A Joyful Pause for Premature Baby on Heart Transplant List

Sky Mattan Gopin is a miracle child. He went from needing a heart transplant to near-normal heart function in just a few months’ time—without heart surgery. It was an amazing feat that few babies with such severe illnesses can achieve. 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. 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 was a symbol which helped her and Adam fight alongside each other.

Hearing hard news during pregnancy

Britt discovered that Sky, along with other conditions, was diagnosed by specialists while Britt was pregnant. His chances of living were 1%.

“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. Ebstein anomaly can cause some babies to become adults without any symptoms. Others may experience heart problems 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 variant of a gene 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 TNNC1Sky is responsible for one of the proteins that builds the heart. Sky is responsible for Sky’s creation of one of the proteins that build the heart. 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.

Left ventricular non-compaction was a second condition of the heart that was discovered at birth

The second type of heart condition that Sky developed after his birth was also 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 at risk. 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.

Six weeks of intensive cardiovascular 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. This was difficult news for the family as preemie-sized donors are rare.

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

After long discussions with the care team, and considering all options, the couple chose to list Sky for a heart transplant.

Sky had another plan. Sky decided to be a miracle baby and his heart started to heal 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. Sky was able to grow stronger by his own efforts.

“It’s our job to carefully support newborns with Ebstein anomaly by giving them just what they need in the moment and no more. Sky is managed well by our team. 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 was born with a VSD. Fortunately, it closed naturally over time. 

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.” 

Sky will continue to be treated by Dr. Tacy as his primary cardiologist throughout her life. Britt appreciated Dr. Tacy’s positive outlook while she was pregnant, despite surprises along the way. Sky was born with a near normal heart function. Sky is the first child of the couple who had struggled to have healthy babies 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. 

Returning home to grow like a normal child

Sky, who was six weeks old, received amazing news from his parents: Sky was transferred from the top to bottom of the heart transplant waiting list. He was ready to go home. His heart transplant is currently on pause. It could become permanent. The miracle baby is getting stronger by the 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.”

Sky may need surgery in the near future to fix his tricuspid tube, but his heart is currently performing well.

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

Learn more about our heart care for children with complex heart conditions >