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Official Description

Repair of pulmonary artery arborization anomalies by unifocalization; with cardiopulmonary bypass

© Copyright 2025 American Medical Association. All rights reserved.

Common Language Description

The procedure described by CPT® Code 33926 involves the surgical repair of pulmonary artery arborization anomalies through a technique known as unifocalization, which is performed with the assistance of cardiopulmonary bypass. Arborization anomalies are often associated with congenital heart defects, particularly pulmonary atresia and ventricular septal defects (VSD). These anomalies manifest as collateral arteries, commonly referred to as major aorto-pulmonary collateral arteries (MAPCAs), which serve as alternative pathways connecting the aorta to the pulmonary arteries in the lungs. The primary goal of the unifocalization procedure is to reconfigure these collateral arteries into a more centralized confluence, thereby improving blood flow to the lungs and enhancing oxygenation. The surgical approach typically involves either a median sternotomy or a thoracotomy, allowing access to the heart. If the procedure necessitates cardiopulmonary bypass, the aorta and both the superior and inferior vena cava are cannulated to facilitate this process. Once cardiopulmonary bypass is established, the aorta is cross-clamped to control blood flow. The surgeon then meticulously dissects the collateral arteries free from surrounding tissues and mobilizes them at their origins, which may be located in the aorta or the brachiocephalic branches. The procedure requires careful management of the pulmonary artery branches and collateral vessels, which are clamped to maintain control during the operation. Following the division of the collateral arteries from the aorta, the aortic ends are oversewn, and the distal ends of the collaterals are subsequently anastomosed to the pulmonary artery. After ensuring hemostasis at the anastomosis sites, the patient is weaned off cardiopulmonary bypass if it was utilized. The procedure concludes with the placement of chest tubes and closure of the chest incision, marking a critical step in the staged repair of complex congenital heart defects.

© Copyright 2025 Coding Ahead. All rights reserved.

1. Indications

The procedure described by CPT® Code 33926 is indicated for patients presenting with pulmonary artery arborization anomalies, which are often seen in conjunction with congenital heart defects such as pulmonary atresia and ventricular septal defects (VSD). These anomalies can lead to inadequate blood flow to the lungs, necessitating surgical intervention to improve oxygenation and overall cardiac function.

  • Pulmonary Atresia A congenital condition where the pulmonary valve does not form properly, obstructing blood flow from the heart to the lungs.
  • Ventricular Septal Defect (VSD) A defect in the septum dividing the heart's ventricles, which can lead to increased blood flow to the lungs and pulmonary hypertension.
  • Major Aorto-Pulmonary Collateral Arteries (MAPCAs) Abnormal collateral vessels that develop to supply blood to the lungs when the normal pulmonary arteries are absent or underdeveloped.

2. Procedure

The unifocalization procedure performed under CPT® Code 33926 involves several critical steps to repair pulmonary artery arborization anomalies. The surgical approach begins with either a median sternotomy or a thoracotomy, providing the necessary access to the heart. If cardiopulmonary bypass is indicated, the aorta and both the superior and inferior vena cava are cannulated to facilitate this process. Once the patient is on cardiopulmonary bypass, the aorta is cross-clamped to control blood flow during the procedure.

  • Step 1: Dissection of Collateral Arteries The surgeon carefully dissects the major aorto-pulmonary collateral arteries (MAPCAs) free from surrounding tissues, mobilizing them at their origins in the aorta or brachiocephalic branches. This step is crucial for ensuring that the collateral arteries can be properly reconfigured.
  • Step 2: Mobilization of Pulmonary Artery Segment The intraparenchymal or hilar segment of the pulmonary artery is mobilized to facilitate the connection with the collateral arteries. This mobilization is essential for achieving a successful anastomosis.
  • Step 3: Control of Branch Vessels Clamps are placed on the pulmonary artery branches and collateral vessels to maintain control during the procedure. This step helps to minimize blood loss and allows for precise surgical manipulation.
  • Step 4: Division of Collateral Arteries The collateral arteries (MAPCAs) are divided from the aorta, and the aortic ends are oversewn to prevent any potential bleeding from these sites.
  • Step 5: Anastomosis to Pulmonary Artery The distal ends of the collateral arteries are then attached to the pulmonary artery, creating a new pathway for blood flow to the lungs. This anastomosis is a critical component of the unifocalization procedure.
  • Step 6: Hemostasis and Weaning Off Bypass After the anastomosis, clamps are released, and additional sutures are placed to control any bleeding at the anastomosis sites. If cardiopulmonary bypass was used, the patient is carefully weaned off bypass to restore normal circulation.
  • Step 7: Closure Finally, chest tubes are placed to facilitate drainage, and the chest incision is closed, completing the surgical intervention.

3. Post-Procedure

Post-procedure care following the unifocalization surgery involves monitoring the patient for any complications related to the surgery and ensuring proper recovery. Patients are typically observed in a critical care setting initially, where vital signs, fluid balance, and cardiac function are closely monitored. The placement of chest tubes allows for the drainage of any excess fluid or air that may accumulate in the pleural space. Pain management is also an essential aspect of post-operative care, and patients may require medications to manage discomfort. The recovery process may vary depending on the individual patient's condition and the complexity of the surgery performed. Follow-up appointments are necessary to assess the success of the procedure and to monitor for any potential complications or need for further interventions.

Short Descr REPR PUL ART UNIFOCAL W/CPB
Medium Descr RPR P-ART ARBORIZJ ANOMAL UNIFCLIZJ W/BYPASS
Long Descr Repair of pulmonary artery arborization anomalies by unifocalization; with cardiopulmonary bypass
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 61 - Other OR procedures on vessels other than head and neck

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)
51 Multiple procedures: when multiple procedures, other than e/m services, physical medicine and rehabilitation services or provision of supplies (eg, vaccines), are performed at the same session by the same individual, the primary procedure or service may be reported as listed. the additional procedure(s) or service(s) may be identified by appending modifier 51 to the additional procedure or service code(s). note: this modifier should not be appended to designated "add-on" codes (see appendix d).
62 Two surgeons: when 2 surgeons work together as primary surgeons performing distinct part(s) of a procedure, each surgeon should report his/her distinct operative work by adding modifier 62 to the procedure code and any associated add-on code(s) for that procedure as long as both surgeons continue to work together as primary surgeons. each surgeon should report the co-surgery once using the same procedure code. if additional procedure(s) (including add-on procedure(s) are performed during the same surgical session, separate code(s) may also be reported with modifier 62 added. note: if a co-surgeon acts as an assistant in the performance of additional procedure(s), other than those reported with the modifier 62, during the same surgical session, those services may be reported using separate procedure code(s) with modifier 80 or modifier 82 added, as appropriate.
AS Physician assistant, nurse practitioner, or clinical nurse specialist services for assistant at surgery
Date
Action
Notes
2011-01-01 Changed Short description changed.
2006-01-01 Added First appearance in code book in 2006.
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