CPT® Code 33697

Official Description

Complete repair tetralogy of Fallot with pulmonary atresia including construction of conduit from right ventricle to pulmonary artery and closure of ventricular septal defect

Common Language Description

The procedure described by CPT® Code 33697 involves a complete surgical repair of tetralogy of Fallot (TOF) in neonates, specifically when accompanied by pulmonary atresia. Tetralogy of Fallot is a complex congenital heart defect characterized by four primary abnormalities: pulmonary stenosis, which is a narrowing that obstructs blood flow from the right ventricle to the pulmonary artery; right ventricular hypertrophy, which is the thickening of the right ventricular walls due to increased pressure; ventricular septal defect (VSD), which is a hole in the wall separating the heart's lower chambers; and overriding aorta, where the aorta is positioned directly over the VSD, allowing unoxygenated blood to mix with oxygenated blood. In cases where pulmonary atresia is present, there is a complete blockage of blood flow from the right ventricle to the pulmonary artery, necessitating the construction of a conduit to facilitate blood flow. The surgical approach typically involves a median sternotomy to access the heart, followed by the establishment of cardiopulmonary bypass and cardioplegic arrest to protect the heart during the procedure. The VSD is repaired using a synthetic patch, and the pulmonary valve is addressed by resecting obstructive tissue, potentially utilizing a transannular patch to enlarge the outflow tract. The construction of a conduit from the right ventricle to the pulmonary artery is critical in cases of pulmonary atresia, ensuring that blood can flow to the lungs for oxygenation. This comprehensive repair aims to correct the anatomical defects and restore normal blood flow, significantly improving the neonate's prognosis.

1. Indications

The procedure is indicated for neonates diagnosed with tetralogy of Fallot accompanied by pulmonary atresia. The specific conditions warranting this surgical intervention include:

Tetralogy of Fallot (TOF) - A congenital heart defect characterized by a combination of four related heart defects: pulmonary stenosis, right ventricular hypertrophy, ventricular septal defect (VSD), and overriding aorta.
Pulmonary Atresia - A condition where there is a complete obstruction of blood flow from the right ventricle to the pulmonary artery, necessitating surgical intervention to establish a conduit for blood flow.

2. Procedure

The surgical procedure for CPT® Code 33697 involves several critical steps to repair the heart defects associated with tetralogy of Fallot and pulmonary atresia:

Step 1: Median Sternotomy - The procedure begins with a median sternotomy, which is an incision made along the sternum to provide access to the heart. This approach allows the surgeon to visualize and operate on the heart effectively.
Step 2: Establishing Cardiopulmonary Bypass - Once access to the heart is achieved, cardiopulmonary bypass is established. This technique temporarily takes over the function of the heart and lungs, allowing the surgeon to operate on a still and bloodless field. Cardioplegic arrest is then initiated to protect the heart muscle during the procedure.
Step 3: Repairing the Ventricular Septal Defect (VSD) - The VSD is repaired using a synthetic patch. This step is crucial as it prevents the mixing of unoxygenated blood from the right ventricle with oxygenated blood from the left ventricle, thereby correcting the defect associated with the overriding aorta.
Step 4: Addressing Pulmonary Stenosis - The narrowed pulmonary valve is treated by resecting obstructive tissue in the right ventricle. If necessary, a transannular patch may be placed to enlarge the outflow tract, facilitating improved blood flow from the right ventricle to the pulmonary artery.
Step 5: Constructing the Conduit - In cases of pulmonary atresia, a conduit is constructed from the right ventricle to the pulmonary artery. This involves incising the front surface of the right ventricle while carefully avoiding the coronary artery. One end of a synthetic tube is sutured to the right ventricle, and the other end is sutured at the bifurcation of the pulmonary arteries, creating a pathway for blood to flow to the lungs.

3. Post-Procedure

Post-procedure care involves close monitoring of the neonate in a critical care setting to assess heart function and recovery. The expected recovery includes observation for any complications related to the surgery, such as bleeding or infection. The neonate may require additional support for respiratory function as they adjust to the new blood flow dynamics. Follow-up evaluations will be necessary to ensure the success of the repair and to monitor for any potential long-term complications associated with tetralogy of Fallot and pulmonary atresia.

Short Descr	COMPL RPR TOF W/PULM ATRESIA
Medium Descr	COMPLETE REPAIR TOF W/PULMONARY ATRESIA
Long Descr	Complete repair tetralogy of Fallot with pulmonary atresia including construction of conduit from right ventricle to pulmonary artery and closure of ventricular septal defect
Status Code	Active Code
Global Days	090 - Major Surgery
PC/TC Indicator (26, TC)	0 - Physician Service Code
Multiple Procedures (51)	2 - Standard payment adjustment rules for multiple procedures apply.
Bilateral Surgery (50)	0 - 150% payment adjustment for bilateral procedures does NOT apply.
Physician Supervisions	09 - Concept does not apply.
Assistant Surgeon (80, 82)	2 - Payment restriction for assistants at surgery does not apply to this procedure...
Co-Surgeons (62)	1 - Co-surgeons could be paid, though supporting documentation is required...
Team Surgery (66)	0 - Team surgeons not permitted for this procedure.
Diagnostic Imaging Family	99 - Concept Does Not Apply
APC Status Indicator	Inpatient Procedures, not paid under OPPS
Type of Service (TOS)	2 - Surgery
Berenson-Eggers TOS (BETOS)	P2F - Major procedure, cardiovascular-Other
MUE	1
CCS Clinical Classification	49 - Other OR heart procedures

This is a primary code that can be used with these additional add-on codes.

33257	Addon Code MPFS Status: Active Code APC C Illustration for Code Operative tissue ablation and reconstruction of atria, performed at the time of other cardiac procedure(s), limited (eg, modified maze procedure) (List separately in addition to code for primary procedure)
33259	Addon Code MPFS Status: Active Code APC C Illustration for Code Operative tissue ablation and reconstruction of atria, performed at the time of other cardiac procedure(s), extensive (eg, maze procedure), with cardiopulmonary bypass (List separately in addition to code for primary procedure)
33924	Addon Code MPFS Status: Active Code APC C CPT Assistant Article Ligation and takedown of a systemic-to-pulmonary artery shunt, performed in conjunction with a congenital heart procedure (List separately in addition to code for primary procedure)
34714	Addon Code MPFS Status: Active Code APC N ASC N1 Open femoral artery exposure with creation of conduit for delivery of endovascular prosthesis or for establishment of cardiopulmonary bypass, by groin incision, unilateral (List separately in addition to code for primary procedure)
34716	Addon Code MPFS Status: Active Code APC N ASC N1 Open axillary/subclavian artery exposure with creation of conduit for delivery of endovascular prosthesis or for establishment of cardiopulmonary bypass, by infraclavicular or supraclavicular incision, unilateral (List separately in addition to code for primary procedure)
34833	Addon Code Resequenced Code MPFS Status: Active Code APC C CPT Assistant Article Open iliac artery exposure with creation of conduit for delivery of endovascular prosthesis or for establishment of cardiopulmonary bypass, by abdominal or retroperitoneal incision, unilateral (List separately in addition to code for primary procedure)