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Published on: July 18, 2014
Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy
Shu-Chien Huang1, En-Ting Wu2, Shyh-Jye Chen3
1Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Insights
Tricuspid valvuloplasty using cone reconstruction is a viable surgical option for neonates with severe Ebstein anomaly, offering good survival and preserving biventricular repair potential.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Neonates with severe Ebstein anomaly pose significant surgical challenges.
- The Starnes operation is a common single ventricle palliation, but biventricular repair is preferred.
- Cone reconstruction for tricuspid valvuloplasty (TVP) is established, but rarely reported in neonates.
Purpose of the Study:
- To describe a surgical strategy for neonatal Ebstein anomaly aiming for biventricular repair.
- To evaluate the outcomes of TVP in neonates with severe Ebstein anomaly.
Main Methods:
- Seven neonates with severe Ebstein anomaly underwent TVP using cone reconstruction.
- Leaflets were mobilized and reattached to the tricuspid annulus; atrialized right ventricle was not plicated.
- Fenestrated atrial septal defects and systemic-pulmonary shunts were used in cases with pulmonary stenosis.
Main Results:
- All 7 patients presented with critical heart failure or cyanosis.
- Simultaneous repair of associated intracardiac anomalies was performed in 3 patients.
- Six of 7 patients (86%) survived with no late deaths or repeat TVPs at a median follow-up of 4.3 years.
Conclusions:
- Tricuspid valve reconstruction is an acceptable surgical strategy for severe neonatal Ebstein anomaly.
- Fenestrated atrial septal defects and shunts can manage pulmonary stenosis and high resistance.
- This approach offers good survival and maintains the possibility of future biventricular repair.
Background:
Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair.
Methods:
Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3).
Results:
All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years).
Conclusions:
Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
