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Updated: May 11, 2026

In vitro Assessment of Aortic Regurgitation Using Four-Dimensional Flow Magnetic Resonance Imaging
Published on: February 25, 2022
Immunoglobulin G4-related disease of the heart causing aortic regurgitation and heart block
Hidetoshi Yamauchi1, Hiroyuki Satoh, Tomoyoshi Yamashita
1Division of Cardiovascular Surgery, Hokkaido PWFAC (Prefectural Welfare Federation of Agricultural Cooperative) Obihiro-Kosei General Hospital, Obihiro, Hokkaido, Japan. okh-i14byam@obihirokousei-hp.jp
Abstract:
Immunoglobulin (Ig) G4-related disease is a novel disease entity characterized by diffuse lymphoplasmacytic infiltrates and the presence of abundant IgG4-positive plasma cells in extensive fibrosis, frequently associated with tumorous swelling lesion and elevated serum IgG4 concentrations. Immunoglobulin G4-related disease has been described in almost every organ system but rarely affects the heart. We describe a rare case of IgG4-related disease of the heart causing aortic regurgitation and heart block in a 59-year-old woman. The excised lesion revealed a high ratio of IgG4-positive to IgG-positive plasma cells, providing a definite diagnosis of IgG4-related disease. The aortic valve was replaced using Manouguian technique, resulting in a favorable outcome.
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