CTICasesErdheim-Chester Disease with Right Coronary Artery and Interatrial Septal Involvement
[...]. Erdheim-Chester Disease with Right Coronary Artery and Interatrial Septal Involvement. CTICases. Published May 17, 2018. https://str-case-explorer.pages.dev/c/4rpyb
Imaging findings
In a previously published case, soft tissue infiltration is seen surrounding the right coronary artery and extending into the interatrial septum on cardiac CT, with an appearance closely resembling the circumferential retroperitoneal soft tissue pattern seen in other cases of the same disease.
Key takeaways
This well-known published case illustrates that the same infiltrative periarterial soft tissue pattern characteristic of Erdheim-Chester disease in the retroperitoneum can also involve the heart, encasing the coronary arteries and infiltrating the interatrial septum, a rare but increasingly recognized cardiac manifestation of the disease.
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