Figure 6
Congenital cardiac and outflow tract anomalies in Tagln-Cre:Tgfbr2 flx/flx. (a–g) micro-CT scan cross sections, volume rendering and segmentation of the heart and outflow tract in mutant and WT littermates. (a,b) Comparison between mutant and WT heart (gold) and outflow tract (bronze). Dilated aneurysmal outflow tract is evident in the mutant, extending towards the neck and filling up the superior mediastinal space. (c,d) sagittal cross sections showing the aneurysmal OF tract. (c) the left main carotid artery (arrow) is not dilated. (d) thoracic organs and liver segmentation showing the configuration of the organs in the thorax. The liver herniates cephalically and the lung occupies a small space between the OF tract and the liver. (e) coronal section at the level of the emerging outflow tract (arrow) showing the OF originating from the right ventricular overriding the large VSD. (f,g) sagittal and coronal sections (respectively) in a WT littermate embryo showing the outflow track; left sided aortic arch (arrow) is present and the emerging brachiocephalic trunk (arrowhead in g). (h–k) transverse (i,k) and coronal (h,j) sections and IHC of E14.5 WT and mutant embryos. (h) the OF tract has a TAGLN + thick wall that displaces the right main bronchus to the left. The bronchi are otherwise showing a patent lumen and a TAGLN + smooth muscle layer surrounds the main bronchi. (i,j) VSD anomaly and thin walled ventricular wall is seen. (k) WT littermates at E14.5 show complete ventricular septum. A: atrium, OF: outflow tract, Lu: lung, LV: left ventricle, R: right side. Scale bars: 1,000 µm in (a–g) 200 µm in (h–k).
