The Open Heart Club

by Gabriel Brownstein

  Though she wasn’t interested in taking care of sick babies, she was interested in research on the heart. In Boston, she wrote to Park, “they have very kindly introduced me to another group of ‘cardiologists’ who meet one evening a week at Dr. Paul White’s laboratory.” That she put the word “cardiologists” in quotation marks begins to describe her skepticism but also the state of cardiac medicine at the time.

  Cardiology wasn’t a recognized medical specialty. In 1911, in Osler’s suggested list of hospital units, there isn’t one devoted to the heart. It wasn’t until 1914 in London that the first clinic specifically devoted to the heart opened. By the 1920s, new technologies—the EKG, the X-ray, and blood pressure measurement—were being used by doctors like Thomas Lewis in London, who was beginning to consider heart function and cardiac efficiency in clinical terms. Harvard’s Dr. Paul Dudley White brought this new cardiology to the United States. At one of White’s weekly meetings in Boston, Helen Taussig encountered a strange old woman: short, plump, rumpled, with her hair a mess, her dress stained with food, carrying various odd packages. At least one of those packages contained a very interesting specimen. A single-ventricle deformity? A tetralogy of Fallot? We’ll never know. In a letter to Edwards Park, Taussig wrote, “Last night there was a most interesting informal meeting in Dr. Paul White’s laboratory at which Dr. Abbott showed a most extraordinary heart.” Soon after, Taussig accepted the position at the Harriet Lane dispensary.

  Initially, the majority of the pediatric cardiology patients at the Harriet Lane Home were children not with congenital heart defects but with rheumatic heart disease. Before the widespread use of penicillin, strep infections frequently spread from the throat to the heart, and in children this could cause scarring and lifelong dysfunction of the heart valves. The devastation was widespread. A Depression-era study in New York City surveyed forty-eight schools and found sixty-eight special-ed classes devoted specifically to kids with scarred heart valves—at least 7,000 students in the New York public school system suffered from rheumatic heart disease.

  “Congenital abnormalities were the last thing in the world I expected to be interested in,” Taussig once explained. “I started with a busy rheumatic clinic.… It [congenital heart disease] fell on me—or I fell on it.”

  Abbott had published her much-praised encyclopedia article on congenital heart disease in Osler’s A System of Medicine, but not much else had been published on the subject. In the late 1920s, the literature on congenital heart disease was both ancient and foggy, going all the way back Steno, but still no one was trying to cure kids with defective hearts.

  Park invested in the clinic. He made sure it had access to X-ray and EKG equipment, and he procured a fluoroscope, the latest in diagnostic technology: a fluoroscope projected a continuous X-ray image of the heart onto a fluorescent screen, providing a rudimentary movie of the heart.

  “Dr. Taussig,” Park told her, “you are now going to learn about congenital heart malformations.”

  Taussig remained ambivalent. She told Park that she didn’t particularly want to learn about them.

  He said, “It doesn’t make any difference how you feel, you are going to learn about them.”

  In an interview, she remembered, “I vowed then and there not to get trapped in this ‘narrow’ field.”

  The congenital defects were incurable, but Taussig treated those patients as she did the ones with rheumatic heart disease. She did EKGs and X-rays. She fed them pudding containing barium, set them in the fluoroscopy machine, and ran the machine herself. She became intrigued, intellectually, by their conditions. Taussig called her patients the “crosswords of Harriet Lane”: they were little puzzles that no one else could solve. In the 1930s, there was no system for diagnosing children with congenital heart disease—which child had transposition of the great arteries, which had tetralogy of Fallot, which had a septal defect, which had a stenotic valve. There was no clinical reason to get an accurate diagnosis, because the children could not be cured. In 1931, when Abbott first presented the exhibit that would become her atlas at the Graduate Fortnight at the New York Academy of Medicine, Helen Taussig was there. (They were two of only four women’s names I found in a program that includes at least a hundred doctors.) Taussig traveled to Montreal to look at Abbott’s collection of hearts.

