title: IgG4-related sclerosing disease involving the superior vena cava and the atrial septum of the heart.
abstract: A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.
authors.affiliation: Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea.
authors.forename: Changho
authors.initials: C
authors.lastname: Song
authors.forename: Myoung Ju
authors.initials: MJ
authors.lastname: Koh
authors.forename: Yong-Nam
authors.initials: YN
authors.lastname: Yoon
authors.forename: Boyoung
authors.initials: B
authors.lastname: Joung
authors.forename: Se Hoon
authors.initials: SH
authors.lastname: Kim
mesh_terms: D054087:Atrial Septum / Q000473:pathology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008875:Middle Aged; D010138:Pacemaker, Artificial; D012598:Sclerosis / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D013575:Syncope / Q000209:etiology; D014683:Vena Cava, Superior / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4-related sclerosing disease; pacemaker; sinoatrial node dysfunction
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