PURPOSE: To examine the morphologic characteristics of focal fatty foci in the myocardium of patients with tuberous sclerosis complex (TSC) at computed tomography (CT). MATERIALS AND METHODS: Institutional review board approval was obtained, and patient informed consent was waived. Fifty-five patients with TSC (mean age, 37 years; range, 16-67 years; 22 male patients) who had CT results available that included at least the basal portions of the heart were included. Fifty-five age- and sex-matched control subjects without TSC were selected from a CT database. Images were reviewed for the presence of areas of fat attenuation in the depicted portions of the myocardium. Descriptive statistics and the McNemar test for case-control comparisons were used. RESULTS: CT results demonstrated foci of fat attenuation within the myocardium in 35 (64%) of 55 patients with TSC. Foci were well circumscribed and focal and located in the interventricular septum, left ventricle wall, right ventricle wall, and papillary muscles. Size varied between 3 x 1 mm and 62 x 31 mm. Multiple lesions were seen in 19 patients. In the control group, only one (2%) lesion with fat attenuation was found (P < .001). Its linear shape and subendocardial location in the left ventricular wall differed from the morphology of fatty foci seen in patients with TSC. CONCLUSION: Despite incomplete depiction of the heart with CT, the majority of patients with TSC demonstrated well-circumscribed foci of fat attenuation in the myocardium that were not present in age- and sex-matched control subjects. This suggests that such fatty foci may be another characteristic of TSC. (c) RSNA, 2009.
PURPOSE: To examine the morphologic characteristics of focal fatty foci in the myocardium of patients with tuberous sclerosis complex (TSC) at computed tomography (CT). MATERIALS AND METHODS: Institutional review board approval was obtained, and patient informed consent was waived. Fifty-five patients with TSC (mean age, 37 years; range, 16-67 years; 22 male patients) who had CT results available that included at least the basal portions of the heart were included. Fifty-five age- and sex-matched control subjects without TSC were selected from a CT database. Images were reviewed for the presence of areas of fat attenuation in the depicted portions of the myocardium. Descriptive statistics and the McNemar test for case-control comparisons were used. RESULTS: CT results demonstrated foci of fat attenuation within the myocardium in 35 (64%) of 55 patients with TSC. Foci were well circumscribed and focal and located in the interventricular septum, left ventricle wall, right ventricle wall, and papillary muscles. Size varied between 3 x 1 mm and 62 x 31 mm. Multiple lesions were seen in 19 patients. In the control group, only one (2%) lesion with fat attenuation was found (P < .001). Its linear shape and subendocardial location in the left ventricular wall differed from the morphology of fatty foci seen in patients with TSC. CONCLUSION: Despite incomplete depiction of the heart with CT, the majority of patients with TSC demonstrated well-circumscribed foci of fat attenuation in the myocardium that were not present in age- and sex-matched control subjects. This suggests that such fatty foci may be another characteristic of TSC. (c) RSNA, 2009.
Authors: Neda Rastegar; Jeremy R Burt; Celia P Corona-Villalobos; Anneline S Te Riele; Cynthia A James; Brittney Murray; Hugh Calkins; Harikrishna Tandri; David A Bluemke; Stefan L Zimmerman; Ihab R Kamel Journal: Radiographics Date: 2014-10 Impact factor: 5.333
Authors: H Hannoush; V Sachdev; A Brofferio; A E Arai; G LaRocca; J Sapp; S Sidenko; C Brenneman; L G Biesecker; K M Keppler-Noreuil Journal: Am J Med Genet A Date: 2014-11-06 Impact factor: 2.802