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The Ebstein Anomaly

  • Azin AlizadeaslEmail author
  • Anita SadeghpourEmail author
  • Mohammad Mahdavi
Chapter
  • 1.2k Downloads

Abstract

The exaggerated apical displacement of the septal and posterior leaflets of the tricuspid valve (TV) in tandem with leaflet dysplasia and adherence of the tricuspid valve leaflets to the underlying right ventricle (RV) myocardium are the diagnostic features of the Ebstein anomaly. Usually, in the normal heart, there is a 10 mm distance between the mitral annulus and tricuspid annulus. In the Ebstein anomaly, the apical displacement of the septal leaflet of the TV is 8 mm/m 2 or more, associated with elongated, tethered anterior TV leaflet [1–3].

Keywords

Ebstein Anomaly Atrialization Atrial septal defect (ASD) Tricuspid valve Tricuspid regurgitation 

Supplementary material

310404_1_En_26_MOESM1_ESM.mpg (732 kb)
Video 26.1 Apical four chamber view in a typical Ebstein anomaly showing increased apical displacement of septal TV leaflet compared to the mitral leaflet insertion and tethered anterior TV leaflet. (MPG 732 kb)
310404_1_En_26_MOESM2_ESM.avi (4.4 mb)
Video 26.2 Apical four chamber view and RV inflow 2D study showing significant apical displacement of both septal and posterior TV leaflet and normal attachment of anterior TV leaflet (AVI 4,512 kb)
Video 26.3

RV injection showing huge cardiomegaly, trilobe sign (RA, Arterialized RV and functional RV) and severe TR suggestive of Ebstein anomaly (WMV 1,028 kb)

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Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Echocardiography Lab, Department of CardiologyRajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran
  2. 2.Echocardiography Lab, Department of CardiologyAdult Congenital Heart Disease Clinic, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran
  3. 3.Pediatric Ward, Department of Pediatric CardiologyRajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran

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