Ebstein's anomaly is rare, but it is the most frequent cause of congenital tricuspid valve anomaly. For many years valve replacement was performed. Conservative techniques are now preferred due to improvement of the results. The goals of surgery are to restore a normal tricuspid valve function, to preserve the right ventricular contractility and to decrease the risk of rhythm disturbances. Basically, the technique is based on mobilization of the anterior leaflet and longitudinal plication of the right ventricle. A bidirectional cavo pulmonary shunt is used in severe cases. Results are correlated with the severity of the disease, the expertise of the surgical team and also with the perioperative management.
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