Successful Transcatheter Diagnosis and Medical Treatment of Right Atrial Involvement in IgG4-related Disease A Case Report and Review of the Literature

Toshiyuki Yano; Motohisa Yamamoto; Atsushi Mochizuki; Toshifumi Ogawa; Nobutaka Nagano; Takefumi Fujito; Junichi Nishida; Daigo Nagahara; Koki Abe; Takayuki Miki; Chisako Suzuki; Hiroki Takahashi; Hatsue Ishibashi-Ueda; Tetsuji Miura

doi:10.1536/ihj.17-467

Case Reports

Successful Transcatheter Diagnosis and Medical Treatment of Right Atrial Involvement in IgG4-related Disease

A Case Report and Review of the Literature

Toshiyuki Yano, Motohisa Yamamoto, Atsushi Mochizuki, Toshifumi Ogawa, Nobutaka Nagano, Takefumi Fujito, Junichi Nishida, Daigo Nagahara, Koki Abe, Takayuki Miki, Chisako Suzuki, Hiroki Takahashi, Hatsue Ishibashi-Ueda, Tetsuji Miura

Author information

Toshiyuki Yano
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Motohisa Yamamoto
Department of Rheumatology and Clinical Immunology, Sapporo Medical University
Atsushi Mochizuki
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Toshifumi Ogawa
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Nobutaka Nagano
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Takefumi Fujito
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Junichi Nishida
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Daigo Nagahara
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Department of Infection Control and Laboratory Medicine, Sapporo Medical University
Koki Abe
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Takayuki Miki
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University
Chisako Suzuki
Department of Rheumatology and Clinical Immunology, Sapporo Medical University
Hiroki Takahashi
Department of Rheumatology and Clinical Immunology, Sapporo Medical University
Hatsue Ishibashi-Ueda
Department of Pathology, National Cerebral and Cardiovascular Center
Tetsuji Miura
Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University

Keywords: Cardiac tumor, Heart, Biopsy, Intracardiac echocardiography

JOURNAL FREE ACCESS

2018 Volume 59 Issue 5 Pages 1155-1160

DOI https://doi.org/10.1536/ihj.17-467

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Abstract

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder characterized by lymphoplasmacytic infiltration of numerous IgG4-positive plasma cells, leading to fibrous thickening in the affected tissue. Typical cardiovascular manifestations of IgG4-RD are periaortitis, coronary arteritis, and pericarditis. Rare cases of myocardial involvement in IgG4-RD have been reported, but surgical resection or open biopsy was required for the diagnosis in those cases. Here, we report a case in which percutaneous transcatheter biopsy under the guidance of intracardiac echocardiography was useful for diagnosis of IgG4-RD manifested as an intracavitary right atrial mass, extending into the superior vena cava. Successful transcatheter diagnosis of myocardial involvement of IgG4-RD led to immediate favorable response to steroid therapy. Including the present case, previous IgG4-RD cases with myocardial involvement are reviewed to delineate its clinical characteristics.

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