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Official Description

Repair of pulmonary atresia with ventricular septal defect, by construction or replacement of conduit from right or left ventricle to pulmonary artery

© Copyright 2026 American Medical Association. All rights reserved.

Common Language Description

Repair of pulmonary atresia with ventricular septal defect (VSD) is a complex surgical procedure aimed at correcting a congenital heart defect where the pulmonary valve does not develop properly, leading to an obstruction of blood flow from the right ventricle to the lungs. In this condition, the heart's ability to deliver oxygenated blood to the body is compromised. The presence of a VSD, which is an abnormal opening in the septum separating the right and left ventricles, further complicates the situation by allowing blood to flow between the two ventricles. This procedure is specifically designed for patients who exhibit both pulmonary atresia and a VSD, and it requires careful planning and execution due to the variability in the anatomical presentation of each patient. The surgical approach is often tailored to the individual, and the repair may be performed in stages, depending on the severity of the defects and the overall health of the patient. The procedure involves accessing the heart through a median sternotomy, harvesting a patch of pericardium for grafting, and establishing cardiopulmonary bypass to facilitate the repair. The surgeon evaluates the anatomy of the pulmonary artery and its branches to determine the best site for constructing or replacing a conduit that will allow blood to flow from the ventricle to the pulmonary artery, thereby restoring proper circulation to the lungs.

© Copyright 2026 Coding Ahead. All rights reserved.

1. Indications

The procedure is indicated for patients diagnosed with:

  • Pulmonary Atresia - A congenital heart defect characterized by the improper development of the pulmonary valve, which obstructs blood flow from the right ventricle to the pulmonary artery.
  • Ventricular Septal Defect (VSD) - An opening in the septum between the right and left ventricles that can lead to abnormal blood flow and increased pressure in the heart.

2. Procedure

The surgical procedure involves several critical steps to ensure the successful repair of pulmonary atresia with VSD:

  • Step 1: Exposure of the Heart - The procedure begins with a median sternotomy, which involves making an incision along the sternum to gain access to the heart. The pericardium, the protective sac surrounding the heart, is then incised, and a patch of pericardium is harvested for later use as a patch graft.
  • Step 2: Establishing Cardiopulmonary Bypass - Cardiopulmonary bypass is initiated to take over the function of the heart and lungs during the surgery, allowing the surgeon to operate on a still and bloodless field.
  • Step 3: Evaluation of the Pulmonary Anatomy - The main pulmonary artery and its branches, along with the external anatomy of the heart, are carefully evaluated to identify the optimal location for the conduit that will be constructed or replaced.
  • Step 4: Dissection of the Pulmonary Arteries - The main pulmonary artery is dissected free from surrounding tissues, followed by the dissection of the right and left pulmonary arteries. Rubber loops are placed around these vessels to gently occlude them, controlling blood flow during the procedure.
  • Step 5: Construction of the Conduit - A longitudinal incision is made in the main pulmonary artery. A conduit is then constructed using autologous pericardium, an allograft (homograft), xenograft, or synthetic material. This conduit is sutured to the arteriotomy site in the main pulmonary artery.
  • Step 6: Insertion of the Conduit - The incision is extended into the right ventricle, and the conduit is inserted into the right ventricle, where it is secured with sutures. Depending on the patient's unique anatomy, the conduit may alternatively be attached to the left ventricle.
  • Step 7: VSD Repair (if applicable) - If the VSD is repaired during the same surgical session, it is reported separately, ensuring that all aspects of the patient's condition are addressed.
  • Step 8: Weaning Off Cardiopulmonary Bypass - After the repairs are completed, the patient is gradually weaned off cardiopulmonary bypass. Chest tubes are placed as needed to drain any excess fluid or blood, and the chest incision is then closed.

3. Post-Procedure

Post-procedure care involves monitoring the patient for any complications and ensuring proper recovery. Patients may require intensive care following the surgery to manage their recovery effectively. The placement of chest tubes is common to facilitate drainage and prevent fluid accumulation. The healthcare team will monitor vital signs, cardiac function, and overall recovery progress. Follow-up appointments will be necessary to assess the success of the repair and to plan any further interventions if needed.

Short Descr REPAIR PULMONARY ATRESIA
Medium Descr RPR PULMONARY ATRESIA W/CONSTJ/RPLCMT CONDUIT
Long Descr Repair of pulmonary atresia with ventricular septal defect, by construction or replacement of conduit from right or left ventricle to pulmonary artery
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 43 - Heart valve 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)
Date
Action
Notes
1994-01-01 Added First appearance in code book in 1994.
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