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01-09-2016 | Case Report

Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation

Authors: Yasuyuki Shiraishi, Takashi Kohno, Yoko Fujii-Nishimura, Masayuki Shimoda, Yoshihiko Ikeda, Kazuaki Nakajima, Takahiko Nishiyama, Nobuhiro Nishiyama, Mitsushige Murata, Yuichiro Maekawa, Motoaki Sano, Keiichi Fukuda

Published in: Heart and Vessels | Issue 9/2016

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.

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Title: Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation
Authors: Yasuyuki Shiraishi
Takashi Kohno
Yoko Fujii-Nishimura
Masayuki Shimoda
Yoshihiko Ikeda
Kazuaki Nakajima
Takahiko Nishiyama
Nobuhiro Nishiyama
Mitsushige Murata
Yuichiro Maekawa
Motoaki Sano
Keiichi Fukuda
Publication date: 01-09-2016
Publisher: Springer Japan
Published in: Heart and Vessels / Issue 9/2016
Print ISSN: 0910-8327
Electronic ISSN: 1615-2573
DOI: https://doi.org/10.1007/s00380-015-0792-8

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