Chapter 13 - Ebstein Anomaly, Palliated

Summary

Ebstein’s anomaly is a rare and highly variable congenital abnormality affecting the tricuspid valve and adjacent right ventricular myocardium. Depending on the degree of apical tricuspid valve displacement, there is loss of effective right ventricular volume and size, with “atrialization” of the right ventricle and right ventricular outflow tract obstruction limiting functional ejection and resulting in decreased pulmonary blood flow. Conduction system abnormalities may also be present in children with Ebstein’s anomaly. Initial management of the newborn with severe Ebstein’s anomaly is focused on maintaining effective pulmonary blood flow and oxygen saturation. Longer term surgical decision making directs the patient toward either a single-ventricle palliation pathway or a two-ventricle repair based on the size and function of the right ventricle. When the size and function of the tricuspid valve and right ventricle are adequate, a two-ventricle repair may be performed. This chapter is focused on the perioperative management of a patient who has undergone staged single-ventricle palliation for Ebstein’s anomaly.