Highly specialized program offers exciting alternative for kids with single ventricle hearts
Hands down, 3-year-old Liam’s favorite structure on the playground is the jungle gym.
“I like the jungle gym. The top is really high,” he says.
Not too long ago, climbing was a near impossibility for the energetic toddler. Because of his congenital heart defect, he could barely run, let alone climb on the jungle gym before his highly complex heart reconstruction surgery.
“It was hard to watch. He’d want to run on the playground with his friends, but he’d only make it a few steps before he’d need to squat down to catch his breath,” says his mom, Mai Nguyen.
When Mai was 17 weeks pregnant, she and her husband, David, learned that Liam had a serious congenital heart defect that affected several areas of his heart. They were told he might not survive after birth, or he would survive and immediately need a heart surgery with more to follow. They had a hard decision to make.
“It was really scary. We debated whether we should bring him into the world if he would have to suffer, but the Fetal Cardiology Program’s team at Lucile Packard Children’s Hospital Stanford monitored him closely. A large team of doctors felt his heart condition was fixable, so we decided to move forward,” Mai says.
“We can tell if a congenital heart disease is mild or severe well before a baby is born. We help parents figure out what is important to them, and then we establish a plan of care that is best suited to them,” says Theresa Tacy, MD, fetal cardiologist and medical director of the Fetal Cardiology Program at the Betty Irene Moore Children’s Heart Center.
Choosing a two ventricle vs. single ventricle approach
Liam survived his birth and endured two heart surgeries in his first two years of life, with a major surgery expected at the age of 3—a surgery called a Fontan procedure, which is meant to improve blood flow to the lungs, and subsequently the body, by creating one strong pumping chamber—called a single ventricle heart.
“When children are born with only one usable pump instead of two, like Liam, the pump is assigned to pump blood from the heart to the body. It works for a couple of decades, but when children with single ventricles become adults, they often need a heart transplant,” says Deborah Ho, MD, MPH, pediatric cardiologist and medical director of the Complex Biventricular Reconstruction Program.
Repairing a flawed heart so that it has a single ventricle that functions well often means survival and improved heart function for children with certain types of congenital heart disease. Yet, having one pump is not as ideal as having two. That’s why the Moore Children’s Heart Center offers an exciting alternative—biventricular (two ventricle) reconstruction, to restore a child’s heart anatomy to as close to normal as possible.
Instead of creating one strong pump, the Complex Biventricular Reconstruction Program’s multidisciplinary team of innovative cardiovascular surgeons, cardiologists, and congenital heart disease specialists devise a plan for rebuilding, rewiring, and reconfiguring the structure of a child’s heart so that there are two working ventricles. To achieve this, they perform a number of procedures to improve the workings of the heart, often all at once during one surgery.
“Biventricular reconstruction increases the complexity of the initial surgical repair but can enable a much better long-term outcome, with less need for future surgeries and avoidance of heart transplantation down the road,” says Michael Ma, MD, cardiothoracic surgeon and surgical director of the Complex Biventricular Reconstruction Program.
“Because our fetal cardiology team caught Liam’s condition well in advance of his birth, our team had ample time to speak with the family, offer a variety of solutions, and help mom and dad make a well-informed decision,” adds Dr. Ma. “Ultimately, what we offer each patient is very customized for that family’ wishes, and in Liam’s case, I felt confident we could give Liam a brighter future.”
Liam’s parents were thrilled that there was an alternative for Liam, who was born with a condition called heterotaxy. It’s a rare genetic syndrome that causes organs throughout the body to form incorrectly, including the heart. Heart defects are often very complex, with heart structures and blood vessels in the wrong positions.
“If you think of the normal heart as a carefully coordinated Lego build, the heart Liam was born with had all of the individual pieces, but arranged in a very dysfunctional manner. During complex biventricular reconstruction, we rearrange and reshape those pieces to improve function and blood flow,” Dr. Ma says.
Undergoing a lengthy but life-changing operation
Liam’s surgery took 18 hours and demanded incredible skill and ingenuity. These types of procedures are performed by only a few pediatric heart centers in the world. To prepare, Dr. Ma used innovative 3-D modeling to create a physical model of Liam’s heart.
“I bring these models home with me and actively visualize every reconstruction for several weeks before the operation, even dream about them at times, to help create a thorough plan for surgery day. Each patient is memorable. I have a bookshelf lined with these models, each representing a child whose heart we were able to bring closer to normal anatomy. I’m very proud of our program,” Dr. Ma says.
Each year, Lucile Packard Children’s Hospital Stanford performs close to 40 complex biventricular reconstructions, about three to four a month. Only the top children’s hospitals across the nation offer biventricular reconstruction, and rarely for such highly complex conditions. That’s why the program receives a large number of referrals from other pediatric heart programs, and also from adult heart programs around the nation and world.
“We can often provide alternatives to single ventricle surgeries, even for the most challenging anatomies. If we can bring a patient’s heart to more normal circulation, they can often have a higher quality of life,” Dr. Ho says.
“At Stanford Medicine Children’s Health, we have a culture that inspires us to act boldly to save our patients’ lives, and we are used to performing highly complex heart surgeries. We always want to explore the limits, while being extremely mindful of the health and livelihood of the children we are trying to help,” Dr. Ma adds.
During Liam’s reconstructive surgery, Dr. Ma had the challenge of repairing a very large hole in his heart, moving three vital heart structures, and creating an aortic baffle, a structure that directs blood flow in the heart. He spent extra time preserving Liam’s pulmonary valve and moving it to its correct location, rather than placing an artificial valve. Because the valve is Liam’s own, it can grow with him as he grows, eliminating the need for a future surgery to replace it.
“We were so happy and relieved to hear Liam made it through the surgery and it went well. It was hard just sitting there and waiting, so I can’t imagine what it was like to be in the operating room working that long,” Mai Nguyen says. “We had such a great team on our side; everyone was focused on one thing—helping Liam have a better life. Packard Children’s has the best heart team around, and that’s why we chose to give them 100% of our trust.”
Newfound energy to tackle the jungle gym
During follow-up visits, Mai revels in viewing an echocardiogram of Liam’s heart—seeing a solid area where the big hole used to be and watching blood flow freely through his heart. Liam’s oxygen levels are normal, at a steady 97%. It’s a huge improvement over his levels before the reconstruction, which would hang around 72%, indicating poor circulation, which was evident in the blue tint of his lips, fingertips, and toes.
The Packard Children’s heart team appreciates the close collaboration with the Nguyens’ cardiologist in San Jose, California, which has helped Liam receive exceptional follow-up care.
“Dr. Ma is our hero. He pretty much saved Liam’s life. I am so grateful for him and the entire team for giving us a different option for Liam. It was a glimpse of hope and light, and Packard Children’s gave that to us,” Mai says. “We can now think about Liam living a long life and growing into an old man.”
Today, Liam is unstoppable on the playground. He runs and chases his friends for hours. Most important, he climbs the jungle gym and dreams of the day he will make it to the top.
Learn more about our Betty Irene Moore Children’s Heart Center services >
