Rosai-Dorfman disease is rare and typically presents with cervical lymphadenopathy, but may manifest as extranodal disease. This disease is generally indolent and self-limited, but it carries a poor or fatal prognosis when it is advanced or when it involves and compresses vital structures. We present a case of Rosai-Dorfman disease affecting the pulmonary arteries in a 22-year-old woman with severe, symptomatic right heart failure.

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http://dx.doi.org/10.1016/j.athoracsur.2009.05.016DOI Listing

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