IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart

• Published in: Yonsei medical journal Vol. 54; no. 5; pp. 1285 - 1288
• Main Authors: Song, Changho, Koh, Myoung Ju, Yoon, Yong-Nam, Joung, Boyoung, Kim, Se Hoon
• Format: Journal Article
• Language: English
• Published: Korea (South) Yonsei University College of Medicine 01.09.2013; 연세대학교의과대학
• Subjects: Atrial Septum - pathology; Case Report; Female; Humans; Immunoglobulin G - blood; Middle Aged; Pacemaker, Artificial; Sclerosis - complications; Sclerosis - diagnosis; Sclerosis - therapy; Syncope - etiology; Vena Cava, Superior - pathology; 의학일반; IgG4-related sclerosing disease; sinoatrial node dysfunction; pacemaker
• ISSN: 0513-5796; 1976-2437; 1976-2437
• DOI: 10.3349/ymj.2013.54.5.1285

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.