Conditions and Treatments 

Within our program, we focus on diagnosing and treating children with advanced pulmonary hypertension or developmental lung disease.

Understanding pulmonary hypertension and developmental lung disease

  • Advanced pulmonary hypertension. A condition where there is high blood pressure in the lungs. Pulmonary hypertension can be genetically passed down within families, or it can occur without a known cause. It can also be associated with another condition, such as congenital heart disease, connective tissue disorders, or lung diseases. Severe pulmonary hypertension can cause the right side of the heart to work harder to pump blood, which can lead to right-sided heart failure.
  • Developmental lung disease. A broad term that describes different types of lung disease. Lung disease can be inherited, result from an autoimmune disease, or start after chemical exposure or infection. In severe lung disease, the lungs become scarred and stiff, making it hard to breathe and difficult for the oxygen-rich blood to move from the lungs to the heart and the rest of the body. Damage to lungs may not be possible to heal. In our CEAL program, we treat complex, advanced lung disease that isn’t responding to traditional treatments. For many of the children we see, we are the family’s last option while they await lung transplant.
  • Interstitial lung disease. Interstitial lung disease (ILD) is a general term for a group of rare lung disorders that make it hard to breathe. Many of these diseases can be difficult to diagnose and manage, and they can affect people at any age. At our Center for Advanced Lung (CEAL) Therapies, we collaborate with pediatric experts in interstitial lung disease who understand the intricacies of these rare disorders in children.


We are one of a very few pediatric centers in the nation to offer a combination of unique therapies. These include providing a bridge to transplant (getting your child strong and ready for transplant), providing lung and heart-lung transplants, and/or performing Potts shunt surgery.

Bridge to lung transplant therapies

With our bridge to lung transplant therapies, our goal is to help your child survive to lung transplant and become as healthy as possible while awaiting lung transplant. The healthier your child is going into lung transplant, the better she or he will adjust to new lungs and recover. Bridge to lung transplant therapies include:

  • Medicines. We provide the full range of medicines for pulmonary hypertension and lung disease, as well as new, innovative medicines.
  • ECMO. Extracorporeal membrane oxygenation (ECMO) is a lifesaving device that serves as an artificial lung in emergencies, but it’s not meant to be used long term. For this reason, we aim to get your child off of ECMO and onto a mechanical circulatory support device to empower him or her to recover and feel better.
  • Mechanical circulatory support devices. These devices are ideally used before your child’s health becomes critical, helping to avoid some of the late-stage complications that can come with lung or heart failure, such as failure of multiple organs. These devices allow your child to move around more freely and breathe on his or her own without a breathing tube attached to a ventilator. Being able to walk around and breathe independently greatly improves your child’s strength and overall health. Our surgical and medical teams will work closely together to determine the right type of device to place in your child so that he or she can gain strength while awaiting lifesaving lung transplant.

Mechanical circulatory support devices

We use the following devices to support your child’s heart and lungs as he or she awaits transplant in our Cardiovascular Intensive Care Unit, which offers the most advanced critical care, with access to the very latest life-sustaining technology. Some children need just lung support, while others need both heart and lung support. Devices are placed during a surgery. We use two main types of support devices, which sit outside of the body and are connected to your child’s circulatory system via tubes through the skin.

  • Oxygenator. These devices stand in for your child’s lungs. They take in oxygen and release carbon dioxide. They can be used safely in combination with a pulmonary to left atrium device (PLAD), a device that helps move blood from the lungs to the left atrium (chamber) of the heart.
  • Oxygenator with right ventricular assist device (RVAD). If your child needs both heart and lung support, we use an oxygenator with a pump, or RVAD. The oxygenator replaces the function of the lungs, and the RVAD helps the heart pump and circulate blood. We’re a national leader in using VADs in children, and our heart surgeons are especially innovative in how they place VADs. We are fortunate to partner with the Pediatric Advanced Cardiac Therapies (PACT) team, who have extensive, world-renowned experience in surgical placement of VADs and medical management of patients who live with these devices.

Lung and heart-lung transplant

Lung transplant is the main treatment for advanced pulmonary hypertension and advanced lung diseases. Through innovative solutions, we work to keep your child stable while he or she awaits donor lungs. There are seven pediatric lung transplant programs in the United States, and Stanford Medicine Children’s Health’s is the only one on the West Coast.

Potts shunt

For children with advanced pulmonary hypertension with a working right heart ventricle, we explore the option of placing a Potts shunt. We have achieved significant benefits with the Potts shunt in children who have advanced pulmonary hypertension.

About the Potts shunt

  • In the last five-plus years, the Potts shunt has been repurposed from its original use to what’s known as a reverse Potts shunt to treat advanced pulmonary hypertension.
  • Simply put, a shunt changes the flow of blood between the heart and the lungs. A shunt is created by strategically placing a small tube during surgery. The tube connects the left pulmonary artery with the aorta that comes from the heart. It helps blood go around the pulmonary vessels (that are under high pressure) and flow to the body, relieving the strain on your child’s heart, specifically the right ventricle. Right ventricle function is a main driver of survival and symptoms in patients with pulmonary hypertension, so this procedure can improve length and quality of life.
  • A strength of our Center for Advanced Lung Therapies (CEAL) is that our heart doctors, heart surgeons, lung transplant doctors, and pulmonary vascular disease doctors all weigh in on your child’s Potts shunt placement to make better and more timely decisions. In addition, in our region, Lucile Packard Children’s Hospital Stanford uniquely offers both lung transplant evaluation and Potts shunt evaluation at once, which is critical for providing your child with every option available.

Benefits of the Potts shunt

  • Five-to seven-year research data is very promising in terms of improved symptoms and improved survival for children who receive a Potts shunt.
  • Some children who undergo Potts shunt placement can reduce the medications needed for pulmonary hypertension.
  • A Potts shunt can delay the need for a lung transplant.
  • Children often feel better after the placement of a Potts shunt. They tend to have more energy and are often able to enjoy limited activity.