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Chapter 13 - Ebstein Anomaly, Palliated

from Section 2 - Right-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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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.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 75 - 82
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Holst, K. A., Dearani, J. A., Said, S. M., et al. Surgical management and outcomes of Ebstein anomaly in neonates and infants: a Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis. Ann Thorac Surg 2018; 106: 785–91.Google Scholar
Kumar, S. R., Kung, G., Noh, N., et al. Single-ventricle outcomes after neonatal palliation of severe Ebstein anomaly with modified Starnes procedure. Circulation 2016; 134: 1257–64.Google Scholar
Kumar, T. K. S., Boston, U. S., and Knott-Craig, C. J. Neonatal Ebstein anomaly. Semin Thorac Cardiovasc Surg 2017; 29: 331–7.Google Scholar
Leyvi, G. and Wasnick, J. D. Single-ventricle patient: pathophysiology and anesthetic management. J Cardiothorac Vasc Anesth 2010; 24: 121–30.Google Scholar
Luxford, J. C., Arora, N., Ayer, J. G., et al. Neonatal Ebstein anomaly: a 30-year institutional review. Semin Thorac Cardiovasc Surg 2017; 29: 206–12.Google Scholar

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