Laughter and a Potts Shunt Are the Best Medicine for Boy With Severe Pulmonary Hypertension

Stanford Medicine Children’s Health gives kids with advanced pulmonary hypertension another option besides lung transplant

Up until age 9, Hayden from Visalia, California, was unstoppable. Having lost his mother very early on to a hereditary form of severe pulmonary hypertension, he intrinsically knew how important it was to get the most out of every single day. Then, out of the blue, he started passing out while having fun.

“If I moved fast or if I laughed, I would pass out,” Hayden, now age 11, says. “It was scary to pass out, and we would have to go to the emergency room. It got worse, and I couldn’t run or ride my bike. All I could do was sit around.”

Pulmonary hypertension (PH) is an incurable disease that tends to worsen over time. Blood vessels in the lungs become blocked or narrowed progressively over time, causing high blood pressure in the lungs and failure of the right side of the heart. Symptoms of PH include shortness of breath, chest discomfort, fatigue, poor growth, lightheadedness, and fainting. Hayden passed out because his heart and lungs couldn’t keep up with the extra demand when he was active.

The first time it happened was when Hayden was playing football in the backyard with his dad, Ethan. His family took him to their local hospital, where he was diagnosed with the same pulmonary hypertension that his mother had. He was given medications, which initially improved his pulmonary hypertension, but it did not end his passing out.

“It’s not uncommon for patients to experience symptoms that seem to occur out of the blue,” says Rachel Hopper, MD, associate director of the Pulmonary Vascular Disease program at Stanford Medicine Children’s Health and co-director of the newly established Center for Advanced Lung (CEAL) Therapies program. “Hayden carries a gene mutation that is known to cause pulmonary hypertension. The disease slowly progresses, and it is so gradual that people often do not notice symptoms until the disease reaches a critical stage.”

Stanford Medicine Children’s Health’s Pulmonary Vascular Disease program treats children of all ages with all different types of pulmonary vascular disease, including pulmonary hypertension. The program is one of the longest-standing programs in the nation, and one of the largest, caring for hundreds of children from across the country and the world.

“We came to Stanford Medicine Children’s Health, and the doctors said that we needed to take the next step,” says Vanessa, Hayden’s stepmom.

The next step for Hayden was to receive medical therapies, including treprostinil, an advanced medication delivered continuously through a pump that is worn on the body. While it helped decrease pressure in his heart and lungs, Hayden experienced side effects, such as infusion site pain and severe stomachaches, that kept his dose low. Therefore, it was not enough to make him better.

An alternative to lung transplant: The Potts shunt

“Traditionally, lung transplant has been the only option to treat severe pulmonary hypertension when medications aren’t enough. Now, we can give these kids more options. Helping Hayden inspired us to formally develop our new multispecialty Center for Advanced Lung Therapies,” Dr. Hopper says. “Collaborative, multidisciplinary care is essential for these patients.”

The Center for Advanced Lung Therapies gives kids like Hayden a fighting chance at survival. As the only center of its kind on the West Coast, it brings together heart, lung, and lung transplant specialists and subspecialists under one roof to provide revolutionary treatments to keep kids healthy until transplant. These include leading-edge mechanical circulatory support devices (devices that stand in for the lungs and/or heart) and the exciting Potts shunt for children whose hearts are still working well. This latter group included Hayden.

“The Potts shunt holds a lot of promise for kids like Hayden who experience side effects from medications or who have maxed out their doses of medications and are not getting better. It’s a game changer,” says Rebecca Kameny, MD, co-director of the Center for Advanced Lung Therapies.

Doctors partner with computational biology experts at Stanford Medicine to design a Potts shunt that’s personalized for each patient, zeroing in on the exact length and positioning for their unique anatomies.

Potts shunt—a natural fit for Stanford Medicine Children’s Health

“This was our first Potts shunt surgery, and it was a team effort. I felt very comfortable performing it because we do highly complex pulmonary artery reconstruction within our Betty Irene Moore Children’s Heart Center. We had several discussions to prepare for Hayden’s procedure, which helped the surgery go really smoothly,” says Elisabeth Martin, MD, surgical director of the Center for Advanced Lung (CEAL) Therapies and Bloodless Surgery Program at Stanford Medicine Children’s Health.

Benefits of the Potts shunt can include improved symptoms and improved survival. It can also mean less medication and a delayed need for lung transplant. Best yet, the procedure allows kids to be kids. Today, Hayden’s pulmonary hypertension is managed with oral medications.

A successful first surgery

“Hayden was the perfect patient for our first Potts shunt procedure. He was scared, which is understandable, but he was very brave and mature. He wanted to understand the surgery, and he was actively involved in our discussions,” Dr. Martin says.

The Potts shunt is not a new technique, but it was reconsidered by doctors in Paris, France, for this purpose. The method used for pulmonary hypertension is technically a reverse Potts shunt.

During this procedure, heart surgeons strategically place a custom-designed shunt (small tube) to provide a pop-off for blood flow from the lungs and relieve strain on the heart, specifically the right ventricle. Right ventricle function is a main driver of survival for kids with advanced lung disease.

“Kudos to Dr. Martin and the cardiac anesthesia team for getting Hayden through surgery safely and successfully. The procedure is relatively straightforward. The tricky part is working with the blood vessels, so it’s critical to have a skilled surgeon who is experienced doing that,” Dr. Kameny says.

“I can’t believe how much the Potts shunt helped me. Now I can play with my little brother. We play hide and seek, and I chase him around the house speed walking,” Hayden says.

Besides Hayden’s incredible doctors and nurses, the family is grateful to the social workers at Stanford Children’s for securing housing, meals, and supplies near the hospital during Hayden’s six-week stay. That way, they could be close by and focus on their son.

“It was one of the hardest times of our lives, but we had so much support at every step, from arrival to discharge,” Vanessa says. “We really felt like we were in the best of hands, and we are so grateful to Stanford.”

Back to laughing and loving life

“It broke my heart that the kid who liked to come in and tell jokes couldn’t laugh for fear of passing out, so when I saw him after he received his Potts shunt and he said, ‘I can laugh,’ it made me so happy,” Dr. Hopper says.

Recent tests showed that while he still has pulmonary hypertension, the function of Hayden’s right ventricle is close to normal. It’s a great relief for the family.

Hayden is more surprised than anyone about how great he feels now, compared with before he had the Potts shunt. He is back to riding his bike, making up beats and dance moves, chasing his little brother, fishing, and swimming with his friends on hot days. And of course playing with his pet bugs—a praying mantis and a spider. “If you are a kid and you are thinking about the Potts shunt, do it,” Hayden says. “Take the risk. I am so happy that I did it because I feel so much better. If I didn’t, I wouldn’t be able to laugh right now.”

Learn more about our Pulmonary Vascular Disease Program and CEAL (Center for Advanced Lung Therapies) >