Looking at him now, you’d never guess 10-year-old Ryan Gates was born with a combination of five serious heart defects. Photo by Mason Poole

Issue: Spring 2006

Tender Hearts

Learning that your baby has a congenital heart defect no longer means certain heartbreak. Thanks to intricate surgeries and earlier diagnoses, most tots with broken tickers don’t just survive: They thrive.

By Katie Sweeney

Jennifer ’84 and Steve Gates had no reason to suspect anything was wrong with Ryan. He was a pink and perfectly formed newborn, weighing in at a whopping 9 pounds. Thrilled, they took him home to join big brother Connor, an active 2-year-old.

For about a week everything was fine. Then troubling signs began to surface. First there was Ryan’s breathing – it seemed too fast. Concerned, Jennifer called the pediatrician.

It’s typical for newborns to have irregular breathing, the doctor reassured her.

Jennifer hung up the phone. He’s right, she told herself. I’m just overreacting.

But more problems followed. Ryan began to experience eating difficulties. His weight was dropping. At three weeks, the pediatrician made an ominous-sounding diagnosis: “failure to thrive.” The term is commonly used to describe infants who aren’t gaining weight normally. Jennifer and Steve took their son to a nearby hospital for a battery of tests, including an echocardiogram – an ultrasound of the heart.

It soon became clear why Ryan was struggling. The baby who had looked so healthy in the delivery room in fact suffered from a combination of five serious heart defects – including hypoplastic right heart syndrome, a condition where one side of the heart is severely underdeveloped.

Steve and Jennifer were stunned. Congenital heart defects? How could this be? No one in their families had ever had such a problem.

“I worried about everything during my pregnancy,” Jennifer recalls. “Except this.”

A generation ago, a baby born with such a severe set of heart problems would have had a slim chance of survival. But over the past quarter century, babies like Ryan have been given a good chance not only to survive, but to live normal lives, too.

“In the ’60s and ’70s, many of the complex forms of heart disease were fatal in infancy,” says Roberta Williams, chair of pediatrics at the Keck School of Medicine of USC and vice president for pediatrics and academic affairs at Childrens Hospital Los Angeles. “Now, the vast majority are reaching adult life, and most of them are quite healthy.”

While heart transplants are performed in some cases, they aren’t the norm, due to both a dearth of available donor hearts and the potential for organ rejection throughout the child’s life. Instead, surgical advances and improved care have made it possible to repair an infant’s own heart in the first days or weeks of life.

That’s good news, especially because congenital heart problems are the most common birth defects, occurring in about eight of every 1,000 live births. Many of those defects are relatively mild, but some, like Ryan’s, are life-threatening.

In most cases, there’s no known cause for these defects, although genetic errors are presumed to be at fault. But when you consider the complexity of the heart’s development, it seems amazing that it ever turns out right.

The fetal heart begins to beat about three weeks into gestation, making it the first organ to function. Initially, it’s a tiny, pulsing tube. Over the next three months that tube grows quickly, looping around and folding on itself to create four chambers – two atria and two ventricles – as well as four valves and the circulatory, coronary, electrical and lymphatic systems. Throughout this intricate chain of events, the heart sustains the fetus’s life.

If all goes well, the newborn baby will have a healthy heart. Usually no bigger than a walnut, this tireless workhorse will pump blood continuously through the circulatory system. The right ventricle will send blood to the lungs for fresh oxygen, while the left ventricle will move the oxygen-rich blood out to the body to nourish tissues and organs. The valves will open and close to let blood flow in only one direction.

Occasionally, though, something goes wrong during the heart’s development, resulting in a defect. Defects can range from small “holes” between the chambers of the heart to major malformations, such as missing or underdeveloped chambers, or vessels attached to the wrong tubing.

Los Angeles magazine had already called Vaughn Starnes “the foremost pediatric cardiothoracic surgeon in the world” in 1995. Photo by Walter Urie, courtesy of CHLA

“There’s a wide range of things that can happen to the development of the heart and arteries during the embryology of a developing child,” says Vaughn A. Starnes, chair of cardiothoracic surgery at the Keck School and director of the Heart Institute at Childrens Hospital Los Angeles.

