**Author details**

Milrinone is a very effective drug since it has lusitropic and inotropic effects on the right ventricle. Furthermore, it decreases the pulmonary vascular resistance which promotes antegrade pulmonary blood flow. Frequent echocardiograms during the first week are useful to assess antegrade flow across the RV outflow tract and degree of TV regurgitation. This assessment will help guide weaning prostaglandins, and initiation of nitric

In summary, medical management when pulmonary ductal dependency exists is analogous to single ventricle physiology whereby a balance between systemic and pulmonary circulation needs to be established. This is best done with maintenance of prostaglandins and low oxygen supplementation. Once the pulmonary vascular resistance drops and there is antegrade flow across the pulmonary valve then this management is more analogous to two ventricle physiology with a poor right ventricular pump. As such this is best managed by stopping prostaglandins and allowing for ductal closure. Concomitantly administration of nitric oxide,

The goal of medical therapy is to avoid an operation particularly during the neonatal period

Surgical indications for EA include failure to wean from mechanical ventilator support, failure to wean off prostaglandin with systemic oxygen saturation below 75%, functional or anatomic pulmonary atresia, Great Ormond Street Echocardiography (G.O.S.E.) score of 3 or 4,

To date there has been many procedures described for the surgical treatment for neonatal EA

Danielson at the Mayo Clinic first described some of the essential principles for EA repair in any age group [25]. This includes plication of the atrialized RV, posterior tricuspid annulo-

The cone reconstruction first described by da Silva and colleagues has now evolved into the technique of choice when repairing the tricuspid valve for EA [27]. In this procedure, the anterior and posterior leaflets of the tricuspid valve are mobilized from their attachments to the RV endocardium maintaining free edge attachments. The mobilized leaflets are rotated clockwise and then reattached to the true annulus [36]. To date there has been a growing number

Starnes et al. reported a single ventricle palliation strategy for neonates with good outcome [29]. In the Starnes procedure the RV is excluded by performing a fenestrated patch closure of the tricuspid valve. An atrial septectomy allows for excellent mixing. Finally, a modified Blalock-Taussig-Thomas shunt is performed to establish a regulated source of pulmonary blood flow. Most neonates undergoing a Starnes RV exclusion procedure are then channeled

down a single ventricle pathway with a bidirectional Glenn and Fontan procedures.

milrinone and higher oxygen supplementation will augment antegrade flow.

when mortality is highest for any surgical procedure performed.

oxide and inotropes.

158 Congenital Anomalies - From the Embryo to the Neonate

**5.6. Surgical indications**

**5.7. Surgical procedures**

[11, 25–28, 31–35].

and patients with right heart failure.

plasty, closure of ASD and right reduction atrioplasty.

of reports of utilization of the Cone procedure for neonatal EA [27].

Umar Boston\*, Ken-Michael Bayle, TK Susheel Kumar and Christopher Knott-Craig

\*Address all correspondence to: uboston@uthsc.edu

Heart Institute at Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis, United States
