Cardiac involvement in Erdheim- Chester disease: MRI findings and literature revision

Abstract

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis, characterized by the involvement of several organs. The lesions may be skeletal or extra-skeletal: in particular, long bones, skin, lungs, and the cardiovascular and the central nervous systems can be affected. In this report, we describe a case of a 34-year-old man, who came to our observation with symptomatic ECD, for a correct assessment of the degree of cardiac involvement through magnetic resonance imaging (MRI).

Keywords: Erdheim-Chester disease; cardiac magnetic resonance; histiocytosis; non-Langerhans.

Fig. 1.

Cardiac MRI cine-balanced fast field echo four-chamber view, showing hypointense tissue surrounding the right coronary artery and extending into the right atrio-ventricular groove.

Fig. 2.

Cardiac MRI cine-balanced fast field echo sagittal view, showing extensive hypointense tissue surrounding the ascending aorta and the right ventricular chamber.

Fig. 3.

Cine video clip showing the involvement of the right coronary artery surrounded by the extensive hypointense tissue and extending through the atrio-ventricular groove, with preserved right ventricular motion.

Fig. 4.

Axial postcontrast T1W MR image showing mild enhancement of the hypointense tissue, surrounding the right coronary artery.

Fig. 5.

Axial T2W (a) and postcontrast T1W MR (b) images showing involvement of both kidneys that were surrounded by an extensive inhomogeneous tissue, which was hypointense to the contiguous structures on T2W images, showing mild contrast enhancement on axial postcontrast T1W images.