CPT® Code 33783

1. Indications

The Nikaidoh procedure is indicated for patients with specific congenital heart defects that include:

• Transposition of the Great Arteries (TGA) - A condition where the two main arteries leaving the heart are reversed, leading to inadequate oxygenation of blood.
• Ventricular Septal Defect (VSD) - A defect characterized by a hole in the wall (septum) separating the heart's lower chambers, which can lead to increased blood flow to the lungs and heart failure.
• Pulmonary Stenosis (PS) - A narrowing of the outflow tract from the right ventricle to the pulmonary artery, which can obstruct blood flow and cause right ventricular hypertrophy.

2. Procedure

The Nikaidoh procedure involves several critical steps to correct the identified congenital heart defects:

• Median Sternotomy - A surgical incision is made along the sternum to provide access to the heart and great vessels, allowing the surgeon to perform the necessary repairs.
• Harvesting of Patch Graft - A patch graft is harvested from the pericardium, which will be used later in the reconstruction of the right ventricular outflow tract.
• Cardiopulmonary Bypass - The patient is placed on cardiopulmonary bypass using bicaval cannulation, which diverts blood away from the heart and lungs, allowing the heart to be stopped for the procedure.
• Cardioplegia - Cardioplegia is initiated to induce cardiac arrest, protecting the heart muscle during the surgical intervention.
• Mobilization of Coronary Arteries - The proximal coronary arteries are mobilized to ensure they can be properly reimplanted if necessary.
• Incision of Right Ventricle - The right ventricle is incised to facilitate access to the aortic root and pulmonary artery.
• Separation of Aortic Root - The aortic root is separated from the right ventricle, allowing for the necessary translocation to correct the defects.
• Transection of Pulmonary Artery - The pulmonary artery is transected, and the infundibular septum is divided to create a pathway for the reconstruction.
• Suturing of Aortic Root - The posterior aspect of the aortic root is sutured to the pulmonary valve annulus, while the anterior aspect is sutured over the ventricular septal defect, effectively closing the defect.
• Transection and Reconfiguration of Ascending Aorta - The ascending aorta is transected, a small section is removed, and the aorta is reconfigured to prevent bowing, ensuring proper blood flow.
• LeCompte Maneuver - A LeCompte maneuver is performed to position the ascending aorta behind the pulmonary artery, optimizing the anatomical arrangement.
• Suturing of Ascending Aorta - The transected ends of the ascending aorta are sutured together to restore continuity.
• Reconstruction of Right Ventricular Outflow Tract - The anterior aspect of the hypoplastic main pulmonary artery is incised longitudinally, and the posterior aspect is sutured to the right ventricular outflow tract. A pericardial patch graft is then used to reconstruct the right ventricular outflow tract and enlarge the hypoplastic main pulmonary artery.

3. Post-Procedure

Post-procedure care following the Nikaidoh procedure involves close monitoring of the patient in a critical care setting. Patients may require support for hemodynamic stability and respiratory function as they recover from cardiopulmonary bypass. The surgical site will be monitored for signs of infection or complications. Patients typically undergo imaging studies to assess the success of the procedure and the function of the heart and great vessels. Follow-up appointments are essential to evaluate the long-term outcomes of the surgery and to manage any potential complications that may arise as the patient grows and develops.