Sky Mattan Gopin is a miracle baby. He went from a heart transplant to nearly normal heart function in just a few months, without heart surgery. Everyone agrees it was amazing and something that rarely happens for such a sick baby. He went from the top of the heart transplant list – needing a transplant immediately – to the backlog of his transplant list.
A carefully orchestrated approach to care, combined with the love and commitment of her family, helped Sky survive. And, of course, his strong spirit.
“A big part of Sky’s survival was because he had doctors who knew what to do, but the biggest reason he survived was his own fighting spirit,” says Sky’s mother, Brittany or Britt. “When I was pregnant and we heard about her heart defect and other health issues, I put my hands on my stomach and asked her, ‘Do you want to be born?’ A ‘Yes!’ loud and clear resonated throughout my body.
After birth, every time Sky had his right arm thrown above his head – as if raising his fist to the sky – Britt was reminded of his strong will and desire to live. It became a symbol that helped her and her husband, Adam, fight alongside him.
Hearing Harsh News During Pregnancy
When Britt was pregnant, she and Adam were found out by specialists near their home in Mill Valley, Calif., that Sky had heart disease, along with other health issues. He was given a 1% chance to live.
“It was then that we decided to transfer the care to the Fetal Cardiology Program at Stanford Medicine Child Healthwhere we met Therese Tacy, MD, the director of the fetal cardiology program. She and her team gave us a much more optimistic prognosis during antenatal visits,” says Britt.
Sky was diagnosed with a rare Congenital heart defect called Ebstein’s anomaly, where the tricuspid valve, which regulates blood flow between the upper and lower right chambers of the heart, does not form properly in early pregnancy. It is a rare condition that cardiologists describe as unpredictable. Some babies with Ebstein’s anomaly become adults before showing symptoms. Others suffer from heart failure in the womb or shortly after birth. It is not always easy to tell which direction a child will go during fetal life or even after birth.
“Doctors caring for Ebstein babies can be marked by the challenge the disease can present. Yet with some Ebstein babies, if we are patient and nurture them carefully through a difficult initial period right after birth, some may adapt after a while and begin to act like babies with more normal physiology,” explains Dr. Tacy.
A related genetic variant and a new genetic discovery
In utero, Sky received a fetus echocardiogram (ultrasound of the heart), which showed his Ebstein anomaly, but could not show everything. He also underwent a prenatal genetic test which revealed a genetic variant associated with adult heart failure and, thanks to Sky and a few other children around the world, may provide some insight into why the abnormal formation of the heart muscle which is sometimes observed. in Ebstein’s 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 suffered from several heart problems,” explains Brendan FloydMD, PhD, pediatric cardiology fellow, medical geneticist and member of the Cardiogenomics care team.
A second heart disease discovered at birth: left ventricular non-compaction
After the birth, a second heart disease was discovered. Left ventricular non-compaction (LVNC)a kind of cardiomyopathymade the left side of Sky’s heart muscle spongy and weak rather than smooth and strong.
“Not only was Sky born with two heart problems, he was also born premature at 35 weeks. It all added up and he had heart failure shortly after he was born,” says Seth Hollander, MDpediatric cardiologist and medical director of heart transplantation at the Betty Irene Moore Children’s Heart Center at Stanford Medicine Children’s Health.
For Sky, the conditions on both sides of the heart meant that her heart was not pumping well and couldn’t pump enough blood to her lungs, body and brain. His life was in danger. Fortunately, he was at Stanford Children’s, where cardiologists have treated Ebstein’s anomaly and other complex heart defects countless times and are experts in caring for premature babies with difficult heart conditions.
Six bumpy weeks in cardiovascular intensive care
Since Sky wasn’t big enough or good enough to heart surgery—a decision that was made after a thoughtful conversation with Frank Hanley, MDThe Heart Center’s Chief of Cardiac Surgery – Heart Failure/Heart Transplantation Pediatric Advanced Cardiac Therapies (PACT) the team recommended that he go to the Heart transplant listing. Since preemie-sized donor hearts are rare, this was difficult news for the family.
“We thought, is this really his best chance of survival?” said Britt.
After a long discussion with the healthcare team and weighing the options, the couple decided to register Sky for a heart transplant.
Yet Sky had a completely different plan. He decided to become a miracle baby, and surprisingly, his heart began to grow stronger on its own. Her heart team recognized and capitalized on every slight sign of improvement, helping Sky’s life plan take root.
“We are able to take care of premature babies with rare and high-risk diseases and offer them the best way forward thanks to a cardiac team strong in all areas, from critical care and heart surgery to Heart transplantsays Dr. Hollander.
Knowing when to act and when to hold back defines expert care, including, in Sky’s case, when to remove her breathing tube, feeding tube and intravenous medication for her troubled heart. It helped Sky grow stronger on his own.
“It’s our job to carefully support newborns with Ebstein’s anomaly by giving them just what they need at the time and nothing more. Huge thanks to our team for never overmanaging Sky. To me, that’s exceptional care,” says Dr. Tacy.
“There were so many doctors all working together as a team, but their egos weren’t part of it,” Britt adds. “All of these brilliant minds figured out the puzzle that was Sky’s heart.”
Britt appreciated how the doctors and nurses put her in charge of some aspects of Sky’s care, helping her hold on despite the tubes and breastfeeding her.
“Britt and Adam are committed to treating him like a normal baby, and when he started breastfeeding I knew he was getting stronger,” says Dr Hollander.
Babies born with an Ebstein anomaly often have one or more ventricular septal defects (VSD) – a fancy way of saying holes in the heart. Sky had a VSD, which luckily shut itself down over time, which usually happens.
The Betty Irene Moore Children’s Heart Center adopts the rare approach of cardiologists and neonatologists (specialist 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 nationwide by US News and 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 critical to a good outcome,” says Sonia Bonifacio, MD, Sky’s neonatologist. “Cardiologists focus on repairing the heart, while the neonatologist’s perspective is more holistic: helping to support and protect the baby so they can lead a healthy life.”
Dr. Tacy would follow Sky throughout his life as a senior cardiologist. Britt appreciated Dr. Tacy’s positive attitude during her pregnancy, despite the surprises along the way. After all, it gave birth to Sky, a baby whose heart function is almost normal today. Sky is the first child for the couple, who have struggled to have a healthy baby in the past.
“Prenatally, we talk a lot about hope — rational, clear-headed hope,” says Dr. Tacy. “Sky’s parents have been extremely dedicated day and night to help her improve. By their constant presence, they sent her love, which I’m sure was as good as any medicine we gave.
Stanford’s childhood Cardiogenomics Program– a specialist team of cardiologists and genetic counselors dedicated to identifying inherited heart conditions – hope to publish the genetic discovery that Sky helped confirm so they can provide a clearer pathway for other patients to coming.
Go home and grow like a normal baby
When Sky was 6 weeks old, her parents received incredible news: Sky had been 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 hiatus, which could become permanent. For now, this miracle baby is getting stronger every day.
“He started laughing, which is really adorable. He smiles a lot and he likes to verbalize,” Britt says. “Every day he’s developing his ability to be in this world.”
While Sky may still need surgery in the future to fix his tricuspid valve, for now his heart is functioning almost normally.
“Sky has a bunch of cheerleaders at the Heart Center waiting, watching, and wanting to see him do well,” Dr. Tacy concludes.
Learn more about our cardiac care for children with complex heart conditions >