Papillary Fibroelastoma of the Aortic Valve
Appearance in 64-Slice Spiral Computed Tomography, Magnetic Resonance Imaging, and Echocardiography
A 60-year-old man with an acute stroke of unknown origin was referred to our institution for further diagnostic evaluation. Contrast-enhanced ECG-gated 64-slice spiral computed tomography depicted a well-defined, pedunculated, mobile, spherical lesion (density 69±21 Hounsfield units) attached to the commissure of the left coronary and noncoronary aortic valve leaflet. The lesion showed a relatively homogenous inner structure with a slightly villous outer margin (Figure 1A through 1D). ECG-gated magnetic resonance imaging exhibited a solid lesion with intermediate signal intensity on both T1- and T2-weighted spin-echo sequences (Figure 2A and 2B). Transthoracic and multiplane transesophageal echocardiography also demonstrated a spherical solid structure within the aortic root, confirming the computed tomography and magnetic resonance imaging findings (Figures 3A and 3B and 4A through 4C). Dynamic computed tomography files (using the temporal information of the recorded 4D computed tomography dataset), cine magnetic resonance imaging (using dynamic steady-state free-precession gradient-echo sequences), and color-coded Doppler echocardiography demonstrated a competent aortic valve with a highly mobile, slightly deformable intraluminal mass (Movies I through IV). Surgery was performed and the tumor was completely removed. The gross pathological specimen appeared as a translucent, gelatinous mass (Figure 5A). Histological examination revealed a benign tumor with multiple papillary fronds of different size that consisted of an acellular matrix surrounded by a single layer of endothelial cells, leading to the definite diagnosis of papillary fibroelastoma (Figure 5B and 5C).
The online-only Data Supplement, consisting of movies, is available with this article at http://circ.ahajournals.org/cgi/content/full/115/1/e3/DC1.