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Ebstein's anomaly: contemporary management strategies
Sandeep Sainathan1, Luciana da Fonseca da Silva2, Jose Pedro da Silva2
1Department of Surgery, University of Tennessee Health Sciences Center, Memphis, TN, USA.
Journal of Thoracic Disease
|April 11, 2020
Summary
Ebstein's anomaly (EA) is a rare congenital heart defect affecting the tricuspid valve and right ventricle. Surgical repair, particularly the da Silva Cone valvuloplasty, offers good long-term outcomes for many patients, with heart transplantation reserved for severe cases.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiac Surgery
Background:
- Ebstein's anomaly (EA) is a rare congenital cardiac malformation involving the tricuspid valve and right ventricular myocardium.
- Clinical presentation of EA exhibits wide variability, ranging from severe neonatal symptoms to incidental findings in later life.
Purpose of the Study:
- To review the management strategies for Ebstein's anomaly, focusing on surgical interventions and long-term outcomes.
- To highlight the efficacy of the da Silva Cone repair for tricuspid valve valvuloplasty in Ebstein's anomaly.
Main Methods:
- Review of current medical and surgical management options for Ebstein's anomaly.
- Discussion of various surgical approaches including palliative single ventricle pathways, biventricular repair, tricuspid valve valvuloplasty, and valve replacement.
- Emphasis on the da Silva Cone repair as a preferred valvuloplasty technique.
Main Results:
- Neonatal EA requires prompt medical management, with surgical intervention (biventricular repair or palliative approach) indicated for non-responders.
- The da Silva Cone repair demonstrates good long-term durability for valvuloplasty in EA, applicable across diverse morphologies.
- Early valvuloplasty is recommended to prevent cardiac dilation; Bidirectional Glenn may aid marginal cases; bioprosthetic valves are preferred over mechanical for replacement.
Conclusions:
- Surgical management of Ebstein's anomaly should be tailored to individual patient presentation and valve morphology.
- The da Silva Cone repair is a durable and effective option for tricuspid valve repair in Ebstein's anomaly, especially beyond the neonatal period.
- Heart transplantation is a consideration for patients with significant left ventricular dysfunction secondary to Ebstein's anomaly.
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