  She began to notice patterns. She began learning about the size and shape of the hearts. She learned to distinguish between the babies with transposition of the great arteries—they had essentially two separate circulatory systems—and the other babies who were not getting enough oxygen in their blood.

  But just as she was committing herself to a lifelong study of congenital heart defects, something awful happened. Taussig’s hearing, compromised all her life from a case of whooping cough when she was two, began to desert her. Birdsong was lost to her. She found herself turning up the volume of her radio and then creeping closer to hear it. She still played her music, pressing her fingers to furniture to feel the vibrations of symphonies. Soon, she couldn’t hear other people’s voices. She started wearing a hearing aid—in those days a bulky vacuum tube device that hung on a chain like a necklace. She struggled to hear her patients’ heartbeats. She tried to use a specially amplified stethoscope, but it was unsatisfactory.

  Frustrated, she approached the children’s chests as she had the sound of the radio. She came closer. She pressed her hands to their skin. She touched each patient gently, sensitively, from head to toe. She taught herself to listen to their heartbeats with her fingertips. Eventually her touch became more effective diagnostically than a stethoscope. She learned to lip read. She sat up front at conferences. Names and numbers were the hardest things for her to follow, so she asked people to write them down. Neither her deafness nor her dyslexia got in the way of her accumulation of knowledge.

  Her focus in the mid-1930s was on the cyanotic children, the blue babies, the ones who did not have enough oxygen in their blood. She was particularly fascinated by the children with tetralogy of Fallot. She noticed that some tet kids did better than others, and strangely it wasn’t the kids with healthier hearts but the children with an additional cardiac deformation, yet another hole in their circulatory system, something called a patent ductus arteriosus (PDA).

  In the womb, the fetus swims in amniotic fluid. It doesn’t breathe. Its lungs are closed. The placenta is highly vascular, and oxygenated blood comes in through the umbilical cord. This is why a fetus can survive with the most grotesque heart defect. Right up to birth, babies depend on the mother’s cardiopulmonary system for oxygen. Every child is born with two holes in the heart, conduits that divert blood from the useless fetal lungs. As the infant breathes, the heart matures, and these holes close.

  One of these holes is at the top of the heart, between the two atria, and it’s called the Foramen ovale. In most infants, the Foramen ovale closes in the first weeks of life, but a substantial minority (about 25 percent of the population) live with a patent, or open, Foramen ovale. According to the American Heart Association, a patent Foramen ovale is not a serious concern: “It causes no adverse health effects.”

  The second conduit is the ductus arteriosus. This runs between the pulmonary artery and the aorta. In most people, this closes in the first fifteen hours of life, but in some babies it can take as long as three months. A patent (or open) ductus arteriosus is common in premature babies. Most children with small PDAs live without symptoms until the ductus closes. On the other hand, a large, chronic, untreated PDA—one that never closes—means a slow, painful death.

  Some kids were born with both a tetralogy and a PDA. Taussig followed these cases closely. She felt the children from head to toe to see how their hearts were beating, how their bodies were swelling, and how poor their coloration was. She noted their heart rates and blood pressures. She took EKGs and X-rays. She fed them barium pudding, set them in the fluoroscopy machines, and took movies of their hearts. Then she put them in their cribs and described their worsen
ing conditions.

  There are two available book-length biographies of Helen Taussig. One, To Heal the Heart of a Child by Joyce Baldwin, is a book for intelligent young readers. The other, A Gentle Heart by Geri Lynn Goodman, is a scholarly dissertation available through Yale University’s website. Both writers interviewed Taussig when she was older, and both present her as a sweet and kindly woman with a soft spot for kids. This is not how the people I interviewed remembered Taussig.

