When Your Child is Born with Congenital Heart Defects
Keely O'Brien was just 2 weeks old when a cardiologist told her parents she wouldn't survive without surgery
Photo by Lisa Helfert
Just shy of 2 years old, Keely O’Brien knows what she wants, and on this November evening in the kitchen of her family’s Glen Echo home, she wants to put a bowl of popcorn upside down on her head.
Her parents, Jenny and Andrew, laugh as the popped kernels get caught in her blond curls. “She’s a wild and crazy precocious toddler,” Andrew says. “A spitfire.” Jenny nods. Keely’s an adventurer, her mother says, prone to frightening other parents at the playground as she totters perilously at the top of the slide. Her daughter is also a fighter. She ought to be—at just 10 weeks old she fought for her life.
Looking back, the O’Briens say, they can see the warning signs. They’d left the hospital thinking their new baby was fine, but she was unusually lethargic at home. They hoped it meant that she was just a good sleeper, unlike her older brother, Liam. Their son, 3 years old at the time, was a voracious eater when he was born, but Keely barely nursed. Could it be that she just wasn’t hungry? “It just didn’t seem normal to me,” says Jenny, 35.
At Keely’s two-week checkup in January 2015, the pediatrician listened to her parents’ concerns and also heard something troubling. Holding a stethoscope to Keely’s chest, the doctor noticed the whisper of what she thought might be a heart murmur. It’s probably nothing, the couple remembers her saying, but she urged them to see a specialist just to be sure.
The next day, a cardiologist performed an echocardiogram, moving an ultrasound probe over Keely’s body to produce an image of her tiny heart. There, in the middle of the newborn’s heart, he saw a hole where the upper and lower chambers meet, and a second one just below it. The condition, called complete atrioventricular canal defect (CAVC), occurs in one of every 5,000 births, according to the National Institutes of Health. Instead of having two valves separating the heart’s chambers, the doctor told them, Keely had just one. Without surgery, she would die.
The cardiologist referred Keely to Dr. Richard Jonas, chief of cardiovascular surgery at Children’s National Health System in Washington, D.C., and two weeks later her parents were sitting in his office. Jonas, who helped pioneer open-heart surgery on newborns, told Keely’s parents that he wanted to operate on their daughter, but that she should get a little bigger first. For more than two months, the O’Briens waited as Keely put on weight and her heart valve tissue grew thicker and stronger. “It felt like an eternity,” Jenny says.
Keely O’Brien, pictured with her parents and her brother, Liam, underwent a three-hour operation on her heart when she was a newborn. Two years later, her father, Andrew, describes her as a “wild and crazy precocious toddler.” Photo by Lisa Helfert
The family stayed inside as much as they could, hoping to prevent Keely from catching even a cold. Andrew, a partner at DCI Group, a public affairs consulting firm in D.C., took Liam to his fourth birthday party while his wife and daughter stayed home. Keely’s parents kept track of how much she ate and tried to bulk her up by supplementing breast milk with infant formula. “You do a lot of praying,” says Andrew, 35, “and hand-holding, and crying, and trying to look for the silver lining.”
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Every year, more than 35,000 babies in the United States—about eight of every 1,000—are born with congenital heart defects, according to NIH. In some cases, these are minor problems that don’t need medical attention. Even some holes in the heart can repair themselves over time. In other cases, like Keely’s, the defects are life-threatening.
Keely’s condition caused oxygen-rich blood on the left side of the heart to mix with oxygen-poor blood on the right, resulting in a lack of oxygen in the bloodstream. Her heart was pumping too much blood back to the lungs, which forced it to work harder than a normal heart. Left untreated, Keely’s heart would have become enlarged and damaged. The newborn also had another problem. Her main artery was too narrow—the result of a congenital heart defect called coarctation of the aorta—which was blocking normal blood flow in her body.
According to the Mayo Clinic, coarctation of the aorta may not cause any symptoms, and mild cases might not be diagnosed until adulthood. The condition often occurs along with other heart defects, as it did with Keely.
A generation ago, a patient like Keely probably would have died, Jonas says. Doctors first operated on a child with CAVC in 1954, but it wasn’t until decades later that better technology and scientific understanding made corrective heart operations on newborns more feasible, he explains. The advent of the heart-lung machine, called a cardiopulmonary bypass, in the 1950s made it possible to stop the heart without the patient dying. The discovery in the 1970s that the compound prostaglandin could keep babies with heart problems alive until surgery increased the chances of survival. In the 1980s, improvements in echocardiography and MRI technology helped give doctors a clearer picture of pediatric heart defects.
“It was really in the 1980s that we started to learn more about how to operate on her condition and started to develop better techniques,” Jonas says. Since then, refinements in surgical techniques have helped doctors repair increasingly complex heart problems.
In the early days, Jonas says, surgeons waited as long as they could before operating. Now they’re performing surgeries early to avoid complications. Damaged hearts often work harder, Jonas says, and blood that’s too low in oxygen thickens and can cause brain damage. “That’s been a philosophy that I brought to this hospital when I came here in 2004,” says Jonas, who lives in Chevy Chase. “There’s been a gradual transition that’s occurred since 1983, a gradual shift to operating younger and younger.”
Today, he says, most neonatal heart procedures carry little more than a 1 to 2 percent risk of death. Adorning his office are holiday cards and photos from the families of children he’s treated. Jonas takes a card off the shelf from a patient he operated on more than a decade ago. “She’s doing amazingly well,” he says of the girl, who spent months in the hospital after a complicated surgery that required a breathing tube. She’s in high school now—a dancer, he says. Parents often write to tell him about their kids’ ballet performances or sports successes.
“It’s like you have a whole extended family,” Jonas says with an easygoing Australian accent. Nearly 10 years ago, he performed successful open-heart surgery on Fox News anchor Bret Baier’s son Paul, who was born with five congenital heart defects; Jonas and Baier have since become friends. “In adult surgery, you’re operating on 70-, 80-, 90-year-olds. When you operate on a newborn, they’re in touch with you, or their parents are, for years,” he says.
Born and raised in Adelaide, Australia, Jonas studied in Melbourne and in Auckland, New Zealand. He had just become a fellow at Harvard Medical School and Boston Children’s Hospital in January 1983 when he joined a small team that assisted two surgeons as they successfully completed an “arterial switch” on a newborn. The delicate procedure to correct a misplaced aorta and pulmonary artery had never been performed on an infant. It was like watching history being made, says Jonas, the author of a textbook called Comprehensive Surgical Management of Congenital Heart Disease. The procedure opened the door to surgeons being able to successfully operate earlier on babies who previously might not have survived, he says. Now, improved ultrasound technology allows doctors to spot heart defects in many babies before they’re born and to operate on them in the first weeks of life.