Young at Heart
On a recent Thursday morning, a nurse anesthetist held open the door to Operating Room No. 25 for William Douglas, M.D., a pediatric cardiothoracic surgeon affiliated with Children’s Memorial Hermann Hospital and chair of the Division of Pediatric Cardiovascular Surgery at McGovern Medical School at UTHealth. His patient had already been prepped by the surgical team, who paused and individually stated their names before assuming their respective roles for the 2.5-hour procedure ahead. Douglas would be opening up the chest of a young baby whose heart was no larger than the size of a plum.
The baby was born with a congenital heart defect (CHD) that disrupted the directional flow of the arteries, and Douglas and his team were tasked with re-routing the blood so the patient’s circulatory system would function properly. The procedure required the creation of a small hole between the upper two chambers of the heart as well as the placement of a shunt between the pulmonary arteries and the aorta; without the surgery, blood would continue to bypass the lungs and the patient would not survive.
The field of fixing children’s hearts is at once specialized and comprehensive—and vastly different from the world of adult cardiology. The focus is not acquired heart disease but rather a malformation of the organ itself. Pediatric cardiologists and cardiovascular surgeons are skilled in handling these organs that, while mechanically and anatomically are nearly identical to the adult heart, present the challenge of being miniaturized. Moreover, congenital heart defects, also known as congenital heart disease, can run the gamut of potential malformations: leaky or blocked valves, holes in the heart’s chambers or walls, a narrowing of the aorta, the reversal of major arteries, underdevelopment of entire sections of the heart—the list goes on. Because the condition is characterized by an inherent failure of development, it is often a significant health concern.
According to the Centers for Disease Control and Prevention (CDC), CHD affects nearly one percent, or about 40,000 births each year in the United States. It is the most common type of birth defect and approximately 25 percent of babies born with CHD will have a critical form, meaning they will need either surgery or some other form of treatment within the first year of their lives. Options range from monitoring and medication to catheter-based interventions, open-heart surgery and, in the most severe cases, transplantation—all of which are offered in the Texas Medical Center between the two primary pediatric heart institutions: Texas Children’s Heart Center at Texas Children’s Hospital and the Children’s Heart Program at Children’s Memorial Hermann Hospital.
Currently ranked No. 2 in the nation for pediatric heart care and heart surgery by U.S. News & World Report and the only hospital in Texas to be named an Accredited Pediatric Heart Failure Institute, Texas Children’s has been at the forefront of pediatric heart care since the beginning, and, in fact, was largely responsible for the formation of the field itself. A perfect union between some of the top pediatric specialists in the country at a time when the cardiopulmonary bypass machine made open heart surgery possible laid the foundation for Texas Children’s to perform many of the earliest, most successful lifesaving procedures over five decades ago.
Historically, CHD was discovered in newborns through a predictable litany of symptoms: labored breathing, weak pulse, poor feeding, excessive sleepiness or a bluish tint to the skin indicating that the blood is not receiving oxygen—a condition known as cyanosis or “blue baby.” In recent years, however, prenatal screening for heart malformations has become routine, meaning a majority of these conditions are caught as many as 20 weeks before birth. It’s a practice that is largely heralded as a central factor in the uptick of survival rates for babies born with CHD.
Using a sophisticated imaging technique called fetal echocardiogram ultrasound, sonographers are able to evaluate multiple views of high-quality images of a developing fetus’ heart. If a problem is suspected, the mother is referred to a fetal cardiologist for diagnosis and management.
“Getting a clearer look at the function of these babies’ hearts enables us to improve risk stratification before birth,” said Helena Gardiner, M.D., co-director of the Fetal Cardiology Program at The Fetal Center at Children’s Memorial Hermann Hospital and McGovern Medical School at UTHealth. “The most serious heart conditions will require very careful management within the first hours after a baby is born, and for that reason we recommend that these patients be delivered in the medical center so that they can be close to the experts who specialize in this kind of care. Having a baby with a heart problem should not be a surprise.”
“In a single lifetime, we’ve gone from being unable to offer anything to approaching 100 percent survival.” — Charles Fraser, M.D., Texas Children’s Hospital’s Surgeon-in-Chief and Chief of Congenital Heart Surgery and Professor of Surgery and Pediatrics at Baylor College of Medicine
While this innovation has afforded lifesaving opportunities in the form of preparation, the instruments are, as they say, only as good as their operators. Through a first-of-its-kind public health campaign, the fetal cardiology team at Children’s Memorial Hermann Hospital has made it their personal mission to provide advanced training to sonographers throughout Harris County and beyond. Their goal is to teach these clinicians not only how to become proficient in the equipment itself, but to also recognize when the developing heart looks abnormal; if a sonographer suspects an anomaly, he or she will send the image to Memorial Hermann for evaluation and diagnosis—an elegant example of the telemedicine activities for which the hospital system is renowned.