  Dr. Eugenia Doyle, a student of Taussig’s at Johns Hopkins and later Michael Freed’s mentor at NYU, found Taussig forbidding: “Not the kind of person you’d like to wrap your arms around, not a warm person, very cerebral.” Doyle’s abiding image of Taussig is of a doctor looking at corpses, “poring over the autopsies” of kids with congenitally deformed hearts. For her colleague Abraham Rudolph, Taussig was “an interesting character, very proud of herself, somewhat isolated, someone remote.” I asked Dr. Rudolph if he thought this remoteness had something to do with her deafness, and Rudolph agreed that probably it had. To Sylvia Griffiths, Taussig was a New England aristocrat. “Think Barbara Bush without all the makeup,” she said. Taussig was famous for her devotion to her patients, her students, and her discipline but not for her personal warmth. This was a formidable woman, as determined in the face of incurable disease and male-dominated medicine as her grandfather Taussig had been in riding out into the Missouri wilderness alone, ready to face down roving gangs of bandit terrorists in order to rescue a bunch of mental patients in need.

  Through relentless analysis, Taussig discovered that the tet kids with PDAs did better than tet kids whose patent ductuses had closed. They had more energy. Their skin was a healthier color. Some of them were nearly asymptomatic. She watched her patients over long periods. Taussig was famous—even to the point of being resented by other doctors—for her persistent attention to her patients, her possessiveness of them, and her fascination with their cases.

  As the tet babies grew, some of them grew out of their PDAs. The ductuses closed naturally. When this happened, the kids didn’t get better. They got worse. They got bluer. They got less energetic. With an open ductus, a tet kid had a heart murmur but good coloration. With the ductus closed, the kid got very sick. Kids with tetralogies or chronic PDAs didn’t do well. Kids with tetralogies and chronic PDAs did better.

  Taussig saw that the PDA was in some ways compensating for the tetralogy. She speculated that the diversion of blood from the aorta back into the pulmonary artery meant that more blood was going to the lungs, and this in some ways made up for the oxygen deprivation that came from the hole in the center of the tet kids’ hearts and for their pulmonary stenosis. The tet kids with PDAs were getting more oxygen in their blood than the tet kids without.

  In 1938, about the same time that Taussig was collecting the data about her tet patients with PDAs, she learned that a surgeon in Boston, Dr. Robert Gross at Boston Children’s Hospital, was beginning to put his scalpel in places no one had ever dared. He was not operating on the heart itself but right above it. He was curing children with PDAs. He was sewing shut the ductuses.

  So Helen Taussig made a wild leap of imagination: she imagined a surgeon cutting into the chest of a dying baby, a baby with a tetralogy of Fallot, and she imagined that surgery giving the infant yet another defect, a man-made PDA. When she first began to describe this idea to people—giving a dying baby one more birth defect—they thought it absurd. They thought that Helen Taussig had gone out of her mind. She went to Boston to see Gross to see what he would say.

  22.

  ON SUNDAY, JUNE 14, 2018, I drove out to St. Stephen’s Lutheran Church in South Plainfield, New Jersey, to see Pastor Chris Halverson at work. He’s in his mid-thirties, very blond, very fair, and physically quite small. His face is triangular, and his manner is reserved. He’s balding and youthful, and he has bright blue eyes. His church sits on Park Avenue in South Plainfield, a long stretch of two-lane blacktop lined with modest houses and square little lawns. St. Stephen’s is set back from the road. The main building of the church complex was built in 1960, and it has a Jetsons-era swoop to its roof, high on the sides and low in the middle (stylish, said Chris, but a nightmare, drainage-wise). The church interior is simple, with an open bare-beamed vault. The unpainted wood shines a golden yellow. The low windows of the clerestory fill the room with light. Cheerful green and white banners hang at the back walls of the chancel.

  I first visited on a cold Sunday during Martin Luther King Jr. weekend. One musician in a camel hair coat moved from the organ to the piano and back. A choir in purple robes gathered to the pulpit’s right. Some families brought their children, and some middle-aged folks came alone. There were a lot of older women in cardigans, trifocals, and short hair. When I’d first met Chris, months before, he’d been wearing a shirt celebrating the NPR program The Moth, but now he was in his vestments. His hair had grown long in the back, and his pale beard was big and bushy, the beard of a nineteenth-century American preacher.

