IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart.
10.3349/ymj.2013.54.5.1285
- Author:
Changho SONG
1
;
Myoung Ju KOH
;
Yong Nam YOON
;
Boyoung JOUNG
;
Se Hoon KIM
Author Information
1. Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Reports ; Research Support, Non-U.S. Gov't
- Keywords:
IgG4-related sclerosing disease;
sinoatrial node dysfunction;
pacemaker
- MeSH:
Atrial Septum/*pathology;
Female;
Humans;
Immunoglobulin G/*blood;
Middle Aged;
Pacemaker, Artificial;
Sclerosis/complications/diagnosis/therapy;
Syncope/etiology;
Vena Cava, Superior/*pathology
- From:Yonsei Medical Journal
2013;54(5):1285-1288
- CountryRepublic of Korea
- Language:English
-
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.
