CASE REPORT   

Gazzetta Medica Italiana Archivio per le Scienze Mediche 2018 May;177(5):243-5

DOI: 10.23736/S0393-3660.17.03544-6

Copyright © 2017 EDIZIONI MINERVA MEDICA

lingua: Inglese

A rare association of sarcoidosis and severe mitral stenosis in young female

Katerina HLAVATA ¹, Radka KOCKOVA ¹ ✉, Jana MALUSKOVA ², Josef KAUTZNER ¹, Jan PIRK ³

¹ Department of Cardiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic; ² Department of Clinical and Transplant Pathology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic; ³ Department of Cardiovascular Surgery, Institute for Clinical and Experimental Medicine, Prague, Czech Republic

Association of sarcoidosis and severe heart valve stenosis is extremely rare entity. We describe the case of a 35-year-old female who presented with coincidence of symptomatic pulmonary sarcoidosis and rapid progression of mitral valve stenosis. During four months of corticosteroid therapy her pulmonary sarcoidosis resolved but she developed severe mitral stenosis leading to acute heart failure. She was treated by a semi-urgent surgical mitral valve replacement with St. Jude Medical Masters HP valve prosthesis. There was a massive thrombotic mass on the atrial site of both mitral valve leaflets. Histology analysis of the excised mitral valve specimen was negative for noncaseating granuloma but advanced degenerative changes and myocardial fibrosis were identified. Our patient made a good recovery and currently is asymptomatic on Coumadin, corticosteroid and immunosuppressive medication. Clearly five months of corticosteroid therapy influenced the myocardial histology finding together with the resolution of pulmonary sarcoidosis on her chest X-ray prior to surgery. We are convinced that the rapid mitral valve disease progression was directly associated with sarcoidosis.

KEY WORDS: Pulmonary sarcoidosis - Heart valve disease - Mitral valve stenosis