According to Starnes, heart problems vary so much that no two children will exhibit the exact same defect; nor is there a standard surgery to correct them all. At the Heart Institute, surgeons perform a constellation of 20 to 30 different procedures on a routine basis, operating on more than 800 children a year.

Many of the operations are extraordinarily complex, requiring not just skilled and experienced surgeons, but also a team of specially trained nurses, physicians and therapists. For this reason, the delicate surgeries are performed at specialized pediatric heart centers, such as the Heart Institute at Childrens Hospital – one of the largest in the United States.

The only times Steve Gates had been inside a hospital were to witness the births of his two children. But with Ryan’s diagnosis, he and Jennifer suddenly found themselves practically living in one, straining to understand their son’s condition and make life-or-death decisions about his treatment.

The first challenge: choosing a surgeon. Ryan would need a series of three open-heart operations to repair his defects, they had learned. And the first one should happen as soon as possible.

“When you have that type of experience with your child, it almost seems like the world stops,” says Steve. “You don’t know where to turn.”

They got some advice from Jennifer’s brother, Randy Ryan ’87, MD ’92: Go to Childrens and talk to Vaughn Starnes, he had urged. Both siblings are USC alumni (as are their parents, Phyllis ’59 and David Ryan ’60) but Randy had the advantage of studying medicine at the Keck School, so he knew all about Childrens Hospital Los Angeles.

It was the fall of 1995, and Starnes had arrived three years earlier, lured away from Stanford University by the opportunity to restructure the heart-lung program at USC University Hospital and create and head a new Heart Institute at Childrens Hospital. In an April 1995 article, Los Angeles magazine had called him “the foremost pediatric cardiothoracic surgeon in the world.”

Steve and Jennifer had already interviewed one surgeon, and another was on their list after Starnes. They had a deal: no decisions until talking to all three.

But once in Starnes’ office, they forgot all about that. He was on the cutting edge with surgical advances. He was honest and direct. And there was no time to lose. Ryan’s health was quickly deteriorating; his lips and skin had taken on a blue tinge from a lack of oxygen in his bloodstream.

They didn’t even have to discuss it.

“It was as if a stone had been lifted off our backs,” Steve recalls. “We knew Ryan was going to get the best of care.”

When one side of the heart is underdeveloped, a baby is left with essentially a two-chambered heart instead of a four-chambered one. Surgeons are unable to use tissue to build two new chambers. Instead they reconstruct the heart to function using the two working chambers it has.

That reconstruction takes place through a series of three open-heart surgeries. For hypoplastic right heart syndrome, the first operation is performed shortly after birth and is called the Blalock-Taussig Shunt. Surgeons construct a synthetic tube, or shunt, and connect one end to the artery going to the right arm and the other end to the pulmonary artery, which goes to the right lung. This allows some of the blood going mainly to the body to go to the lungs, where it can pick up more oxygen.

But that fix is only temporary. By the time the baby is 6 to 9 months old, the shunt can no longer supply all the oxygen a baby needs to grow. At that point, a second surgery redirects all the blood coming from the upper body to the lungs. At age 2 or 3, a third and final surgery is performed, redirecting the blood coming from the lower part of the body to the lungs.

Today a baby has a greater than 90 percent chance of surviving all three of these surgeries, Starnes says. Lifelong follow-up care will be needed, and many will have to take heart medicines. Still, most will lead fairly normal lives.

“We fully anticipate that they’ll be able to go to school, and their physical activities will be pretty normal,” he explains. “They’re not going to be competitive athletes. But they can certainly participate in playground activities and things like playing soccer with their friends.”

Some heart defects can be corrected with just one surgery, such as when a baby’s main arteries – the aorta and pulmonary artery – are connected to the wrong chambers. (Ryan had this defect as well.) This means that most of the blood returning from the heart to the body is pumped back out without first going to the lungs to get oxygen. To correct this defect, surgeons perform an arterial switch operation in the baby’s first days of life, in effect “re-wiring,” or redirecting, the arteries to the proper ventricles.

For still other problems, surgery is unnecessary. Minor defects often resolve on their own, and others can now be corrected using catheter-based interventions, says cardiothoracic surgeon Winfield Wells of Childrens Hospital and the Keck School.

Just as adult hearts are repaired using catheters, a pediatric cardiologist can guide tiny devices through catheters to various places within the heart to open valves and close vessels and openings in the heart.

