A Timely Evaluation Enables Precise Planning and Surgical Readiness

Major aortopulmonary collateral arteries (MAPCAs) typically present early in life, either in utero or shortly after birth. Timely referral and comprehensive specialty evaluation allow teams to optimize pulmonary vascular development and clinical status before definitive repair. Delayed referral either results in pulmonary over circulation and failure to thrive, or critical cyanosis if there is limited pulmonary blood flow.

“Many of these patients will require catheter-based interventions before undergoing complete repair at around 8 to 10 months of age,” said Marco Ricci, MD, MBA, pediatric cardiovascular surgeon. “An early referral ensures we have time beforehand to educate the family, optimize the child’s pulmonary vascular structures and plan for the most successful outcome possible.”

A Structured Pathway Streamlines Evaluation

Ricci and several colleagues recently launched a MAPCAs program at the Cincinnati Children’s Heart Institute. After referral, the team follows a defined process:

• Collect and review prior medical records and imaging.
• Identify gaps in diagnostic information.
• Complete a comprehensive evaluation that may include advanced imaging (often within a single visit or short stay).
• Develop a coordinated treatment plan through team-based review.

Completing this evaluation in a single visit or short stay is especially important for medically fragile infants, allowing teams to make timely decisions while minimizing delays and the need for repeat testing.

Advanced Imaging and 3D Modeling Define Anatomy

Advanced imaging, including CT and 3D modeling, allows for detailed, patient-specific anatomic definition prior to intervention.

“These tools provide a three-dimensional road map of the native pulmonary arteries and major aortopulmonary collateral arteries, as well as show their relationship with the airway and other structures,” said pediatric cardiologist Sean Lang, MD. “With advancements in high-quality 3D cross-sectional imaging and computer-aided design tools, we’ve been able to enhance the way we interact with 3D datasets.”

Lang adds that the use of augmented and virtual reality platforms provides teams with an opportunity to plan the best intervention strategy for the patient before the procedure. The imaging has also been shown to reduce cardiac catheterization time and limit contrast use for patients.

Multidisciplinary Case Review Guides Decision-Making

Multidisciplinary decision-making is essential. The MAPCAs program brings together specialists in:

• Cardiology (imaging and interventional)
• Cardiothoracic surgery
• Intensive care
• Imaging and 3D modeling

The team reviews imaging and 3D models together—often in real time—and develops an individualized treatment plan for each patient.

“The complexity and variable anatomy of patients with MAPCAs is significant,” explained Lang. “In order to best care for these patients, a teamwise approach of specialists in imaging, interventional cardiology, cardiothoracic surgery and intensive care are vital. We leverage the knowledge and experience of each of these specialists to make the best treatment decisions for each patient.”

Management Extends Beyond Initial Repair

MAPCAs care spans multiple phases, beginning with preoperative optimization in early infancy and continuing through surgical repair, postoperative ICU care and long-term follow-up. Even after reconstruction, patients often need ongoing surveillance and catheter-based interventions to address pulmonary artery stenosis as they grow.

“Transcatheter rehabilitation of the pulmonary arteries is an important component of what we do,” Ricci said. “Even after successful repair, those reconstructed arteries can become narrow over time, so many children require additional catheter-based interventions. It’s not only about what we do before or during surgery. It’s also about how we continue to manage these patients over time.”

Interventional cardiologist Shabana Shahanvaz, MD, MBBS, director of the Cardiac Catheterization Laboratory at the Heart Institute, added that catheter-based care follows a deliberate, staged strategy tailored to each child’s anatomy and development.

“First, we focus on true native pulmonary artery growth whenever feasible at the earliest stage, establishing a more durable and physiologic foundation for repair,” Shahanavaz said. “We then thoughtfully rehabilitate the MAPCAs over time, using targeted catheter-based interventions to optimize pulmonary blood flow as the child grows. This longitudinal, collaborative approach not only addresses the immediate anatomic challenges, but also positions these patients for more stable, sustainable outcomes in the years ahead.”

Collaboration Keeps Referring Physicians Involved

To keep referring cardiologists engaged throughout care, the team:

• Shares evaluation findings and treatment plans
• Invites referring physicians to multidisciplinary conferences (virtual)
• Coordinates follow-up and reintervention planning

This model supports co-management and maintains continuity as patients transition between Cincinnati Children’s providers and local care.

Learn more about referring a patient.

(Published May 2026)

Read More from the Heart Institute