Nikaidoh Procedure for TGA, VSD, and PS in a Child

The patient is a six-year-old boy with a known diagnosis of transposition of the great arteries, a ventricular septal defect, and pulmonary stenosis who presented with severe longstanding cyanosis. The VSD was subpulmonic, and the aorta was right and anterior to the pulmonary artery. As the routability of the VSD was doubtful, the surgical team decided to go ahead with a Nikaidoh procedure. 

First, a routine standard median sternotomy was performed. A large piece of pericardium was harvested and treated with 0.6 percent glutaraldehyde for five minutes. The anatomy was confirmed. The right atrial appendage was juxtaposed to the left, the aorta was right and anterior, the coronaries were similar to routine TGA coronaries, the aortic arch was to the right, and there were bilateral SVCs. 

Next, the ascending aorta was mobilized up to the proximal arch and the branch PAs were dissected and mobilized up to the hilum. Heparin was then administered. The aorta was cannulated as high as possible. The RSVC, LSVC, and the IVC were cannulated separately. Cardiopulmonary bypass was then initiated. The LV was vented via the right upper pulmonary vein. The patient was gradually cooled to 28 degrees centigrade. During cooling, the proximal segments of the right and the left coronary arteries were mobilized using a combination of electrocautery and blunt dissection and vessel loops passed around them. The aorta was then cross-clamped and antegrade root del Nido cardioplegia was given to arrest the heart. The ascending aorta was then divided 2–3 mm distal to the sinotubular junction. The aortic valve was inspected, which was tricuspid and well coapting. The coronary ostia were also of normal size. 

Next, the aortic root was harvested. The initial incision was made 5–10 mm proximal to the aortic annulus. The incision was then extended to both the sides around the annulus passing underneath the proximal segments of the coronary arteries, taking great care to avoid any injury to the coronary arteries and the aortic valve leaflets. The area between the aortic and the pulmonary root was then carefully dissected. The aortic root was then completely separated from the right ventricle, keeping both the coronary arteries intact and leaving 5–10 mm of RV muscle underneath the aortic valve. 

The main pulmonary artery was then divided as close to the pulmonary valve as possible so as to keep the pulmonary trunk as long as possible. The bicuspid stenosed pulmonary valve was then exposed. A right-angle clamp was then passed through the pulmonary valve and the stenosed LVOT so that the tip of the clamp could be seen coming out of the VSD. The conal septum was then incised longitudinally over the right-angle clamp up to the superior margin of the VSD so as to open up the stenosed LVOT. The pulmonary valve leaflets were then excised. The aortic root was then translocated posteriorly over the opened up LVOT. The posterior suture line was completed using a 6-0 Prolene continuous suture. Nearly three-fourths of the circumference of the aortic root was sutured in place. Utmost care was taken to avoid any kinking or distortion of the coronary arteries. A second layer of reinforcement was taken by suturing the remaining sinus wall of the pulmonary root to the aortic root. This helped in achieving excellent hemostasis of the posterior suture line. 

Next, the LeCompte maneuver was then done to bring the pulmonary trunk anterior to the aorta. A short, 3–4 mm circumferential segment of the ascending aorta was excised. This shortened the ascending aorta and prevented any anterior bowing of the aorta, which can potentially compress the anteriorly located pulmonary artery. 

The distal aortic suture line was completed using a continuous 6-0 Prolene suture. A Dacron patch was used to close the VSD using a continuous 5-0 Prolene suture, taking care to avoid the conduction bundle along the posteroinferior margin. The superior margin of the patch was sutured to the remaining anterior margin of the aortic root, thereby creating a straight and wide LVOT. The RVOT was reconstructed by suturing the proximal cut end of the pulmonary trunk to the aortic root posteriorly and augmented anteriorly with the patch of treated autologous pericardium. 

The patient came off CPB with minimal inotropic support in sinus rhythm, RV pressures one-third systemic, wide open LVOT, no aortic regurgitation, and normal biventricular function. The postoperative recovery was uneventful, and the patient was doing well upon follow-up.

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