Given that the heart is the hard-working engine that runs the body, new parents of a baby with a congenital heart defect are understandably frightened and dismayed to learn their son or daughter has a heart that is structurally or functionally imperfect. While pediatric heart problems will never—and should never—be taken lightly, the good news is that great strides have been made in treating them. With an arsenal that includes surgical procedures that can be done during infancy, or, sometimes, years later—even in adulthood, the field of pediatric heart surgery is undergoing dramatic change, with better technical knowledge and a burgeoning understanding of genetics.
Tain-Yen (T-Y) Hsia, MD, MSc, new to Yale Medicine as chief of pediatric cardiac surgery, has been part of this revolution since his training in London with one of the world’s preeminent pediatric cardiac surgeons. Now at Yale, he is working with colleague Peter Gruber, MD, PhD, a cardiothoracic surgeon and geneticist, to build a comprehensive children’s and congenital heart program here in Connecticut so families will never have to leave the state to receive excellent care.
Congenital heart defects affect almost 1 percent of births each year in the United States. That adds up to around 40,000 babies, about a third of whom will probably need surgery. There are many types of heart defects, so a pediatric heart surgeon can end up doing a variety of very different operations in a given month.
Dr. Hsia spoke to us about the vast knowledge a surgeon must have to treat these conditions, what the future holds for pediatric cardiac surgery, and the upcoming launch of Connecticut’s only pediatric heart transplant and mechanical cardiac assist program at Yale New Haven Children’s Hospital.
What inspired you to pursue this field?
I was initially interested in engineering, which I studied at MIT. I was particularly fascinated by fluid dynamics and heat mass transfer, two mathematical fields that deal with fluid flow and how a mass is transferred from point A to point B. These principles came to me quite naturally. Then, as a second-year medical student at Stanford, I was given the opportunity of a one-month observership in heart surgery, which had approaches and concepts that were very similar to engineering. In heart surgery—as in engineering—the essence of what you do is to identify a problem, fix the problem, and immediately see the result of your handicraft. That was very appealing.
That sounds very technical.
But it is very much a human endeavor, filled with expectations and emotions, successes and failures. It was important for me to pursue a career that would combine patient care with scientific exploration. During training, I was fortunate to be able to discover both with one of the most famous pediatric cardiac surgeons in the world, at the oldest children’s hospital in London. I knew then that helping children with heart problems was the right path for me. There is a unique responsibility and gratification that comes with fixing the hearts of infants and children. Knowing that a baby or a child my team and I cared for today can have a whole lifetime of possibilities tomorrow is very satisfying. It's exciting, it's rewarding, it never ceases to touch me to see how happy fathers and mothers are when they are told their baby had a successful operation. What’s more, the anatomy and physiology are fascinating, the surgery is technically demanding, and we always understand what the stakes are. To me, being a pediatric cardiac surgeon is a calling, and it is always a privilege. One must be driven by this awesome responsibility. You must believe you are doing something that is way beyond a simple job.
How is this kind of surgery particularly challenging?
For me, pediatric heart surgery is the very pinnacle of medicine. There is no other surgical field that demands higher perfection of technique, while also requiring a deep knowledge and profound understanding of the variety of problems—with mind-boggling complexity—of physiology. In congenital heart disease, the “defects” are many and the spectrum of operations is wide. Even the same defect will have different anatomy and require a different approach. Two babies may each have a hole in his or her heart, but each can have very different anatomy, requiring a different procedure. This means that a pediatric cardiac surgeon has to be well-versed in all the different types of operations and be prepared with a plan A, B, and C for each patient.
There are also adults who have congenital heart defects. Who treats them?