“A network approach is quite important,” Gardiner explained. “We use several advances to support our network of clinicians who work more remotely from the cardiac surgical center. This begins by ensuring they have good ultrasound equipment and are familiar with the screening heart views, the five transverse views. We can then view these scans from hundreds of miles away using the Memorial Hermann System to address the sonographer’s or physician’s concerns, and we can determine whether or not the mom needs to travel here to see a specialist.”
Although ambitious in its breadth, the team does not expect to produce any overnight experts in the many variations of congenital heart disease.
“It’s important to us that the sonographers in the community know what to look for, not so much so they can make a diagnosis—that’s not the critical component—we want them to know what normal looks like, and when it doesn’t look like that, to send us the images so that we can evaluate them and determine whether or not we need to see the patient,” explained Anthony Johnson, D.O., co-director of The Fetal Center at Children’s Memorial Hermann Hospital and McGovern Medical School at UTHealth. “I’d rather see a dozen cases that might be something that turn out to be fine than have one case be missed because someone didn’t know what they were looking at.”
Texas Children’s has deployed their clinical expertise across the greater Houston community as well by creating a network of pediatric, maternal and fetal clinic locations to assist community physicians and obstetricians in the detection of fetal anomalies, including congenital heart. Over the past year, Texas Children’s Maternal and Fetal Center conducted more than 41,000 ultrasounds and fetal echocardiograms across the Houston area and beyond. The majority of these patients were evaluated and cared for in their own community, however, for the most complex cases, a higher level of care was necessary and 120 mothers with complex cardiac babies were transferred to Texas Children’s to gain the comprehensive expertise needed immediately after delivery.
Management plans for CHD depend on the type and severity of the malformation. Unfortunately, survival depends on something else entirely: location, location, location.
The importance of the where alongside the what underlies Memorial Hermann’s and Texas Children’s efforts to improve detection of fetal anomalies throughout Texas. As Gardiner pointed out, many of these conditions require careful, expert management; if an obstetrician knows his or her patient’s baby has CHD, it is often crucial that delivery takes place at a hospital that specializes in pediatric cardiology and cardiovascular surgery. Troublingly, not all of these centers are created equal.
“The most important thing is getting the patients to the right hospital at the right time,” said Charles Fraser, M.D., Texas Children’s Hospital surgeon-in-chief and chief of congenital heart surgery and professor of surgery and pediatrics at Baylor College of Medicine. “That affects more lives than all of the advancements in our field combined. There is an enormous disparity in outcomes if you compare centers in this country and that is a tragedy; despite how far we’ve come, it can still be an accident of birth—that where you happen to be born will have a direct impact on your prognosis.”
The numbers don’t lie. Although the majority of pediatric heart centers do not publicly release their mortality profiles, most confidentially report their results to the national database managed by the Society of Thoracic Surgeons. Just last year, a composite of this data was made publicly available in a report published by CNN, which revealed that surgical death rates ranged from 1.4 to 12.1 percent, depending on the hospital. That’s an astronomical difference when it comes to life and death.
“If our society was really aware of this issue, I don’t think any of us would accept it,” Fraser said. “Your mortality profile could be 10 times worse depending on where you are born in the United States. Ten times worse. Can you imagine that? That’s not what we as a society believe in, but it’s a fact. It’s an unarguable fact.”
Fraser and colleagues from around the country are becoming increasingly vocal about this disparity in hopes of raising awareness and ultimately fostering change. To turn a mediocre center great, however, first requires an understanding of the numerous and varied components that contributed to the overall decrease in mortality rates in the first place.
“We have 21 children in our dedicated cardiovascular ICU this morning,” Fraser said. “I don’t believe there is a child in there who would have been alive when I was a baby. Not a single child. And almost every single one of these children will survive and move on from the hospital with an acceptable prognosis. In a single lifetime, we’ve gone from being unable to offer anything to approaching 100 percent survival. It’s astonishing, and you honestly couldn’t point to just one thing to explain it. It’s a concert of having a dedicated children’s hospital, focused children’s care, the development of all the sub-specialties that are around us as well as general advancements in cardiology and cardio-surgery, intensive care, neonatology, anesthesia, pharmacology and physiology. On top of that, our focused surgical teams and specialists don’t work on anything else but the heart. That’s all we do, that’s all everybody in this heart center does—our anesthesia team, our ICU—we don’t do anything else.”
Add high patient volume, multiple surgeons, strategic multidisciplinary partnerships and emphasis on the importance of a continuum of care, and one begins to see what sets the Texas Children’s Heart Center at Texas Children’s and the Children’s Heart Program at Children’s Memorial Hermann Hospital apart from the pack.
