Current surgical techniques in the management of the symptomatic neonate with severe Ebstein anomaly: Too much, too little, or just enough?

Abstract

The management of severely symptomatic neonates with Ebstein anomaly is challenging during the early neonatal period. Initial management goals should focus on mitigating a central shunt; providing respiratory mechanical support; providing an adequate but not excessive source of pulmonary blood flow; and minimizing pulmonary vascular resistance. For most patients thus stabilized, definitive repair should be prudently deferred until it is safe for a bailout bidirectional Glenn anastomosis to be added, usually at age 3 to 4 months. For those who remain critical, initial ligation of the large ductus and placing a more peripheral aortopulmonary shunt, or ligating the main pulmonary artery, should be weighed against a primary biventricular repair (Knott-Craig repair), or the Starnes' single-ventricle palliation. The Da Silva cone biventricular repair should generally be avoided during the early neonatal period. An initial Starnes' repair can be potentially converted to a biventricular repair in later infancy.

Keywords: Ebstein anomaly; surgical management; symptomatic neonates.

Christopher J. Knott-Craig, MD, FACS, and Umar S. Boston, MD

Figure 1

Chest radiograph demonstrating severe cardiomegaly in a newborn infant with Ebstein anomaly.

Figure 2

Sebening suture is demonstrated. A suture is passed from the dominant papillary muscle attached to the anterior leaflet and fixed to a point on the ventricular septum directly opposite to move the anterior leaflet to the septum, allowing for coaptation with the septum and/or posterior leaflet.

Figure 3

A and B, Starnes' single ventricle palliation. B-T shunt, Blalock-Taussig shunt.

Figure 4

A and B, Knott-Craig monocusp repair based on the anterior leaflet. A double orifice tricuspid valve is created by passing a suture through the annulus at the anteroposterior commissure and then through the septal annulus adjacent to the coronary sinus. The posterior annulus and right ventricle (RV) is then plicated.

Figure 5

Da Silva cone operation. TTA, True tricuspid annulus; CS, coronary sinus; ASD, atrial septal defect.

Figure 6

Algorithmic approach to the severely symptomatic neonate with Ebstein anomaly. PBF, Pulmonary blood flow; PA, pulmonary atresia; CCF, congestive cardiac failure; BT, Blalock-Taussig; BDG, Bidirectional GLenn; RV, right ventricle; TR, tricuspid regurgitation; PDA, patent ductus arteriosus; MPA, main pulomonary artery.