Erdheim-Chester disease (ECD) is a rare, inflammatory disease of unknown etiology, characterized by xanthogranulomatous tissue infiltration (Ref. 1). Cardiac involvement develops in up to 22% pts, is associated with the worst prognosis and is often underestimated (Ref. 2). The aim of our study was to investigate cardiac involvement in ECD, comparing echocardiography and Cardiac Magnetic Resonance (CMR) in its detection.

We enrolled 10 patients (7 males and 3 females, mean age 56 years)with histologically-proven ECD, who underwent transthoracic echocardiography (TTE) and CMR with contrast medium.

The most characteristical findings were an extra-cardiac mass and pericardial effusion. TTE detected the mass in 5 pts (50%; mean major mass diameter 31 mm; range 25– 37 mm), whereas CMR detected the mass in all pts (100%; mean major mass diameter 27 mm; range 15–40 mm),and better defined peri- ed extra- cardiac involvement, showing extension to the right atrioventricular sulcus, right atrial free wall, right coronary artery and contiguous vessels.
 Detected mass had no specific ultrasound features, whereas at CMR had distinctive features: dishomogeneous...

CMR is more sensitive than TTE in detecting pathological tissue, probably due to right chambers localization, worse studied by TTE, besides the intrinsic TTE operator dependence and risk of suboptimal acoustic window.

Reference 1: Haroche J et al. Erdheim-Chester disease. Rheum Dis Clin North Am 2013 Reference 2: Haroche J et al. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine 2004