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IgG4-related disease (IgG4-RD) is known to involve the cardiovascular system as aortitis or periaortitis1; however, the number of reported valvulopathies are exceptionally rare. This case is the first with immunohistochemistry proven mitral valve involvement.
A 72-year-old Korean woman with paroxysmal atrial fibrillation and end-stage renal disease due to long-standing diabetes and hypertension presented to the emergency department with near-syncope and dyspnoea with exertion. Further evaluation revealed three-vessel coronary artery disease and severe mitral valve stenosis due to suspected rheumatic mitral valve disease with a mitral valve area of 0.62 cm2, peak gradient of 15.5 mm Hg and mean velocity of 136 cm/s. The patient underwent three-vessel coronary artery bypass graft surgery and mitral valve replacement with a 27 mm pericardial bioprosthesis. Both the anterior and posterior chordae tendineae were …
Handling editor Josef S Smolen
Contributors All authors drafted and revised the manuscript. All authors read and approved the final manuscript.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Next of kin consent obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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