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Management and prognosis of Ebstein anomaly

Authors
Heidi M Connolly, MD, FASE
Muhammad Yasir Qureshi, MBBS, FASE
Joseph Dearani, MD
Section Editors
Matthias Greutmann, MD, FESC
David R Fulton, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

Ebstein anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle (figure 1). The clinical presentation of Ebstein anomaly varies widely, ranging from symptomatic neonate to the asymptomatic adult, depending upon the degree of anatomic abnormality and concomitant lesions (eg, atrial septal defect). The management and prognosis of Ebstein anomaly are discussed here.

The clinical manifestations and diagnosis of Ebstein anomaly are discussed separately. (See "Clinical manifestations and diagnosis of Ebstein anomaly".)

MANAGEMENT

Approach to management — The approach to management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention.

All patients with Ebstein anomaly should undergo periodic monitoring. (See 'Monitoring' below.)

For the newborn with Ebstein anomaly presenting with cyanosis or heart failure, medical management with advanced neonatal care is preferred to delay surgery as long as possible except when an indication for surgery during the neonatal period is present. (See 'Management of symptomatic newborn' below and 'For neonates' below.)

                             
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Literature review current through: Nov 2017. | This topic last updated: Feb 06, 2017.
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