As a result, pediatric heart surgeons may do 30 times as many kinds of operations as adult heart surgeons. Plus, while adult cardiac surgeons mainly work on the heart, pediatric surgeons generally operate in the heart. And a child’s heart can be smaller than a walnut.
Every pediatric heart surgeon starts as an adult heart surgeon. “If you do a good job, then you get to be a pediatric cardiac surgeon,” says Dr. Ralph Mosca, 43, Quaegebeur’s partner. “If you don’t, you’re back to being an adult cardiac surgeon.” In New York State, a dozen hospitals do pediatric heart surgery, though Quaegebeur says “some shouldn’t.” He’s probably right. In the hands of some New York surgeons, nearly one in twelve pediatric heart patients will die. “I call them sportsmen,” says Quaegebeur.
NewYork-Presbyterian is one of a handful of elite programs. This year, its surgeons will do 600 operations, making the hospital the state’s busiest facility. It’s also the best, with New York’s lowest mortality rates (adjusted for case complexity).
The stakes, of course, are immeasurably high. “If you fix a 6-month-old, you add 80 years to her life,” says Chen. As if to illustrate the point, the pediatric-heart-surgery suite features snapshots of healthy kids who were once deathly ill. There’s the double-black-diamond skier and the new college grad and the kid in colorful trunks on the beach. “Maybe it’s a cheap heart-tug,” says Chen. “But it’s easy to feel like you want to save children.”
Quaegebeur, 57, is of the generation that helped create modern pediatric cardiac surgery. Short and trim, with a thick head of Ronald Reagan–esque hair, he was born in Belgium and trained in the Netherlands, Boston, and Houston. From the start, he had good hands. “You hear somebody saying it, and then, of course, you believe it,” he says. He’d already trained to be a general surgeon, then a vascular surgeon, then a heart surgeon, when his mentor in the Netherlands, as Quaegebeur says, “pushed me into this congenital business.” This was in the seventies. Columbia recruited him in 1990 because, as then–department head James Malm says, “I was told he was the best pediatric cardiac surgeon in Europe.”
Despite his imposing reputation, Quaegebeur has an almost shy presence. His small stature (he’s five feet six inches at most) invariably makes him the shortest man in the OR, and he still misfires on the occasional English expression (“Umpossible,” he says). He doesn’t travel with a mob of residents in tow, and “gee” is an expression he uses to introduce a thought. When not in the OR, he has a tendency to vanish, disappearing down the stairs in his OR clogs, too impatient to wait for the elevators.
Quaegebeur loves New York—he lives on Riverside Drive and has a house in Sagaponack—and he collects wine (mainly so he can afford to drink good wine) as well as contemporary art. “There’s too much art in our apartment,” says his girlfriend, Annetine Gelijns, a scientist at Columbia (Quaegebeur is divorced). He also dines regularly at Jean Georges and is an ambitious cook—he hopes someday to spend a week in the Jean Georges kitchen learning culinary technique the way he once learned surgical technique.
Still, Quaegebeur says, “Operating is the core of my life.” Indeed, he does 300 operations a year, which means he’s literally in the operating room most daylight hours (he usually skips lunch). He operates at Stony Brook and Cornell, and every couple of months he flies to Europe to operate. He’s delivered lectures on surgical technique only to have his hosts turn to him and, impromptu, ask, “Doctor, we have a difficult case, would you mind operating?” And he will. He also takes cases others refuse. Once, a surgeon had repaired a 12-year-old’s leaking heart, but within a month the heart became infected. The kid’s aorta was rotting. His surgeon refused to reoperate. “He said it was too hard for him,” the patient, now 22, recalls. So in the middle of the night, the patient was shipped to Presbyterian. “You don’t let a patient die,” says Quaegebeur. “You can be wrong. Things don’t always work out. But if you don’t try, you have no chance.”
Before Quaegebeur got involved in the field, heart surgeons hesitated to operate on very young kids. Newborns, it was thought, were too fragile to undergo open-heart surgery. Instead, surgeons palliated and waited. Unfortunately, waiting to operate had high costs. As these kids grew, their diseases undermined their hearts—walls thickened, muscles weakened. Surgeons still occasionally see an older kid who’s survived congenital defects without surgery, and it’s not a pretty sight. “They’re just so blue and their blood is so thick and sludgy that doing anything is very difficult,” says Mosca.
