cardiac-gated CT angiography shows subepicardial fat in the left ventricular lateral wall (arrowheads) and the anterior right ventricular papillary muscle (arrow), (B) Four-chamber cinematic rendering shows the anterior papillary muscle fat (arrow) and left ventricular subepicardial fat (arrowheads). (C) T1-weighted four-chamber view from black-blood cardiac MRI shows the anterior papillary muscle fat (arrow) and left ventricular subepicardial fat (arrowheads).Figure 1:Download as PowerPointOpen in Image Viewer Figure 2: (A) Short-axis reformatted CT angiogram, (B) cinematic rendering, and (C) short-axis steady-state free precision cardiac MRI scan show midmyocardial fat in the anterior and anterolateral basal left ventricle wall as well as subepicardial fat in the inferior basal left ventricle wall (arrowheads) and fat in the anterior right ventricle papillary muscle (arrow).Figure 2:Download as PowerPointOpen in Image Viewer Myocardial fatty foci are frequent findings in patients with tuberous sclerosis, being present in the left ventricle in approximately 50%–64% of patients and in the right ventricle in approximately 17%–23% (2,3). The presence and number of myocardial fatty foci have shown a positive correlation with the degree of extracardiac tuberous sclerosis manifestations and are indicative of more severe multiorgan disease (2).Disclosures of conflicts of interest: M.E. Lecture fee from Siemens. H.A. Institutional grants from Bayer, Canon, Guerbet, and Siemens; speakers bureau, Siemens.References1. Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013;49(4):243–254. Crossref, Medline, Google Scholar2. Tresoldi S, Munari A, Di Leo G, et al. Myocardial Fatty Foci in Adult Patients with Tuberous Sclerosis Complex: Association with Gene Mutation and Multiorgan Involvement. Radiology 2015;277(2):398–405. Link, Google Scholar3. Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Fatty foci in the myocardium in patients with tuberous sclerosis complex: common finding at CT. Radiology 2009;253(2):359–363. Link, Google ScholarArticle HistoryReceived: Feb 15 2023Revision requested: Mar 6 2023Revision received: Mar 8 2023Accepted: Mar 20 2023Published online: June 27 2023 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 307, No. 5 Metrics Altmetric Score PDF download