Building a Coordinated Approach to Pulmonary Vein Stenosis at the Heart Institute at UPMC Children’s Hospital of Pittsburgh

For many years, and until only very recently, pulmonary vein stenosis (PVS) was a condition with few, if any effective treatment options to provide good long-term outcomes. Interventions frequently failed, vessels re-narrowed, and mortality remained high, particularly among premature infants with diffuse disease. The problem was not just a technical one. Clinicians were attempting to treat a progressive biological process as though it were an isolated mechanical obstruction.

That understanding has changed in the field. Early recognition and a better understanding of risk profiles combined medical and procedural management, and longitudinal surveillance protocols have changed how pediatric cardiology teams approach treating PVS.

Among many recent clinical and research advancements of the last five years, the Heart Institute at UPMC Children’s Hospital of Pittsburgh has developed a multidisciplinary pulmonary vein stenosis program designed around the realities of how the disease behaves versus historical approaches.

The program is led by interventional cardiologist Sara M. Trucco, MD, FSCAI, FACC, FPICS, associate director, Cardiac Catheterization Laboratory and director, Quality Improvement for Cardiac Catheterization, and Shawn C. West, MD, MSc, assistant professor of pediatrics and a specialist in advanced cardiac therapies.

Dr. Trucco and Dr. West lead a multidisciplinary team that includes experts from cardiac imaging, cardiothoracic surgery, pediatric pulmonary medicine, and neonatal and cardiac intensive care units.

“When I was training, PVS was something we didn’t necessarily treat because it was largely thought to be futile. You could balloon the veins, but it would come back, and the expectation was that the child was not going to survive. What changed, among other things, was the realization that if you identify it early and treat it aggressively, both medically and in the catheterization lab, there is a chance for survival,” Dr. Trucco says.

Pulmonary Vein Stenosis is a Condition Defined by Progression

PVS is characterized by progressive narrowing that often recurs despite reestablishing or reinforcing patency of the vein. Clinically, the condition presents across several distinct patient populations, including premature infants with chronic lung disease, children with congenital heart disease following pulmonary vein intervention, and less commonly in an idiopathic manner. The severity of PVS is determined by the degree of narrowing within an individual vein, the number of veins affected, and the distribution of disease across the pulmonary circulation.

“Even limited narrowing can redirect blood flow and increase pressure and shear stress in the remaining veins. Over time, that leads to cellular proliferation and further narrowing, creating a cycle that allows the disease to progress,” Dr. Trucco says.

PVS is driven in part by inflammatory and proliferative responses within the affected veins, which promote recurrent narrowing even after a successful intervention. As individual veins become compromised, redistribution of pulmonary blood flow increases pressure and flow through remaining vessels, leading to continued disease activity and the potential for multivessel involvement over time. Progressive obstruction also increases pulmonary venous and arterial pressures, which can lead to pulmonary hypertension and increasing strain on the right ventricle.

“Pulmonary vein stenosis behaves in part as an inflammatory disease. We don’t fully understand what initiates it, but once it begins there is proliferation within the veins, almost like scarring, which contributes to recurrent narrowing,” Dr. West says.

These dynamics require management strategies that extend beyond interventions to open the vein or veins. Maintaining vein patency depends on repeated reassessment of anatomy and physiology, integration of medical therapy to slow inflammatory and proliferative activity, and close surveillance during periods of active disease. The clinical course of PVS involves ongoing interaction between vascular biology, pulmonary physiology, and timing of intervention rather than a single definable treatment endpoint.

Building a Program Around the Biology of the Disease

The PVS program at UPMC Children’s grew from the recognition that no single intervention could address the condition by itself.

The multidisciplinary structure of the program mirrors the multidimensional clinical picture of PVS. Feeding difficulties and aspiration can worsen lung injury. Ventilation strategies influence pulmonary pressures.

Nutrition affects growth and recovery. Inflammation associated with prematurity or infection can speed up the vascular proliferation processes. Managing pulmonary vein stenosis requires coordination across multiple organ systems rather than procedural decision-making in isolation.

“Pulmonary vein stenosis really requires a collaborative approach. It’s not just intervention or medical management. We work with the ICU teams, pulmonology, surgery, imaging specialists — everyone is involved in deciding the best approach for each patient,” Dr. Trucco says.

Regular multidisciplinary meetings allow the team to review echocardiography together, determine when higher-level imaging is needed, and decide when patients should proceed to catheter-based or surgical intervention. The intent is to identify and manage patients early before their condition worsens, and to reassess longitudinally as patients move through repeated cycles of imaging, treatment, and follow-up.

How Patients Move Through the Program

Building the PVS program began with creating screening protocols for premature infants at elevated risk. Infants who meet defined criteria undergo routine echocardiographic surveillance, allowing clinicians to detect early signs of pulmonary vein disease before clinical deterioration occurs. When imaging raises concern, additional studies refine the anatomic picture and guide intervention.

