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

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 t...

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Published inYonsei 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
LanguageEnglish
Published Korea (South) Yonsei University College of Medicine 01.09.2013
연세대학교의과대학
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ISSN0513-5796
1976-2437
1976-2437
DOI10.3349/ymj.2013.54.5.1285

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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.
AbstractList 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.
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.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.
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. KCI Citation Count: 9
Author Yoon, Yong-Nam
Kim, Se Hoon
Song, Changho
Koh, Myoung Ju
Joung, Boyoung
AuthorAffiliation 2 Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
3 Department of Cardiothoracic Surgery, Yonsei University College of Medicine, Seoul, Korea
1 Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
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Issue 5
Keywords IgG4-related sclerosing disease
sinoatrial node dysfunction
pacemaker
Language English
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SubjectTerms 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
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Title IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart
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