Yale Medicine’s Adult Congenital Heart Program was the first integrated program in the state to offer the full spectrum of care, including specialized imaging, catheterization therapies, and surgery. Here, my colleagues and I treat patients with congenital heart diseases in all ages—from newborns to adults. In fact, adult congenital cardiac surgery makes up about a third of my practice. We are seeing this fast-growing population in congenital heart disease as a result of successful medical and surgical treatment of these patients when they were babies and infants 20 or 30 years ago. Adults with a newly diagnosed congenital heart disease or those who had heart surgery when they were little will need continued care from specialists. And when an operation is needed, it should be carried out by a pediatric congenital heart surgeon—we have a better understanding of the altered physiologies that these heart defects create, and we possess the needed specialized surgical skills to treat them. My colleague, Dr. Gruber, and I were fully trained and board-certified in adult cardiac surgery before undertaking additional training in pediatric and congenital heart surgery.
How are advances making all of this easier?
There are a lot of things happening that are quite exciting. About 20 years ago there were conditions that we found so challenging, we were unable to provide treatment. Today, we know a lot more, thanks to refined imaging and surgical techniques, a better understanding of the physiology, and improved postoperative care. As a result, outcomes after these surgeries are much better. So, not only are our patients surviving operations, which was not always the case, their quality of life after surgery has improved as well. In most high-performing centers such as Yale, the overall success rate following pediatric cardiac surgery is more than 98 percent.
How is a better understanding of genetics changing the way you work now?
Genetics have not yet changed the way pediatric cardiac surgeons work, but it is an area that is becoming more important to us. For example, we now know that two babies with the same defect can have the same operation, but if one has a particular genetic predisposition, the outcome after surgery can be different. So while we are gaining knowledge, we are still in the very early stages of understanding the impact of genetics in congenital heart disease. There is so much more we need to know. We have world-leading experts here at Yale who are using data they gather from our patients to try to address some of these questions.
What else is on the horizon?
We are looking forward to new devices that can support failing hearts in small children and even infants. This progress has been slow—a lot of innovations in devices and surgical instruments for heart surgery have been aimed at adults, because they represent a much bigger population and, thus, demand. There has been less interest from the private sector in investing resources into the pediatric population, which is much smaller and requires devices with a much higher level of sophistication and innovation. So, there is a double whammy of small market and higher investment cost. There are a lot of things available for adults that are not yet available for babies and children.
One effort specifically tailored to children is a heart support device for babies born with only one ventricle (pumping chamber). Whereas you and I have two pumps—one for the right side of the heart and one for the left—these babies are born with only one functioning pump. They will need up to three operations over their first two to three years of life to have a survivable circulation, and living with this altered circulation has its consequences and complications. So, a group of us is focused on developing a device that will provide an artificial means to improve their blood flow.
The big news is that you will be providing pediatric heart transplants at Yale, correct?
That’s true. In February, we will launch the only pediatric heart transplant and mechanical cardiac assist program in Connecticut and Rhode Island at Yale New Haven Children’s Hospital. Every year, children in the states of Connecticut and Rhode Island have to travel to other states to undergo evaluation and management for heart transplantation as their hearts fail. This places enormous social, economic, and emotional stress on them and their families as they commute long distances away from home and loved ones, staying at remote hotels or other accommodations, to receive desperately needed care. What’s more, adults with congenital heart disease who need heart transplantation will receive the best care possible through a collaboration between pediatric and adult heart transplantation physicians and surgeons. This will make Yale New Haven Children’s Hospital the only destination for comprehensive heart care in newborns, infants, and children in Connecticut and Rhode Island.
How do you see congenital heart surgery changing at Yale?
While we have strong expertise here now, our hope is to create a world-class congenital heart program so any child or adult with congenital heart disease in Connecticut will not need to go out of the state to get the best possible medical, interventional, and surgical care. We will take care of patients from birth through adulthood in all facets of congenital heart disease, as well as for any heart problem developed during childhood. In that regard, we plan to establish the first and only heart transplantation and mechanical heart assistance program in the state to look after children with failing hearts. At Yale we strive to foster a personalized approach while delivering outstanding care. We believe every baby, infant, child, and adult patient, along with his or her family, is unique and deserves individual attention.
Anyone who is concerned about congenital heart defects should come and see us. We aim to provide hope for better, brighter, and healthier futures. I feel very blessed to be part of a dedicated team of experts and specialists that can deliver on these promises.