CPT® Code 33619

1. Indications

The Norwood procedure (CPT® Code 33619) is indicated for patients diagnosed with hypoplastic left heart syndrome (HLHS), a congenital heart defect characterized by the following conditions:

• Single Ventricle: The heart has only one functional ventricle, which is insufficient for normal circulation.
• Aortic Outflow Obstruction: There is a blockage preventing blood from flowing properly from the heart to the aorta.
• Aortic Arch Hypoplasia: The aortic arch is underdeveloped, which affects blood flow to the body.

2. Procedure

The Norwood procedure involves several critical steps to repair the anatomical defects associated with hypoplastic left heart syndrome:

• Step 1: The procedure begins with a median sternotomy, which is an incision made along the sternum to access the heart. This approach allows the surgical team to fully visualize and operate on the heart.
• Step 2: Cardiopulmonary bypass is established, which temporarily takes over the function of the heart and lungs, allowing the surgical team to operate on a still and bloodless field. Hypothermic circulatory arrest may also be employed to protect the brain and other organs during the procedure.
• Step 3: The upper aspect of the heart is incised, and the atrial septum is completely excised to facilitate proper blood flow between the heart chambers.
• Step 4: The ductus arteriosus, a vessel that is normally present in fetal circulation, is ligated, divided, and completely excised, as it is no longer needed after birth.
• Step 5: The main pulmonary artery is divided proximal to the bifurcation of the pulmonary arteries, and the distal pulmonary artery stump is closed using a pericardial patch or a synthetic or cadaver donor graft.
• Step 6: The undersurface of the aortic arch is opened, starting at the ductus arteriosus and extending distally along the descending aorta until reaching a section of normal diameter and appearance.
• Step 7: The aortic incision is also extended proximally along the aortic arch to the level of the main pulmonary artery to allow for proper graft placement.
• Step 8: A cadaver donor pulmonary graft is prepared for anastomosis, which will be used to enlarge the hypoplastic aortic arch and connect the proximal aspect of the main pulmonary artery to the aorta.
• Step 9: An aortopulmonary shunt or a right ventricle to pulmonary artery conduit is created to ensure adequate lung perfusion. This shunt is constructed using a synthetic graft that connects either the innominate artery or the right ventricle to the central pulmonary artery.
• Step 10: After the surgical repairs are completed, the patient is rewarmed, and the team weans the patient off cardiopulmonary bypass.
• Step 11: Finally, chest tubes are placed to drain any excess fluid, and the chest incision is closed to complete the procedure.

3. Post-Procedure

Post-procedure care following the Norwood procedure includes monitoring the patient in a critical care setting to ensure stable hemodynamics and adequate recovery. Patients may require support for respiratory function and fluid management. The surgical team will closely observe for any complications, such as bleeding or infection, and will manage pain effectively. Follow-up care is essential to assess the patient's recovery and to plan for any additional surgical interventions that may be necessary as the child grows.