A Joyful Pause for Premature Baby on Heart Transplant List

Sky Mattan Gopin is a miracle baby. He went from needing a heart transplant to near-normal heart function in just a few months’ time—without heart surgery. Everyone agrees that it was astounding and something that rarely happens for a baby so sick. He moved from the top of the heart transplant list—needing a transplant immediately—to having his transplant listing placed on hold.

A carefully orchestrated approach to care, combined with his family’s love and commitment, helped Sky survive. And, of course, his strong spirit.

“A huge part of Sky’s survival is that he had doctors who knew what to do, but the number one reason for his survival is his own fighting spirit,” says Sky’s mother, Brittany, or Britt. “When I was pregnant and we received news of his heart defect and other health issues, I put my hands on my belly and I asked him, ‘Do you want to be born?’ A loud and clear ‘Yes!’ rang through my whole body.”

After he was born, whenever Sky had his right arm flung over his head—as if raising his fist to the sky—Britt was reminded of his strong will and his desire to live. It became a symbol that helped her and her husband, Adam, to fight alongside him.

Hearing hard news during pregnancy

When Britt was pregnant, she and Adam found out from specialists near their home in Mill Valley, California, that Sky had a heart condition, along with other health conditions. He was given a 1% chance to live.

“That’s when we decided to transfer care to the Fetal Cardiology Program at Stanford Medicine Children’s Health, where we met Theresa Tacy, MD, the director of the Fetal Cardiology Program. She and her team gave us a much more hopeful prognosis during prenatal counseling,” Britt says.

Sky was diagnosed with a rare congenital heart defect called Ebstein anomaly, where the tricuspid valve, which regulates blood flow between the upper and lower right chambers of the heart, doesn’t form properly during early pregnancy. It’s a rare condition that heart doctors describe as unpredictable. Some babies with Ebstein anomaly grow into adults before they have symptoms. Others experience heart failure in the womb or shortly after birth. It’s not always easy to tell which direction a child will go during fetal life or even after birth.

“Doctors who care for Ebstein babies can be scarred by how challenging the disease can be. Yet with some Ebstein babies, if we are patient and we carefully nurse them through an initial rocky period right after birth, some can adapt after time and begin to act like babies with more normal physiology,” Dr. Tacy says.

A related gene variant and a new genetic discovery

In utero, Sky received a fetal echocardiogram (ultrasound of the heart), which showed his Ebstein anomaly, but it couldn’t show everything. He also had a genetic test prenatally that revealed a gene variant that is associated with adult heart failure, and—thanks to Sky and a few other children in the world—may provide some insight into the reason for abnormally formed heart muscle that is sometimes seen in Ebstein anomaly.

“The gene, called TNNC1, is responsible for making one of the proteins that build the heart. For Sky, his TNNC1 variant meant that he had multiple heart conditions,” explains Brendan Floyd, MD, PhD, pediatric cardiology fellow, medical geneticist, and member of the Cardiogenomics care team.

A second heart condition discovered at birth: Left ventricular non-compaction

After birth, a second heart condition was discovered. Left ventricular non-compaction (LVNC), a type of cardiomyopathy, made the left side of Sky’s heart muscle spongy and weak rather than smooth and strong.

“Not only was Sky born with two heart conditions, he was also born premature at 35 weeks. All of this added up, and he went into heart failure shortly after birth,” says Seth Hollander, MD, pediatric cardiologist and medical director of heart transplantation at Stanford Medicine Children’s Health’s Betty Irene Moore Children’s Heart Center.

For Sky, conditions on both sides of the heart meant that his heart wasn’t squeezing well and couldn’t sufficiently pump blood to his lungs, body, and brain. His life was in danger. Fortunately, he was at Stanford Children’s, where heart doctors have treated Ebstein anomaly and other complex heart defects countless times and are experts in caring for premature babies with challenging heart conditions.

