Ebstein's Anomaly

Methodist Debakey Cardiovasc J. 2019 Apr-Jun;15(2):138-144. doi: 10.14797/mdcj-15-2-138.


Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.

Keywords: Ebstein's anomaly; adult congenital heart disease; arrhythmia; tricuspid valve.

Publication types

  • Review

MeSH terms

  • Cardiac Surgical Procedures* / adverse effects
  • Cardiovascular Agents / adverse effects
  • Cardiovascular Agents / therapeutic use*
  • Ebstein Anomaly / complications
  • Ebstein Anomaly / diagnostic imaging
  • Ebstein Anomaly / physiopathology
  • Ebstein Anomaly / therapy*
  • Hemodynamics / drug effects
  • Humans
  • Recovery of Function
  • Treatment Outcome
  • Tricuspid Valve / abnormalities
  • Tricuspid Valve / drug effects*
  • Tricuspid Valve / physiopathology
  • Tricuspid Valve / surgery*
  • Tricuspid Valve Insufficiency / drug therapy
  • Tricuspid Valve Insufficiency / etiology
  • Tricuspid Valve Insufficiency / physiopathology
  • Tricuspid Valve Insufficiency / therapy*
  • Ventricular Function, Right / drug effects


  • Cardiovascular Agents