CTICasesEndocardial Fibroelastosis
Imaging findings
Non-gated cardiac CT in a five-month-old with congenital aortic stenosis and heart failure shows a marked circumferential band of low attenuation approximately one to two millimeters in thickness along the entire left ventricular endocardial surface. This band of low attenuation is most visible in the delayed venous phase and corresponds pathologically to the glistening white-tan fibrous tissue that was stripped surgically. The aortic valve appears thickened.
Key takeaways
Endocardial fibroelastosis is a rare condition characterized by diffuse endocardial thickening of the left ventricle that occurs in the setting of increased mechanical stress on the developing left ventricle, most commonly from congenital aortic stenosis. On CT it appears as a thin circumferential band of low attenuation along the left ventricular endocardial surface in the delayed phase. On MRI, it produces diffuse subendocardial delayed enhancement not following a coronary vascular territory. Surgical stripping of the fibroelastic layer can be performed as part of repair.
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