A bumpy six weeks in cardiovascular intensive care

Since Sky wasn’t big enough or well enough for heart surgery—a decision that was made after a thoughtful conversation with Frank Hanley, MD, the Heart Center’s chief of heart surgery—the heart failure/heart transplant Pediatric Advanced Cardiac Therapies (PACT) team recommended that he goes on the heart transplant list. Since preemie-sized donor hearts are uncommon, this was difficult news for the family.

“We thought, is this really his best chance at survival?” Britt says.

After a long discussion with the care team and a weighing of options, the couple decided to list Sky for heart transplant.

Yet, Sky had a different plan altogether. He decided to become a miracle baby, and amazingly, his heart began to get stronger on its own. His heart team recognized and capitalized on every slight sign of improvement, helping Sky’s plan to live take hold.

“We’re able to take premature babies with rare and high-risk conditions and provide them with the best path forward from a heart team that’s strong in every area, from critical care and heart surgery to heart transplant,” Dr. Hollander says.

Knowing when to act and when to hold back defines expert care, including, in Sky’s case, when to remove his breathing tube, feeding tube, and IV medications for his troubled heart. It helped Sky grow stronger on his own.

“It’s our job to carefully support newborns with Ebstein anomaly by giving them just what they need in the moment and no more. I credit our team immensely for never overmanaging Sky. To me, that’s exceptional care,” Dr. Tacy says.

“There were so many doctors all working together as a team, but their egos were not a part of it,” Britt adds. “All of these brilliant minds figured out the puzzle that was Sky’s heart.”

Britt appreciated how doctors and nurses gave her the reins on certain aspects of Sky’s care, helping her hold him despite tubes and breastfeed him.

“Britt and Adam were committed to treating him like a normal baby, and when he started breastfeeding, I knew he was getting stronger,” Dr. Hollander says.

Babies born with Ebstein anomaly often have one or more ventricular septal defects (VSD)—a fancy way to say holes in the heart. Sky had a VSD, which fortunately closed on its own with time, something that typically happens.

The Betty Irene Moore Children’s Heart Center takes the rare approach of heart doctors and neonatologists (specialty doctors who care for sick newborns) collaborating within the Cardiovascular Intensive Care Unit (CVICU) to determine the best next steps to support premature babies. Neonatology care at Stanford Children’s is currently ranked in the top 10 in the nation by U.S. News & World Report.

“Having a neonatologist in the CVICU to focus on prematurity and consider the best course of action for the baby’s underdeveloped lungs, growth, and development is vital to a good outcome,” says Sonia Bonifacio, MD, Sky’s neonatologist. “The heart doctors focus on fixing the heart, while the neonatologist’s perspective is more global: helping to support and protect the baby so they can have a healthy life.”

Dr. Tacy will follow Sky through life as his primary cardiologist. Britt appreciated Dr. Tacy’s positive outlook while she was pregnant, despite surprises along the way. After all, it resulted in Sky, a baby with near-normal heart function today. Sky is the first child for the couple, who struggled to have a healthy baby in the past.

“Prenatally, we talk a lot about hope—a rational, clear-eyed hope,” says Dr. Tacy. “Sky’s parents were extremely dedicated day and night to help him improve. By their constant presence they sent him love, which I am sure was as good as any medicine that we gave.”

The Stanford Children’s Cardiogenomics Program—a specialized team of cardiologists and genetic counselors dedicated to identifying inherited heart conditions—hopes to publish the genetic discovery that Sky helped confirm so that they can provide a clearer path for other patients in the future.

Going home and growing like a normal baby

When Sky was 6 weeks old, his parents received incredible news: Sky was moved from the top of the heart transplant list to the bottom. It was time for him to go home. His heart transplant is on pause, which might become permanent. For now, this miracle baby is growing stronger every day.

“He started laughing, which is really sweet. He smiles a lot, and he likes to verbalize,” Britt says. “Every day he grows his capacity to be in this world.”

While Sky might still need a surgery in the future to repair his tricuspid valve, for now his heart is performing close to normal.

“Sky has a bunch of cheerleaders in the Heart Center who are waiting, watching, and wanting to see him do well,” Dr. Tacy concludes.