Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation

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

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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.

Original languageEnglish
Pages (from-to)1-4
Number of pages4
JournalHeart and Vessels
DOIs
Publication statusAccepted/In press - 2016 Jan 8

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Keywords

  • Endomyocardial fibrosis
  • Inflammation
  • Prognosis
  • Right ventricle thrombus

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Shiraishi, Y., Kohno, T., Fujii-Nishimura, Y., Shimoda, M., Ikeda, Y., Nakajima, K., Nishiyama, T., Nishiyama, N., Murata, M., Maekawa, Y., Sano, M., & Fukuda, K. (Accepted/In press). Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation. Heart and Vessels, 1-4. https://doi.org/10.1007/s00380-015-0792-8