Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation

Heart Vessels. 2016 Sep;31(9):1579-82. doi: 10.1007/s00380-015-0792-8. Epub 2016 Jan 8.

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.

Keywords: Endomyocardial fibrosis; Inflammation; Prognosis; Right ventricle thrombus.

Publication types

  • Case Reports

MeSH terms

  • Autopsy
  • Chordae Tendineae / diagnostic imaging
  • Disease Progression
  • Echocardiography, Doppler, Color
  • Endomyocardial Fibrosis / complications*
  • Endomyocardial Fibrosis / diagnostic imaging
  • Endomyocardial Fibrosis / physiopathology
  • Fatal Outcome
  • Heart Failure / etiology
  • Heart Failure / physiopathology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Papillary Muscles / diagnostic imaging
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Artery / physiopathology*
  • Pulmonary Circulation*
  • Thrombosis / diagnostic imaging
  • Thrombosis / etiology*
  • Thrombosis / physiopathology
  • Tricuspid Valve* / diagnostic imaging
  • Tricuspid Valve* / physiopathology