  That weekend, the sexual assault scandals that had been sweeping the nation had hit the Lutheran Synod of New Jersey. A colleague of Chris’s had recently been disbarred, and as pastor Chris took the news to his flock. The day’s readings were from 1 Corinthians, Samuel, and John, and Chris’s thoughts went to the apostle Paul. As Chris explained to his quiet, conservative, suburban congregation, body and soul were inseparable. The violation of the body of a child was not just a crime but a blasphemy against God. “Bodies matter,” he said, and he reminded them of the surgeries he had been through on his heart.

  Chris shut his eyes. His childhood surgeries had been traumatic, he explained, but beneficial. His doctors had cared for him. He tried to imagine—he said he could not—what such a violation would have been like had it been malicious. His congregation became still and very somber. I thought of the scars on his chest, underneath his robes.

  Chris was born in Fargo, North Dakota, in 1983 and turned blue soon after birth. Surgeons put a shunt between the great arteries above his heart, allowing blood to flow into the pulmonary artery and to the lungs, and so he was able to survive until the age of three, at which point he had surgery at the Mayo Clinic. Chris’s diagnosis—pulmonary atresia with ventricular septal defect—is different from mine. Though we were both born with a hole in the middle of our hearts, people with Chris’s condition lack a pulmonary valve entirely. For Chris, a conduit had to be implanted between his right atrium and right ventricle, and the surgery when he was three years old was successful, a “complete repair” like mine. His parents assumed that meant Chris was all better, and off they went, out into the world.

  Chris had a poor sense of his body’s strength, so typical of patients with congenital heart disease. When he was little, between the ages of three and nine, his heart condition posed few difficulties for him. “I felt like I was in really good shape,” he told me. “I was doing everything you do as a kid.” His grandparents lived in Minnesota, and each year his parents went for a visit; then after the family gathering, Chris and his mom and dad would head to Rochester, to Mayo. The phlebotomy lab was always torture. Chris’s veins are small, and they tend to roll. They’re hard to puncture. The technicians would show him Disney movies while they took his chest X-rays and did the EKG and the echocardiogram. When he was nine years old, the tests revealed a crisis.

  “I never have a sense that there’s anything wrong with my body,” Chris told me, “until they’re cutting me open and doing their thing.”

  Before he really understood what was happening, he was waking up from surgery with an IV dripping into his arm and urine flowing out through his catheterized penis, the world a fog of painkillers and antiseptic smells. As the tubes were pulled out from deep within his chest, they felt like long balloons in a magician’s trick. The split bones of his sternum hadn’t yet knit as he and his parents drove back through the days and nights to Wyoming. His little chest had surgical tape covering the stitches of the incision. There were ba
ndages and sutures in his groin and bruises on his arms.

  They got back home to Cheyenne. Chris, haunted by his near-death experience, was asking profound questions of his mom and dad. What was life all about? After he died, what would happen to him? His parents, both agnostic, refused to give an answer. Could be Buddha, they said, could be Krishna, could be the Culligan Man. So as soon as his bones knit and he was able to walk by himself, the boy began to look for answers. He prowled the bookshelves of the Salvation Army in Cheyenne, dipping past the clothes and cookware to the rows of yellowing paperbacks, squeezing his way through the book sections until he got to religion, and then picking up anything that looked interesting.

  “Some pretty weird shit,” he told me, “in retrospect.”

  Religion, among the kids on the school bus, meant fundamentalist Christianity. “Fundamentalists,” Chris told me, “really love selling answers, even when you’re not asking questions.” Without his parents, he started attending a Nazarene church. The Nazarenes urged Chris to renounce his mother and father as heathens. Chris’s father’s response to this was simple and brilliant. He gave the boy a Bible, which Chris began to read. What he saw there was far different from what the kids on the school bus were telling him.

  “I simply read the book,” Chris said. “It was weird. I fell in love with the Bible before I realized there was a community attached to it.”

  He kept attending the Nazarene church, despite the gap he felt between what was preached there and what he saw in the gospel. He felt strong and well. Then, when he was eleven, his mother enrolled in a program in San Antonio, Texas, to get a public health degree to complement her expertise as a pharmacist. The Halversons’ plan was to drive to Minnesota, visit the grandparents and check out Chris’s heart, and then drive from there down to San Antonio. At Mayo, Chris was scheduled for a cardiac catheterization.