Echocardiographic assessment of Ebstein's anomaly

Echocardiography. 2015 Jan:32 Suppl 2:S177-88. doi: 10.1111/echo.12486. Epub 2014 May 29.


Ebstein's anomaly is a complex congenital lesion which primarily involves the tricuspid valve. The tricuspid leaflets are tethered to varying degrees to the right ventricular free wall and the ventricular septum often resulting in significant tricuspid regurgitation and a small functioning right ventricular chamber. Although the septal leaflet originates normally at the right atrioventricular junction, the proximal portion is often completely tethered to the ventricular septum resulting in a misconception and erroneous statements in many publications that its attachment is apically displaced. Although two-dimensional echocardiography represents the primary modality for the diagnosis of this anomaly, three-dimensional echocardiography provides incremental value in characterizing the extent and severity of tethering of individual tricuspid valve leaflets. This information is useful in surgical decision making whether to repair or replace the tricuspid valve.

Keywords: Ebstein's anomaly; congenital heart disease; echocardiography; three-dimensional echocardiography.

Publication types

  • Evaluation Study
  • Review

MeSH terms

  • Ebstein Anomaly / diagnostic imaging*
  • Ebstein Anomaly / physiopathology
  • Ebstein Anomaly / surgery
  • Echocardiography / methods
  • Echocardiography, Three-Dimensional / methods*
  • Female
  • Follow-Up Studies
  • Heart Valve Prosthesis Implantation / adverse effects
  • Heart Valve Prosthesis Implantation / methods*
  • Humans
  • Male
  • Postoperative Complications / physiopathology
  • Postoperative Complications / therapy
  • Risk Assessment
  • Treatment Outcome
  • Tricuspid Valve / diagnostic imaging*
  • Tricuspid Valve / physiopathology
  • Tricuspid Valve / surgery*