Many procedures that used to require open-heart surgery are now performed using catheters, Wells says, so children may enter and leave the hospital in the same day.

No matter what the defect is, the goal is to repair it as early as possible. In decades past, it was common for surgeons to perform only palliative cardiac procedures on infants, and then perform the total repair when the child was older. But that approach meant the baby’s heart continued to develop abnormally.

“We think intervening earlier is better,” says Keck School pediatrician Michael J. Silka, who is chief of cardiology at Childrens Hospital. “For the first year of life, the heart has a lot of ability to adapt and remodel.”

Jennifer can’t remember how long Ryan’s first surgery lasted: It seemed like forever. Finally, Starnes emerged from the operating room. Ryan wasn’t out of the woods yet, he had told them. The first 24 hours would be critical. But the surgery had gone well.

Two weeks later, they brought their baby home. Ryan was on so many medications that Jennifer had to create a computer spreadsheet to keep track of them. By this time, he was nearly 2 months old and weighed just 5 pounds, 8 ounces. Nurses assured his parents that Ryan would gain weight quickly.

They were right.

“It was like a miracle,” Jennifer says. “He just looked so much better after the surgery. It was incredible.”

Three months later, Ryan went back for the second surgery. This time, he was in the hospital for only four days. With all his health problems, Ryan was behind in his development. But with physical therapy, he caught up by the time he was a year old.

At age 2, he was still doing great, progressing along the path of a normal toddler. But soon it would be time for another operation – this one riskier than the ones before.

“It was very hard to put him through surgery when he was looking so great,” Jennifer says. “He seemed so healthy. But Dr. Starnes explained to us that [his heart] would eventually wear out. He needed the third surgery no matter how good he looked now.”

Two weeks after Ryan’s third and final surgery, he was back home. Once again, Jennifer used a spreadsheet to track his medications. She was exhausted from worry and lack of sleep. But for the first time in two years, no operations loomed ahead. Ryan was OK.

Brian Alessi is living proof of dramatic gains in pediatric cardiology: A survivor of several surgeries, he aims to be a cardiologist himself. Photo by Mason Poole

Since 1995, the survival rates for the hypoplastic right heart surgeries have improved about 10 to 15 percent, Starnes estimates. Survival rates for other surgical heart repairs also have risen steadily, and in some cases dramatically, over the past two decades.

For example, babies born with hypoplastic left heart syndrome, a related and more difficult-to-repair condition where the left side of the heart is underdeveloped, must also undergo a series of three surgeries. Ten to 15 years ago, babies had only a 50 percent chance of surviving the first operation, the most serious. Today, those chances are about 85 percent, Wells says.

There are several reasons behind the gains. Better surgical techniques and materials play a role, although the surgery itself is just one part of the equation. Also important are improvements in how to best care for these babies before and after surgery, including better ways to support their ventilation.

Congenital heart defects also are being diagnosed earlier – often before birth. Since the heart develops early in pregnancy, problems can be detected by the time the fetus reaches 18 to 22 weeks, and sometimes as early as 10 to 14 weeks, says Keck School pediatrician Mark Sklansky, who is director of fetal cardiology at Childrens Hospital.

If an obstetrician spots an abnormality in the fetal heart during a routine pregnancy ultrasound exam, the mother is referred to a specialist for a fetal echocardiogram. This targeted, high-resolution ultrasound study provides a detailed picture of the fetal heart, so specific defects can be identified, parents can be counseled and appropriate delivery plans can be arranged.

Sklansky also is involved in research looking at 3-D and 4-D images of the fetal heart. With this technology, a specialist can acquire a complete volume of the fetal heart in just two seconds, interactively visualize the heart from all viewpoints, including “surgeon’s eye” views, and then continue to evaluate the fetal heart after the patient has gone home.

Prenatal diagnosis helps parents emotionally prepare for having a seriously ill baby and gives them time to evaluate options and make important decisions about treatment.

It also prevents a baby with a serious heart defect from being sent home after birth, as Ryan was. Ironically, many babies with severe heart defects appear healthy at first. That’s because the heart’s circulation is different in the womb, where the fetus gets its oxygen from the mother’s placenta. The trouble often begins a few days after birth, when a passageway between the left and right sides of the heart closes, as it normally should in all infants. Once that happens, a baby with a faulty heart will start to struggle.

