Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy
Journal
Annals of Thoracic Surgery
Journal Volume
104
Journal Issue
3
Pages
917-925
Date Issued
2017
Abstract
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. ? 2017 The Society of Thoracic Surgeons
SDGs
Other Subjects
dopamine; epinephrine; milrinone; nitric oxide; artificial ventilation; biventricular repair; cardiopulmonary bypass; clinical article; Conference Paper; cyanosis; Ebstein anomaly; extracorporeal oxygenation; Fallot tetralogy; female; fenestration; follow up; heart right ventricle; heart right ventricle failure; heart ventricle septum defect; heart ventricle wall; human; infant; low drug dose; lung vascular resistance; medical record review; newborn; pediatric surgery; pericardial patch; priority journal; pulmonary valve stenosis; retrospective study; surgical mortality; surgical patient; surgical technique; systemic pulmonary shunt; transannular patch; tricuspid valve repair; Ebstein anomaly; heart surgery; heart ventricle; male; practice guideline; procedures; reconstructive surgery; severity of illness index; standards; time factor; treatment outcome; Cardiac Surgical Procedures; Ebstein Anomaly; Female; Follow-Up Studies; Heart Ventricles; Humans; Infant; Infant, Newborn; Male; Practice Guidelines as Topic; Reconstructive Surgical Procedures; Retrospective Studies; Severity of Illness Index; Time Factors; Treatment Outcome
Publisher
Elsevier USA
Type
conference paper