Baby born with half a heart supported with unusual ventricular assist device

A baby boy at Lucile Packard Children’s Hospital Stanford is thought to be the first U.S. infant in his precarious medical situation to be supported with a heart pump as he awaits a heart transplant.

Brayden McQuillan, now 3 months old, had a ventricular assist device implanted on his 18th day of life to help his failing heart pump blood. Although VADs are now used in hundreds of children and thousands of adults who have heart failure, their use in newborns is still rare and risky.

But Brayden had no other options. Born Jan. 13 in Redding, Calif., after a full-term pregnancy, the 6.5-pound baby boy seemed fine at first. However, a nurse soon noticed that Brayden’s heartbeat did not sound right. He was flown to the medical center of the University of California, Davis, and diagnosed with hypoplastic left heart syndrome, in which the left side of the heart does not develop properly before birth. Essentially, Brayden has only half a heart.

“It was awful to hear,” said Brayden’s mom, Alysa McQuillan. “There are not really words to describe that kind of pain.”

Doctors at UC Davis thought Brayden’s best hope was a heart transplant. He was airlifted to the Betty Irene Moore Children’s Heart Center at Packard Children’s, the highest-ranked pediatric heart center on the West Coast.

“This heart defect is always severe, but some affected infants are sicker than others,” said David Rosenthal, MD, director of the Pediatric Heart Failure Program at Packard Children’s. “Brayden was born very unstable with this disease. He had to be rescued with a complex initial surgery, but even that was not enough to stabilize him.”

Most children with hypoplastic left heart syndrome can be treated with a series of three surgeries that enable their hearts to pump enough blood to sustain their bodies over the long term, Rosenthal said. But after pediatric cardiothoracic surgeon Katsuhide Maeda, MD, performed the first such surgery, Brayden was still not doing well. The medical team recommended to Alysa and her husband, Dustin, that Brayden be added to the waiting list for a heart transplant.

“We felt better about transplant than the series of surgeries,” Alysa said. “A brand-new heart will give him more of a chance.”

To keep Brayden alive long enough to get that chance, he needed the VAD. The doctors told Alysa and Dustin that their son would probably be the first baby in the country with his medical situation to receive one. A few other infants with unrepaired hypoplastic left heart syndrome in Europe and Canada have been placed on VADs, but there are no U.S. case reports to date, and the VAD that Brayden needed had to be ordered specially for him.

“Our medical team told us there was a 50-50 chance that Brayden would make it out of surgery to implant the VAD, and if he did, a 50-50 chance he’d survive to get a transplant,” Alysa said. “It was really scary.”

So far, Brayden is beating the odds. The pump now sustaining him, which was implanted Jan. 30, is a hybrid of two other forms of ventricular assist device used in children. Like the Berlin Heart, whose U.S. use was pioneered at Packard Children’s in 2004, it has tubes carrying Brayden’s blood from his chest to an external pump. But Brayden was too tiny for the pulsatile pump usually used with a Berlin Heart to be effective, so his blood is being moved by a pump from a different VAD called a PediMag, which uses a spinning electric motor that produces constant blood flow. Brayden is the first patient at Packard Children’s to receive this device.

“This design works well in very small children because it provides a lot of blood flow,” Rosenthal said. The high flow is especially important for a baby with half a heart. Normally, one half of the heart pumps blood to the lungs and the other half to the rest of the body.

“Brayden’s heart has to pump to the lungs and the body simultaneously, and has to supply about twice as much blood to the lungs as to the body,” Rosenthal said. “So the heart’s doing about triple the usual work.” With the VAD, Brayden’s heart can keep up. His blood pressure is stable, he’s breathing on his own and his other organs have recovered from the initial strain of his early heart failure.

Alysa and Dustin finally got to hold their son on March 11 — the first time either of them had held him since Alysa cuddled with him for a few minutes immediately after he was born.

Now, the McQuillans are anxiously hoping Brayden will be matched to a donor heart. “Especially for someone his size, it’s very hard,” Alysa said. According to data from the United Network for Organ Sharing, 3,191 people across the United States received heart transplants last year. Of those, 130 were less than a year of age at transplant.

In total, nearly 2,000 children across the country are awaiting organ transplants. April is Donate Life month, a period when national efforts are underway to encourage people to register as organ donors.

Stanford researchers are also advancing other treatment options for babies who need heart transplants. Christopher Almond, MD, associate professor of pediatrics at the Stanford University School of Medicine, is one of the principal investigators of a clinical trial set to launch next month for the Jarvik 2015, a first-of-its-kind VAD for babies and small children.

Meanwhile, Brayden’s medical team is optimistic. “He’s doing well,” Rosenthal said. “He’s a good candidate for heart transplant, which he was not when he arrived at our hospital.”