But fetal screening isn’t perfect. “While more obstetricians are looking closely at all aspects of the fetal heart during routine exams, many defects continue to be missed or are nearly impossible to pick up prenatally,” Sklansky says.

Three years after Ryan’s final surgery, a different kind of surprise came the Gates’ way: Jennifer was pregnant.

“We were ecstatic, but a little worried, too,” she says.

They had hesitated to have a third child, fearing that that baby, too, might be born with a heart defect or other problem. Doctors had told them the chances were slim, but it was still a risk.

If something was wrong, though, they wanted to be prepared for it. Jennifer’s doctor referred her to a specialist for a fetal echocardiogram. Everything looked good. “We knew it wasn’t a guarantee,” Jennifer explains. “But it definitely calmed our fears.”

A few months later, their baby girl, Erin, was born. She was beautiful, but they were taking no chances this time. Jennifer took her to a pediatric cardiologist for a thorough checkup.

The exam confirmed they had nothing to worry about. Erin had a healthy heart.

Although researchers and physicians have made huge leaps in treating congenital heart disease, there’s still room for improvement. One problem that remains is finding better heart valve substitutes for children.

Currently, when a child needs a new heart valve, surgeons insert one made from donated human tissue. The problem is that the valve does not grow with the child. So a child must endure major surgery every few years to put in bigger replacement valves.

“If you put one of these valves in someone over age 60, it’ll last a lifetime,” Wells explains. “Put that same valve in a 10-year-old, and you have to replace it in two years.”

To solve this problem, researchers at Childrens Hospital and USC are studying how to grow heart valves using the patient’s own cells. The valve would then grow with the child and potentially last a lifetime.

Also, as an alternative to myriad invasive surgeries, researchers in Europe have started to deliver heart valve replacements via catheters. And there is ongoing research into the genetics of congenital heart disease, with a few defects already linked to a particular chromosome.

“There’s probably a genetic marker for all of these defects,” Wells speculates. “Somewhere in the code, there’s a typographical error, but the relationships are complex. I think we will eventually find them, but it’s going to be in the distant future.”

When her son was a baby, Jennifer Gates used a spreadsheet to track all his medications. Today, Ryan takes an aspirin a day. Photo by Mason Poole

Ryan is now 10 years old. Since his last surgery at age 2, he’s never looked back. Today, he’s a healthy, outgoing fourth-grader and a good student. He likes golf and tennis and riding his bike and skateboard. He’s in the Boy Scouts and wants to go out for the volleyball team. His only medication? One low-dose aspirin a day.

“When he runs, he’s not as fast as the other kids,” Jennifer says. “But if you saw him playing on the playground, you would never know the difference. You would never be able to pick him out as the kid who had the heart problem.”

He doesn’t have specific restrictions, although doctors didn’t recommend contact sports like football or wrestling. His brother Connor, now 12, is a competitive swimmer, swimming 60 or more laps at a time without stopping. Doctors don’t recommend that for Ryan. The general guideline: He needs to take a break if he feels tired.

Intense team sports aren’t Ryan’s preference, anyway. His biggest love is acting. He’s starred in school plays and occasionally auditions for television commercials. He landed one national commercial last year.

One of the things everyone notices about Ryan? His happy, positive attitude.

“You kind of wonder, was that his nature at birth, and was that what got him through the surgeries?” Jennifer says. “Or is it because in his own way he’s so appreciative of what he’s been through?”

The family still lives in Tustin, and Erin is now 4. Jennifer is actively involved in the local schools, and also volunteers to counsel parents whose babies have been diagnosed prenatally with heart defects. Steve occasionally talks to parents with her, and he has his own financial services company.

The surgeries that Ryan went through are still too new for doctors to predict outcomes spanning an entire lifetime. But his prognosis continues to be good.

“We consider ourselves very lucky and blessed,” Jennifer says. “This whole experience changed our thinking a bit. You learn to take life one step at a time. You really appreciate each day for what you have.”

Katie Sweeney is a freelance writer based in Los Angeles. She graduated from the USC Annenberg School of Journalism in 1992.