CPT® Code 33782

1. Indications

The Nikaidoh procedure is indicated for patients presenting 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) - An opening in the ventricular septum, which can cause mixing of oxygenated and deoxygenated blood, leading to heart failure and other complications.
• Pulmonary Stenosis (PS) - A narrowing of the outflow tract from the right ventricle to the pulmonary artery, which can obstruct blood flow and increase the workload on the heart.

2. Procedure

The Nikaidoh procedure involves several critical steps to effectively address the identified cardiac anomalies:

• Median Sternotomy - The procedure begins with a median sternotomy, which is a surgical incision made along the sternum to provide direct access to the heart and great vessels.
• Harvesting of Patch Graft - A patch graft is harvested from the pericardium, the fibrous sac surrounding the heart, to be used later in the reconstruction of the right ventricular outflow tract.
• Cardiopulmonary Bypass - The surgical team establishes cardiopulmonary bypass through bicaval cannulation, allowing for the heart to be temporarily stopped while maintaining blood circulation and oxygenation to the body.
• Initiation of Cardioplegia - Cardioplegia is initiated to induce temporary cardiac arrest, facilitating the surgical manipulation of the heart.
• Mobilization of Coronary Arteries - The proximal coronary arteries are mobilized to ensure they are adequately positioned for the subsequent steps of the procedure.
• Incision of the Right Ventricle - An incision is made in the right ventricle, allowing for the separation of the aortic root from the right ventricle.
• Transection of the Pulmonary Artery - The pulmonary artery is transected, and the infundibular septum is divided to facilitate the necessary anatomical rearrangements.
• Suturing of the 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 the Ascending Aorta - The ascending aorta is transected, a small section is removed, and the aorta is reconfigured to prevent bowing, which can lead to complications.
• LeCompte Maneuver - A LeCompte maneuver is performed to reposition the ascending aorta behind the pulmonary artery, ensuring optimal alignment and function.
• Suturing of the Ascending Aorta - The transected ends of the ascending aorta are sutured together to restore continuity.
• Reconstruction of the 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 typically require intensive care to manage hemodynamic stability and monitor for any potential complications such as bleeding, infection, or arrhythmias. The recovery period may vary depending on the patient's overall health and the complexity of the surgery. Follow-up evaluations are essential to assess the function of the heart and the success of the surgical intervention. Long-term management may include regular cardiac assessments and monitoring for any residual or new cardiac issues.