Following development of screening protocols, the team also established a formal management algorithm to guide decision making once PVS is identified. Rather than making decisions around a single procedure, the algorithm organizes care into four areas:

• Criteria for intervention.
• Anatomical assessment.
• Intervention.
• Postintervention management.

The first area defines when disease progression warrants escalation beyond surveillance. Anatomical assessment integrates echocardiography, CT imaging, and catheter-based evaluation to characterize the patients disease and severity. Intervention decisions are made collaboratively, incorporating catheter-based therapy, surgical options, and medical management. The final phase focuses on postintervention surveillance and optimization of comorbid conditions because keeping the veins open and controlling disease progression requires ongoing monitoring.

“The goal isn’t just to open the vessels. It’s to keep them open and remove anything that makes the disease worse. That means surveillance, frequent imaging, and managing the other problems these babies have at the same time,” Dr. West says.

Once PVS is identified, patients enter a cycle of surveillance, intervention, and reassessment. The interventional cardiology team performs balloon angioplasty or place stents to restore flow when narrowing progresses. Surgical intervention is appropriate in select cases depending on anatomy and disease distribution. Many infants require repeat procedures during periods of active disease.

Medical therapy addresses the biological drivers of recurrence. Anticoagulation reduces thrombotic risk following intervention, and antiproliferative agents, including sirolimus, are used when needed to slow inflammatory and proliferative changes within the vessel wall.

“We’ve also learned that things like aspiration, poor ventilation, or poor nutrition can make pulmonary vein disease progress faster. Managing those issues is just as important as what we do in the catheterization lab,” Dr. Trucco says.

Stabilization and Long-Term Uncertainty

Although PVS can be aggressive in infancy, the field has observed that some patients stabilize over time. As lung growth occurs and inflammatory drivers improve during early childhood, disease activity may decrease in some patients. Intervention frequency can decline, and some patients may eventually discontinue medical therapy while remaining under surveillance.

“In some cases, the inflammatory process burns itself out over a couple of years. If you can keep the veins open long enough for the lungs to grow and the underlying problems improve, the intensity of treatment can decrease,” Dr. Trucco says.

As patient survival continues to improve, transition to adult congenital heart disease and pulmonary hypertension programs will become an increasingly important component of long-term care for this patient population.

“This is a new phenomenon. Twenty years ago, many of these babies wouldn’t have survived. We don’t yet know what these patients will look like 20 or 30 years from now, but hopefully we’ll be able to say that more of them made it to adulthood,” Dr. West says

Collaborative Efforts to Advance Patient Care

The PVS program at the Heart Institute at UPMC Children’s continues to evolve alongside national efforts to better understand PVS. Participation in multicenter registries will help to standardize data collection and clarify optimal treatment strategies, including timing of intervention and medication use. Differences between institutional approaches to PVS are common. Collaborative research will help to refine care over time and create more consensus and uniformity in treatment based upon the evidence.

One effort on this front includes a prospective PVS registry launched by the Congenital Cardiac Research Collaborative (CCRC) in 2025. The Heart Institute at UPMC Children’s is one of the members of the CCRC, and Bryan H. Goldstein, MD, director of the Cardiac Catheterization Laboratory at UPMC Children’s is one of the CCRC’s founding members, while Dr. Trucco currently is a member of the CCRC’s QI Committee.

The CCRC prospective registry also includes a retrospective component and is designed to study and promote better treatment pathways for infants and young children with PVS. The registry also was designed to enable embedded clinical trials through a “Trial within a Registry” approach, to speed up the time from clinical question or hypothesis to study completion and potential new treatment options.

“Earlier recognition, coordinated management, and sustained follow-up have transformed how we approach a disease once considered uniformly fatal,” Dr. Trucco says. “The work now focuses on refining that approach and understanding how to improve the long-term trajectory of our patients. That’s why registries like the CCRC one are vital to helping shape how we treat our patients and improve long term outcomes.”

More Information and Patient Referrals

Learn more about the Heart Institute at UPMC Children’s Hospital of Pittsburgh.

For general questions or inquiries, please call 412-692-5540 or send an email to CHPHeartReferral@chp.edu.

You can also visit our referral information web page for specific contact information for our pediatric cardiology and cardiothoracic surgery programs.

Other members of the multidisciplinary team

From left to right:

• Adam Christopher, MD, director, Cardiac MRI and CT, Division of Pediatric Cardiology
• Levent Midyat, MD, medical director, Advanced Lung Diseases and Transplantation program, Division of Pediatric Pulmonary Medicine
• Mario Castro Medina, MD, Division of Pediatric Cardiothoracic Surgery
• Jamie Bloch, CRNP, Division of Pediatric Cardiology
• Catherine Brailer, CRNP, Division of Pediatric Cardiology
• Yolandee Bell-Cheddar, MD/MBBS, MSc, Cardiac Intensive Care Unit
• Abeer Azzuqa, MD, clinical director, UPMC Children’s Neonatal Intensive Care Unit
• Sharon Mazzocco, BSN, RN, MAS, CPN, Quality Coordinator, Cardiac Catheterization Laboratory