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Narrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives
Stephan Neumann1, André Rüffer2, Jörg Sachweh2
1Department of Pediatric Cardiology, University Heart & Vascular Center Hamburg, Hamburg, Germany.
Insights
Ebstein's anomaly, a rare congenital heart defect, involves tricuspid valve malformation and right ventricular myopathy. Early diagnosis and surgical intervention, like cone reconstruction, improve outcomes, though reoperation remains a concern.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Ebstein's anomaly is a rare congenital heart disease characterized by tricuspid valve malformation and right ventricular myopathy.
- Failure of leaflet delamination leads to apical displacement, tricuspid regurgitation, and potential right ventricular dilation.
Purpose of the Study:
- To provide a comprehensive review of Ebstein's anomaly management.
- Focus on imaging, cardiac surgery, outcomes, and arrhythmias.
Main Methods:
- Review of current literature on Ebstein's anomaly.
- Focus on diagnostic modalities (echocardiography, MRI).
- Analysis of surgical interventions and outcomes.
Main Results:
- Variable anatomy dictates clinical presentation and management.
- Medical management is effective for asymptomatic patients; surgery indicated for symptoms or RV dysfunction.
- Modified cone reconstruction is the current state-of-the-art surgical approach.
Conclusions:
- Long-term survival is favorable post-surgery, but rehospitalization and reoperation are significant issues.
- Optimal surgical strategy and timing require further research to prevent adverse ventricular remodeling.
- Comprehensive management involves imaging, timely surgery, and addressing arrhythmias.
Abstract:
Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.
