Cardiac involvement in Erdheim-Chester disease: a case report.

• Autor(a) principal: Pivatto Júnior, Fernando
• Data de Publicação: 2015
• Outros Autores: Piardi, Diogo Silva, Torres, Felipe Soares
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Clinical and Biomedical Research
• Texto Completo: https://seer.ufrgs.br/index.php/hcpa/article/view/51436
• topic: Erdheim-Chester disease; heart failure; magnetic resonance imaging

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis of unknown etiology characterized by proliferation of lipid-containing foamy histiocytes affecting bones and potentially every organ. There is a wide clinical spectrum of the disease, ranging from asymptomatic bone or soft-tissue lesions to life-threatening systemic involvement. Although the initial descriptions published by Jakob Erdheim and William Chester in 1930 included a patient with right atrial infiltration on autopsy, cardiovascular involvement in ECD has only gained more recognition recently. In the present report, we describe a case with cardiac involvement, presenting with symptomatic heart failure and a cardiac mass assessed with echocardiogram and cardiac magnetic resonance imaging.