title: Immunoglobulin G4-related disease of the heart causing aortic regurgitation and heart block.
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.
authors.affiliation: Division of Cardiovascular Surgery, Hokkaido PWFAC (Prefectural Welfare Federation of Agricultural Cooperative) Obihiro-Kosei General Hospital, Obihiro, Hokkaido, Japan. okh-i14byam@obihirokousei-hp.jp
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Yamauchi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Satoh
authors.forename: Tomoyoshi
authors.initials: T
authors.lastname: Yamashita
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Shinshi
authors.forename: Keisuke
authors.initials: K
authors.lastname: Kikuchi
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Sasaki
authors.forename: Yoshiro
authors.initials: Y
authors.lastname: Matsui
mesh_terms: D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006327:Heart Block / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000379:methods*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000175:diagnosis; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D008875:Middle Aged; D012086:Reoperation / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods
chemical_list: D007074:Immunoglobulin G