Rosai-Dorfman disease (RDD) is a nonmalignant disease of histiocyte proliferation.¹ The etiology of RDD is poorly defined, with both viral and genetic contributions reported.^2,3 RDD classically presents with painless cervical lymphadenopathy, although extranodal involvement has been reported in approximately one-third of patients. Cardiac involvement is rare and was documented in <0.1% of cases.⁴ We present a case of cardiac RDD with obstruction at the inferior vena cava (IVC)–right atrial (RA) junction resulting in multisystem organ dysfunction.

A 63-year-old woman presented to medical attention with nonexertional chest pain and presyncope. She was found to be in atrial flutter without evidence of acute coronary syndrome. Further investigation with a transthoracic echocardiogram showed increased flow velocities (1.4 m/s) in the pulmonary veins (PVs).

A cardiac computed tomographic scan revealed asymmetric left atrial (LA) thickening with narrowing of the right-sided PVs (Figure 1). Cardiac magnetic resonance imaging showed a soft-tissue mass arising from the base of the interatrial septum (IAS) partially obstructing the right-sided PVs and mildly narrowing the superior vena cava inflow. A positron emission tomographic scan showed no evidence of malignant metastasis.