“There’s an increasing appreciation that it’s really all part of the continuum and that there is likely a sequence of crucial times during development that are really important,” explained Daniel Penny, M.D., Ph.D., chief of cardiology at Texas Children’s and professor of pediatrics-cardiology at Baylor. “For that reason, we work closely with our partners at Texas Children’s Pavilion for Women, Texas Children’s Fetal Center,TM the Texas Heart Institute, and other pediatric specialists here at Texas Children’s Hospital to make sure we’re addressing every aspect that impacts the child’s wellbeing. One of the big advances over the last 10 years or so is the increasing recognition that heart disease in children isn’t just a disease of the heart. It’s not just about funny valves and funny hearts, it’s a condition that really affects the whole child, it affects the family and ultimately society.”
Penny and his team at Texas Children’s are directing efforts to some of the non-cardiac implications of heart disease in children, including neurodevelopment delays caused by the abnormal hemodynamics in utero common to patients with CHD as well as symptoms of post traumatic stress disorder observed in children and their families after open heart surgery.
And they don’t stop there. Thanks to the field’s considerable advancements in the last five decades, babies born with CHD are now growing up to be adults. Although many are healthy overall, these individuals do require periodic monitoring and care their whole lives. Rather than send them to adult cardiologists, who specialize in a completely different kind of heart disease, Texas Children’s has engaged a large team of specialists to provide dedicated care for these patients.
“It is now well-known that there are more adults living with congenital heart disease than children with congenital heart disease in the U.S. today,” Penny said. “So we’ve put a great deal of effort into developing that integration between pediatric care and adult care. It isn’t something we can just take for granted—we have to plan and build processes and programs to meet the unique needs of these individuals. We need to prepare and be ahead of it and face these challenges rather than wait for other people to do it.”
In keeping with this philosophy, Texas Children’s offers the most advanced treatment options available, including fetal intervention procedures. Widely recognized as a nascent subspecialty only recently put into practice, fetal intervention has been documented to save fetuses with certain heart abnormalities. Currently, the risk runs high and the level of surgical specialization required is equally prohibitive, but Texas Children’s has performed several procedures on these tiny developing hearts with success, in fact pioneering many of the surgical techniques currently used to correct evolving malformations before they become irreparable and fatal.
“The theory is sound, and I do believe it will play a major role in the field in the future,” Fraser said. “We know structural problems with the heart evolve as gestation progresses and that there are certain conditions that can be rescued. In other words, as the heart is developing, the condition is actually getting worse. The theory is, if you could intervene during fetal life, you might mitigate some of the subsequent consequences. We’re still in the proving stage of these theories and the techniques are evolving, so what this translates into is still continuing to unfold, but there have definitely been cases where we have intervened successfully.”
No one doubts that fetal intervention will likely play a major role in treating congenital heart disease in the future. Couple that with new mechanical assist devices tailored for small hearts but engineered to last a lifetime, biomaterial scaffolds designed to guide growth of new tissue, and a host of other technologies only now just being developed, and the field could look completely different than it does today.
“I do think some of this will be revolutionary in the next 10 to 12 years,” Douglas said. “We’re kind of at an inflection point right now with biologically compatible materials, but when these replace the inert patches we’re using now—when we repair blood vessels and heart valves with materials that will eventually incorporate into living tissue—I predict we’ll see better long- term post-surgical outcomes that could make a substantial difference.”
Straight out of the best kind of science fiction, the future for treating congenital heart conditions is bold and innovative. Unfortunately, none of it will fix the millions of perfectly healthy hearts that will grow up and fail every year.
Heart disease—the kind you acquire from poor diet, lack of exercise, smoking and other risk factors—is the No. 1 killer in the world. According to the American Heart Association, it accounts for 17.3 million deaths each year around the globe; the CDC says that’s one in four deaths in the U.S. This number is expected to rise universally. According to Penny, it will be the next big global pandemic in developing countries as well, eclipsing even HIV.
“We know that the seeds for acquired coronary artery disease in adults are actually laid down when they are children,” Penny said. “Our goal is to identify patients with these risk factors so that we can intervene early enough and hopefully reduce their risk of developing heart disease in the future.”
Both Texas Children’s Hospital and Children’s Memorial Hermann Hospital have robust preventative cardiology programs focused on addressing the issue. It’s a move that will certainly help, but even with Texas Children’s being the largest pediatric hospital in the nation and Memorial Hermann Health System’s omnipresence in the Houston landscape, their programs will only go so far. In the end, the responsibility lays bare, ready to be picked up by parents, school districts, lobbyists, even children themselves.
“It’s pervasive and unarguable,” Fraser said. “Approximately one out of 100 children are born with congenital heart disease, but those other 99 face a lifetime risk of acquired heart disease, and we’re doing it to ourselves. There are all these things we can do preemptively to have a healthier population and we’re ignoring it. Ultimately, there’s only so much you can do with knives and medicine.”
(Click to enlarge)