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Yonsei University College of Medicine, Yonsei Medical Journal, 5(54), p. 1285, 2013

DOI: 10.3349/ymj.2013.54.5.1285

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

Journal article published in 2013 by Changho Song, Myoung Ju Koh, Yong-Nam Yoon, Boyoung Joung, Se Hoon Kim ORCID
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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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.