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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

From: Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder

Fig. 5

Laboratory and imaging examinations for TGCV. a Representative images of May-Giemsa staining of blood smears were shown from patients with P-TGCV and I-TGCV. b Bull’s eye images for BMIPP scintigrams from patients with P-TGCV and I-TGCV. The first scan was performed 20 min post-injection to determine early BMIPP uptake, and the second scan was performed 200 min later to study delayed uptake using myocardial SPECT after patients were injected with 123I-BMIPP. WOR was calculated with the Hear Risk View-S (HRSV) software as the difference between early and delayed images (reference value, 19.4 ± 3.2%). c A patient with P-TGCV showed numerous vacuoles (Panel a, H&E) in cardiomyocytes that stained positively for oil red O (ORO) (inset in Panel b). Furthermore, no positive reactivity for ATGL observed in any of cell types (Panel b, ATGL). Cardiomyocytes of patients with I-TGCV showed numerous vacuoles (Panel c, HE) filled with stained lipid (inset in Panel d, ORO), whereas positive reactivity for ATGL observed not only in adipocytes but also in cardiomyocytes (arrows in Panel d, ATGL). Scale bars: Panels a-d, 30 μm. d Coronary CT angiograms (CTA) from patients with P-TGCV and I-TGCV are shown. Bars in CTA correspond to Panels a-d, which are short axial sections of the left anterior descending coronary artery. The segmentation of the coronary artery lumen and wall was done using a workstation (Ziostation 2, Ziosoft, Japan). Constitutive components were classified into 4 colors with the original analysis software as follows. Colors indicate the CT number (yellow, − 25–0; orange, 0–40; green, 40–215; red, 215–700 Hounsfield unit [HU] (M@XNET, Tokyo, Japan) in Panels a-d. Yellow or orange areas indicate lipid components, red shows blood, and green shows the arterial wall without calcification or lipids. Black arrows in Panels a, b, and c indicate outside-in protrusion, which is the characteristics for TGCV

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