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UCSF Medical Center

Advancing Care for Pediatric Pulmonary Vein Stenosis

newborn baby

Pulmonary vein stenosis (PVS) requires highly specialized care, and new treatment strategies are improving outcomes for even the most critically ill patients. At UCSF Benioff Children's Hospitals, our dedicated Pulmonary Vein Stenosis Program is pioneering a team-based approach that's helping children with severe PVS survive and thrive.

The Pulmonary Vein Stenosis Program is the only dedicated clinic for this condition in the Bay Area and the only Pulmonary Vein Stenosis Network collaborating center in California. “The program is one of the few in the nation that has the infrastructure for a comprehensive, multidisciplinary approach to this condition,” says Elena Amin, MD, director of the Pulmonary Vein Stenosis Program and interventional cardiologist at UCSF Benioff Children’s Hospitals. “Our specialization in this condition draws patients from across the Western United States.”

Changing the paradigm for PVS

Five years ago, children with severe PVS rarely survived beyond age 2. Today, the UCSF team is successfully discharging patients who would previously have been offered comfort care. These children return home to continue their care, in partnership with our team. “We've watched a cohort of children with this condition turn 2, and they are thriving at home, which is exciting,” Dr. Amin says.

These improvements are largely due to:

New treatment options

Dr. Amin explains, “Because patients had a grim prognosis in the past, doctors tended to offer comfort care right away. Now, we tell families that comfort care is available, but we could also try a comprehensive treatment pathway. In many cases, the outcomes have exceeded our expectations.” Options for treatment may include:

  • Additional catheter-based interventions to keep the vein open
  • Close, coordinated care in the NICU or PICU
  • Surgery when indicated
  • Tightly coordinated discharge and follow-up

Comprehensive medical management

Patients often have multiple, complex needs, such as ventilator strategies, fluid balance, nutrition and immunosuppression management. A multidisciplinary approach ensures all these needs are covered.

Advanced interventional techniques

The team specializes in leading-edge balloon procedures, with careful monitoring for complications, such as infection in immunosuppressed patients. Interventional options include:

  • Balloon angioplasty. This procedure widens the vein.
  • Cutting balloon angioplasty. This technique uses a catheter with tiny blades to make incisions in the scar tissue, allowing the vein to open more easily.
  • Drug-eluting balloon angioplasty. A balloon coated with medication helps prevent the vein from narrowing again.
  • Sutureless repair techniques. This surgical method repairs the vein without traditional stitches and reduces inflammation and scarring.

Coordinated treatment across specialties

Historically, care teams had to fragment PVS management. An interventionalist would open or widen the vein using balloon angioplasty or a stent, while a separate physician would coordinate follow-up and everyday needs. This siloed model could miss critical aspects of disease management.

“Opening the vein to correct the structural issue is important, but it does not fully restore blood flow in many cases,” Dr. Amin says. “Patients often require specialized medications to remodel the vessels behind it and improve flow. They also need ongoing monitoring and testing to address any complications that arise.”

This model integrates expertise from multiple specialties into a cohesive care team that includes:

Other team members include:

  • Clinical pharmacists
  • Nurse practitioners with specialized training in pulmonary hypertension and interventional cardiology
  • Registered dietitians
  • Social workers

The PVS team meets twice a month to review every patient’s case. “We discuss imaging findings, coordinate interventions and develop individualized care plans,” says Dr. Amin. “For the sickest patients still hospitalized, we perform daily rounds to ensure a rapid response to changes in clinical status.”

The team provides specialized PVS expertise, while the primary and referring providers continue to offer ongoing care. Patients typically only need a transfer to UCSF for cardiac interventions, returning to their local physicians afterward. The Pediatric Heart Center serves as a regional referral center for this complex patient population.

Advancing research

With one of the largest PVS patient populations in the country, UCSF is actively contributing to the growing body of research on this rare condition. The program collaborates with the Pulmonary Vein Stenosis Network, an international research consortium that collects data from centers across the United States and Canada.

While FDA-approved treatments for PVS don't yet exist, there are still medical options. The team is studying off-label medications and sharing their findings with the consortium. “Certain immunosuppressive medications and pulmonary hypertension drugs can benefit patients with PVS,” says Dr. Amin. “These approaches are on a case-by-case basis. We look carefully at each patient’s needs and create a tailored treatment plan.” 

The program also organizes annual sessions on PVS at the This conference brings together national and international experts to share the latest advances.

Accessible care for all ages

While most PVS patients are premature infants or young children, UCSF treats all ages. Adult patients with this condition may have congenital venous anomalies or iatrogenic PVS following catheter ablation for heart rhythm conditions.

The program offers both in-person and virtual visits () to minimize travel burdens. For patients traveling from distant locations, the team coordinates echocardiograms, nuclear medicine pulmonary flow scans, CT scans and laboratory tests during single-day visits at the Mission Bay campus.

“For patients who live farther away, we often coordinate their imaging locally and follow up with virtual visits,” Dr. Amin explains. “Patients typically continue to see their local care teams while we partner with them to provide that PVS specialization.”

To refer a patient to the Pediatric Heart Center, visit our referral page or call (877) 822-4453 (877-UC-CHILD).