Pediatric Pulmonary Artery Reconstruction (PAR) Program

Lurie Children’s is one of the few pediatric hospitals in the nation with a dedicated program for Pulmonary Artery Reconstruction. Our Heart Center is nationally ranked and recognized for its excellence in treating complex congenital heart disease and for other distinctive strengths that set us apart:

• Highly Specialized Expertise: Our surgeons have extensive experience performing complex pulmonary artery reconstruction procedures, including unifocalization—a surgery that connects multiple abnormal blood vessels (called MAPCAs), which form when normal pulmonary arteries are missing or blocked, into one central pulmonary artery system.
• Comprehensive, Multidisciplinary Team Care: Each patient is cared for by a team of leading specialists—including pediatric cardiologists, advanced cardiac imaging experts, otolaryngologists, anesthesiologists, and critical care providers—who work together to provide personalized treatment based on the child’s unique anatomy, health, and condition.
• Innovative Research and Techniques: We are actively involved in research and use the latest surgical methods and imaging technologies to improve outcomes.
• Family-Centered Care: We provide compassionate care and robust support services to help families every step of the way.

Our Pediatric Pulmonary Artery Reconstruction program treats a wide range of complex congenital heart conditions that affect pulmonary blood flow.

These conditions often involve abnormalities in the development of the pulmonary arteries and require advanced surgical techniques, including:

• Tetralogy of fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs)
• Unilateral absence or discontinuity of a pulmonary artery
• Complex congenital heart disease with abnormal pulmonary artery development or MAPCAs
• Single ventricle hearts
• Williams syndrome
• Alagille syndrome
• Other arteriopathies

Evaluation & Diagnosis

The journey typically begins with a comprehensive evaluation in our Heart Center. This may include imaging studies such as echocardiograms, cardiac MRI or CT scans, and cardiac catheterization to fully map your child’s heart and pulmonary artery anatomy. Our team will review your child’s medical history and collaborate to determine the best surgical plan.

Surgery

Pulmonary Artery Reconstruction surgery is performed under general anesthesia and lasts several hours. In cases of pulmonary artery reconstruction and unifocalization, the procedure is complex and will require the use of cardiopulmonary bypass. 

For the majority of children, the pulmonary arteries and MAPCAs can be fully reconstructed in a single-stage surgery. This approach allows the surgeons to complete the entire repair during one operation, resulting in low heart pressures and normal oxygen levels. The primary goal is to reconstruct a quasi-normal circulation, allowing the heart and lungs to function as close to typical physiology as possible.

Some children, however, require staged procedures because their pulmonary arteries are too small or underdeveloped for a complete repair at the first operation. In these cases, the goal remains to ultimately achieve the full reconstruction once the arteries have grown enough to allow it.

Hospital Stay & Recovery

After surgery, patients stay in the Regenstein Cardiac Care Unit (CCU) for close monitoring, followed by recovery on a general cardiac floor. The total hospital stay can range from 2 to 6 weeks, depending on the complexity of the case and the child’s recovery progress.

Follow-Up Care

Ongoing care is essential. Your child will continue to be followed by our pediatric cardiology team to monitor pulmonary artery growth, heart function, and overall health. Non-invasive imaging monitoring is recommended to confirm the adequacy of the repair. A follow-up catheterization is also recommended at approximately 1 year postoperatively to evaluate the new pulmonary artery tree system. In some cases, additional surgeries or catheter-based interventions may be needed as your child grows.

Family Support

Lurie Children’s offers comprehensive support services for families, including child life specialists, social workers, and counseling services. We understand that undergoing a major surgery is emotionally challenging, and we’re here to support both children and their caregivers throughout the journey.

For families traveling from out-of-state, our Heart Center team partners closely with Destination Services to support your family and help make your experience at Lurie Children’s as smooth as possible. Learn more about our Destination Services team.