Modified Bex-Nikaidoh Operation in a Neonate With D-TGA, VSD, and PS

The common approach to a Bex-Nikaidoh procedure in a symptomatic neonate with dextro-transposition of the great arteries (D-TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction is initial palliation by systemic-to-pulmonary shunt, followed by complete correction at an older age (1-5). Currently there is limited experience with this complex procedure in the neonatal period. The authors present a video case report of a modified Bex-Nikaidoh operation in a neonate with D-TGA, VSD, and pulmonary stenosis (PS). 
 
Case Presentation 

A 3.2 kg newborn diagnosed with D-TGA/VSD/PS was referred for a modified Bex-Nikaidoh procedure at two weeks of age due to progressive cyanosis caused by narrowing of the left ventricular outflow tract (LVOT) to 3.5 mm, with a gradient of 65 mmHg. 
 
The Surgery 

After median sternotomy, both pulmonary artery branches were dissected and extensively mobilized. On cardiopulmonary bypass (CPB), both proximal coronary arteries were mobilized from the epicardium and looped to allow circumferential harvesting of the aortic root. On the beating heart, the aortic root was harvested from the right ventricle. The incision was started approximately 15 mm below the aortic annulus, including a large infundibular muscle flap. 

Next, the pulmonary trunk was transected, the valve was excised, and the conal septum was incised down to the ventricular septal defect. After aortic cross-clamp and cardioplegia administration, the aortic root was translocated posteriorly and reimplanted into the opened pulmonary annulus using a continuous suture. 

The ventricular septal defect was closed directly by the infundibular muscle flap using interrupted pledgeted sutures. An additional hemostatic suture line was carried out, suturing the remaining sinus wall of the pulmonary root to the aortic root. After the ascending aorta was shortened and the LeCompte maneuver was performed, the ascending aorta was reanastomosed in an end-to-end fashion. 

Through a right atriotomy, the foramen ovale was completely closed. Finally, the right ventricular outflow (RVOT) was reconstructed using a patch of decellularized equine pericardium. The posterior wall of the main pulmonary artery (MPA) was directly anastomosed to the patched RVOT, and the anterior aspect of the MPA was longitudinally incised and augmented with another patch. 
 
CBP and aortic cross-clamp times were 162 and 82 minutes, respectively. The patient was extubated on the eighth postoperative day and was discharged home on postoperative day 20. Postoperative transthoracic echocardiography showed good myocardial function, free outflow from both the left and right ventricles, and a competent aortic valve. The patient is doing well 5.8 years postoperatively. 

References

1. Morell VO, Jacobs JP, Quintessenza JA. Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up. Ann Thorac Surg. 2005 Jun;79(6):2089-92.
2. Kramer P, Ovroutski S, Hetzer R, Hübler M, Berger F. Modified Nikaidoh procedure for the correction of complex forms of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: mid-term results. Eur J Cardiothorac Surg. 2014 May;45(5):928-34.
3. Nikaidoh H. Nikaidoh procedure: a perspective. Eur J Cardiothorac Surg, 50 (2016), pp. 1001-1005. doi: 10.1093/ejcts/ezw333.
4. Ventosa-Fernández G, Pérez-Negueruela C, Mayol J, Paradela M, Caffarena-Calvar JM. The Nikaidoh procedure for complex transposition of the great arteries: short-term follow-up. Cardiol Young. 2017 Jul;27(5):945-950.
5. Stoica S, Kreuzer M, Dorobantu DM, Kostolny M, Nosal M, Hosseinpour AR, Martinez FL, Generali T, Hasan A, Mair R, Hazekamp M. Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms of transposition of the great arteries and double outlet right ventricle: A multicenter study. J Thorac Cardiovasc Surg. 2022 Nov;164(5):1249-1260. doi: 10.1016/j.jtcvs.2022.05.047.

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