**5.7. Surgical procedures**

To date there has been many procedures described for the surgical treatment for neonatal EA [11, 25–28, 31–35].

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 annuloplasty, closure of ASD and right reduction atrioplasty.

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

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.

The two competing strategies for surgical treatment for neonatal EA are whether to perform a biventricular repair or a single ventricle palliative procedure (Starnes Procedure).

The decision for which type of repair is best for neonatal EA is controversial. Mizuno et al. described their center experience with neonatal EA repair. Their results demonstrated greater survival for the biventricular repair group compared to single ventricle palliation, 60 vs. 25% respectively [30]. A recent follow up study by Kumar et al. evaluated the median 7 year follow up for the Starnes procedure in 27 neonatal repairs [29]. Their overall survival for 5 year follow up was 81%. Boston et al. described their outcomes for neonatal biventricular repair for EA. Early survival was 78.1% in their series, while 15 year survival for EA with and without anatomical pulmonary atresia was 40 and 79% respectively. As such caution should be advised for biventricular repairs in EA neonates with anatomical pulmonary atresia [25, 36].

In summary, neonatal EA continues to carry a high perinatal mortality upon fetal diagnosis. A multidisciplinary approach is required for improved outcomes. Fetal echocardiography predicts outcome and is necessary for monitoring progression of EA complications. Comprehensive care with a multi-disciplinary team including high risk obstetrician, pediatric cardiologist, pediatric cardiothoracic surgeon, neonatal intensivist should occur at a tertiary care center. Surgical management during the neonatal period remains high. If possible medical management through the neonatal period improves mortality.
