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Chapter 12 - Ebstein Anomaly

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 heart defect affecting the tricuspid valve and right ventricle. The tricuspid valve is dysplastic, with downward displacement of the septal and posterior leaflets inferiorly into the right ventricle. While the mildest forms of Ebstein’s anomaly include minimal tricuspid regurgitation and tricuspid valve displacement, more severely affected patients will have greater tricuspid valve displacement with resultant loss of right ventricular volume and right ventricular dysfunction. An atrial level communication, either an atrial septal defect or patent foramen ovale, also exists in nearly all patients. In more severely affected patients right-to-left shunting at the atrial level results in varying degrees of cyanosis. This chapter discusses the considerations involved in the perioperative care and management of a patient with mild right ventricular dysfunction and tricuspid regurgitation undergoing urgent noncardiac surgery.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 70 - 74
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Jaquiss, R. D. and Imamura, M. Management of Ebstein’s anomaly and pure tricuspid insufficiency in the neonate. Semin Thorac Cardiovasc Surg 2007; 19: 258–63.CrossRefGoogle ScholarPubMed
Morray, B. Preoperative physiology, imaging, and management of Ebstein’s anomaly of the tricuspid valve. Semin Cardiothorac Vasc Anesth 2016; 20: 7481.CrossRefGoogle ScholarPubMed
Ross, F. J., Latham, G. J., Richards, M., et al. Perioperative and anesthetic considerations in Ebstein’s anomaly. Semin Cardiothorac Vasc Anesth 2016; 20: 8292.CrossRefGoogle ScholarPubMed
Rutz, T. and Kühn, A. The challenge of risk stratification in Ebstein’s anomaly. Int J Cardiol 2019; 278: 8990.Google Scholar

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