title: Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation.
abstract: Endomyocardial fibrosis (EMF) is a progressive type of obliterative/restrictive cardiomyopathy characterized by fibrosis of the apical endomyocardium of the ventricles. Although the prognosis of EMF patients is poor in tropical regions, the exact clinical course and pathogenesis of patients with EMF are not known. Here, we report the rare case with EMF in the seventh decade, who showed the disappearance of papillary muscles, chordae tendineae, and part of the tricuspid valves due to massive right ventricular thrombus. Because of those unusual findings of the isolated RV involvement, we observed continuous forward pulmonary artery flow, as occurring in a Fontan circulation.
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Shiraishi
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan. kohno.a2@keio.jp.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kohno
authors.affiliation: Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Fujii-Nishimura
authors.affiliation: Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Shimoda
authors.affiliation: Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan.
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Ikeda
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Nakajima
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Takahiko
authors.initials: T
authors.lastname: Nishiyama
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Nishiyama
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Mitsushige
authors.initials: M
authors.lastname: Murata
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Maekawa
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Motoaki
authors.initials: M
authors.lastname: Sano
authors.affiliation: Division of Cardiology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Fukuda
mesh_terms: D001344:Autopsy; D002815:Chordae Tendineae / Q000000981:diagnostic imaging; D018450:Disease Progression; D018618:Echocardiography, Doppler, Color; D004719:Endomyocardial Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010210:Papillary Muscles / Q000000981:diagnostic imaging; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011652:Pulmonary Circulation; D013927:Thrombosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D014261:Tricuspid Valve / Q000000981:diagnostic imaging / Q000503:physiopathology
keywords: Endomyocardial fibrosis; Inflammation; Prognosis; Right ventricle thrombus