Fit to Run: A rare heart procedure for Ziyan Liu

Every Wednesday at Ziyan Liu’s middle school in Pleasanton, California, students run the mile as part of “Workout Wednesday.” 11-year-old Ziyan, who was born with a congenital heart condition, wasn’t healthy enough to join them. Soon she was even too sick to stay in school. But a rare surgery helped her survive long enough to receive a heart transplant. And, just a few months later, she lined up for the race.

KTVU Fox 2 recently featured Ziyan in a touching story about two young patients who received heart transplants at Lucile Packard Children’s Hospital Stanford:

“Before her heart transplant, she didn’t like having to do PE,” says her cardiologist, Sharon Chen, MD, clinical assistant professor of pediatric cardiology at Stanford Medicine Children’s Health. “She said doing PE was hard for her, and that made sense because she would get blue anytime she tried to do any physical activity.”

Ziyan was born in China in 2006 and got very sick when she was about three months old. She was diagnosed with pulmonary hypertension — increased blood pressure in her lungs’ arteries — and abnormal narrowing of her pulmonary vein, which returns blood from the lungs to the heart. She was weak and her skin looked blue, a condition known as cyanosis.

Ziyan’s doctors in China found the root of her condition was a life-threatening structural abnormality in her heart. Instead of having separate mitral and tricuspid valve inlets, Ziyan’s heart had a single opening into the ventricular chambers, and mostly to one side, resulting in a condition called an unbalanced atrioventricular (AV) canal. Her right ventricle was significantly larger than her left, resulting in a heart with a single ventricle. Surgeons in China performed a surgical technique known as pulmonary artery banding — the only procedure that could potentially help her — to reduce her pulmonary artery pressure and excess pulmonary blood flow. The procedure was palliative — meaning it could bring her some relief but would not correct or cure her condition.

Because Ziyan’s heart condition was so dire, there was nothing else her doctors could do for her. Ziyan had traveled to the U.S. a few times to have her care managed by Rajesh Punn, MD, clinical associate professor of pediatric cardiology at Lucile Packard Children’s Hospital Stanford. Then, when she was five years old, her family moved from China to California. Dr. Punn recognized that Ziyan was going to need a heart transplant, and that’s when she met her new care team at Packard Children’s.

“She was eight years old when she was referred to our team,” recalls Dr. Chen. “It was clear then that there were no further surgical options to fix her heart or improve her cyanosis. We first treated her with medicines. When her condition worsened, we had her listed for a heart transplant.”

After about two years of waiting for a heart, Ziyan’s condition became critical in the fall of 2016. Her parents, Bin and Xuan, brought her to the pediatric emergency department at Stanford Health Care. “I thought I would just get better and go home,” Ziyan says. “I did go home, but then I had to go back a second time two weeks later.” It was then that her surgical care team at Packard Children’s, led by Katsuhide Maeda, MD, clinical associate professor of cardiothoracic surgery at the Stanford University School of Medicine, decided to try something unconventional.

Many patients who are awaiting transplant rely on a surgically implanted mechanism called a ventricular assist device, or VAD, but using this device in patients with single-ventricle hearts is very challenging — so much so that these patients are not considered candidates for the device at most hospitals. Yet Packard Children’s has more experience placing VADs in single-ventricle patients than most other pediatric centers.

“I’ve done about 12 or 13 single-ventricle VADs,” says Dr. Maeda, “and they are all very challenging.” The structure of Ziyan’s heart presented an added challenge. “Usually we attach the VAD onto the ventricle,” Dr. Maeda says. “But for Ziyan, we attached the VAD on the atrium. That is something people usually do not do. We are proposing that this method is easier and results in a better hemodynamic,” which refers to the flow of blood throughout the organs of the body.

Dr. Maeda and his surgical team performed Ziyan’s innovative single-ventricle HeartWare VAD placement on December 2, 2016, and added a modified Blalock–Taussig (BT) shunt to increase blood flow to Ziyan’s lungs. “This is also very, very rare,” says Dr. Maeda. “Especially in a single-ventricle, I’ve never heard of anyone adding a BT shunt.” Even Dr. Maeda himself had never performed such a procedure before.

“There was no other way to do it,” Dr. Maeda says.

The surgery was a success, but, as Dr. Maeda points out, Ziyan’s recovery would depend on more than just the surgery. “Her body wasn’t used to tolerating this type of circulation,” he says. “That’s why we had to watch her very carefully after the surgery. And the cardiovascular intensive care unit team and the heart failure team did a great job managing her care after surgery,” he says.

Just a few weeks after the procedure, Ziyan had recovered enough to leave the hospital. She and her family stayed close to the hospital at the Ronald McDonald House Stanford, and she participated in physical therapy and rehabilitation and attended the hospital school. Months passed, and in July 2017, Ziyan’s family finally got the call that a donor heart was available. It wasn’t just the surgeons who helped save Ziyan. The family who chose to donate their loved one’s organ in the face of loss gave Ziyan’s the chance for a fresh start.

Compared to the rare single-ventricle VAD placement Ziyan had received, “the transplant was relatively straightforward,” says Dr. Maeda. Although Ziyan’s heart transplant procedure lasted several hours, it went off without a hitch.

“I got stronger every day, and I felt better than before. My energy was better,” says Ziyan.

A few weeks after her procedure, Ziyan got to do something that most of us will never do: She held her own heart in her hands. As with all transplant recipients, Ziyan was invited by her cardiologist, Dr. Chen, to walk to pathology and take a close look at the heart that had been replaced by a healthy donor heart. “It was a little bit gross and a little bit cool, too,” says Ziyan. “I was glad I saw it.”

Viewer disclaimer – the following images picture a human heart, which may be disturbing to some readers

Three months after her transplant, Ziyan was able to return to school in her hometown, which also meant returning to PE. “My doctors gave me a note that I could do whatever, but if I felt tired I should rest,” she says. “At first, I didn’t do as much because I’d get tired. But then I started to do what everyone else was doing — normal PE things, running and playing.”

“After her transplant, Ziyan was no longer cyanotic and her oxygen saturations were normal,” says Dr. Chen. “Her recovery time was impressive. Some kids who we’ve transplanted are not able to fully participate in PE for another year just because they’re still recovering from it.”

Two “Workout Wednesdays” passed, and then Ziyan made her move. On November 15, she lined up with her classmates and successfully ran the mile, just five months after her heart transplant. “I thought, as long as I don’t come in last I’d be happy. And I didn’t!” says Ziyan. “I felt proud of myself.”