Management and prognosis of Ebstein anomaly
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
- Muhammad Yasir Qureshi, MBBS, FASE
Muhammad Yasir Qureshi, MBBS, FASE
- Senior Associate Consultant, Pediatric Cardiology
- Assistant Professor of Pediatrics
- Mayo Clinic
- Joseph Dearani, MD
Joseph Dearani, MD
- Chair, Division of Cardiovascular Surgery
- Professor of Surgery
- Mayo Clinic
- Section Editors
- Matthias Greutmann, MD, FESC
Matthias Greutmann, MD, FESC
- Head, Congenital Heart Disease Unit
- University Heart Center
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
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".)
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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- Approach to management
- Medical management
- - Pharmacologic therapy for heart failure and cyanosis
- Management of symptomatic newborn
- Management of symptomatic child
- Management of symptomatic adult
- - Prophylaxis against thromboembolism
- - Endocarditis prophylaxis
- - Exercise
- Arrhythmia treatment
- Management of pregnancy
- - Risks and outcomes
- - Approach to management
- Surgical or catheter intervention
- - Indications for surgery
- For neonates
- For children and adults
- - Procedure
- For infants
- For children and adults
- - Surgical intervention
- - Catheter intervention
- - Indications for repeat surgery after tricuspid valve repair
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