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.
2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.