For Quaegebeur, the imperative to operate early crystallized in the mid-seventies with a specific defect. In some instances, the arteries that should go to a child’s lungs connected instead to his aorta, the big vessel that feeds blood to the body. Basically, the kid’s system was backward. The solution was obvious. You had to switch the arteries—and you had to do it immediately.
“The old guys would say, ‘In a neonate?’ ” says Chen, taking their scandalized tone. “And Dr. Q would say, ‘Sure, why not?’ ”
Others saw reasons. The operation was considered extremely difficult, if not impossible. A British cardiologist writing in a British heart journal even called it “unethical.” There were alternative operations to offer these kids. They might not cure, but at least the procedures killed only 5 percent of patients.
Quaegebeur’s initial mortality rate in this procedure was 30 percent. Still, he pressed ahead, spending hours in the lab studying some 7,500 defective hearts in jars. And whenever he did an operation, he stayed up all night, hovering over the recovering child in the ICU. Killing kids is depressing. But Quaegebeur selected the most difficult cases, those who seemed the least likely to survive without intervention. He reasoned that he was, in effect, their best shot. Plus, he says, he was “absolutely convinced that the mortality rate would come down quickly.” Unethical? That was shortsighted, he says. “Just because you failed, you’re not going to say, ‘Sorry, I won’t do it again.’ You learn from it. We were sure we were doing the right thing.”
By the early eighties, Quaegebeur had brought down the mortality rate dramatically—to close to 5 percent (today, it’s closer to 2 percent). He does 60 percent of his operations in the first three months of life, and the “arterial switch,” as his pioneering procedure is called, is his signature operation. “He does this operation that takes most people twelve hours in two hours and fifteen minutes,” says Chen. “The result looks like it should have been that way in the first place. It’s like watching Tiger Woods.”
Raising a child with a heart condition hasn’t been easy. For one thing, it proved impossible to find a nanny. So Kimberly has stayed home with Dorothy for most of the past three years. She works part-time as assistant to a real-estate developer, a job she sometimes does from their snug apartment. Kimberly and Dorothy share the bedroom.
“I want my own room,” says Dorothy.
“So does Mommy,” says Kimberly.
For a time, Kimberly took care of a couple of kids after school, which was nice because Dorothy had built-in playmates, and Kimberly earned a little extra money. This year, Kimberly found a preschool in the neighborhood where she sends Dorothy a couple of times a week. Dorothy seems to fit right in. She thinks of herself as normal, and mostly she is.
When she’s not in school, Dorothy likes to watch movies. She loves The Wizard of Oz, and she’ll act out the parts. She plays Glinda. Her amiable mom plays the Munchkins. “Motherhood,” says Kimberly, “ is the job I seem to excel at.”
By the time Quaegebeur enters the operating room toward noon, Dorothy’s gown, the yellow one decorated with clowns, has been removed. Her eyes have been taped shut, her 36-inch body turned into a workplace. Ten people scurry around her, inserting tubes down her nose, into her lungs, into her bladder, a couple into blood vessels, which in small kids sometimes have to be located by shining light through the flesh. Her procedure is scheduled to take about four hours.
The first assistant, an adult cardiac surgeon training to be a pediatric surgeon, paints Dorothy from groin to neck in Betadine, the iodine-based disinfectant that looks like orange war paint. A cardiologist slips a probe with a tiny camera down Dorothy’s throat, setting the device in position in the esophagus just behind her heart.
To one side, a stack of rolling monitors registers blood pressure, heart rate, and half a dozen other functions. To the other, perfusionists prime a cardiopulmonary-bypass machine with blood donated by Dorothy’s mother. The bypass machine is five pumps, each as big as a car battery—“the one on the right is the heart,” explains the perfusionist in charge, who sometimes refers to himself as a member of “the pump team.”
The team pulls dark-blue sterile drapes over Dorothy, focusing attention on the only bit of real estate that matters for the next few hours: the slender bit of chest that peeks through.