The Man Who Touched His Own Heart

by Rob Dunn

  Taussig felt she had a duty to chronicle heart diseases so that, if someone ever discovered a way to cure one, the curable disease might be diagnosed in other patients. By 1944, building on Abbott’s work, Taussig had compiled a relatively complete catalog of congenital heart diseases, including the signs of each—the tracks left on the body by the disease’s effects on the heart. Her book became the first standard book in pediatric cardiology. As one of her colleagues, Carleton Chapman, would later say, “That book made all the difference. It brought congenital heart disease out of the fairy land” and into the realm of treatment. Some defects, she argued in her book, could always be predicted based on symptoms. Others could not. The cause of the blue skin of blue-baby syndrome could be predicted; it was nearly always due to the four deformities of the tetralogy of Fallot.

  As of the publication of her book in 1944, Taussig could reliably distinguish the tetralogy of Fallot from other diseases that cause the skin to turn blue, but there was still nothing she could do once she made the diagnosis. She braced the parents and waited for the body. It was about this time when she heard some discussion about surgically sealing holes in the heart. She wondered about the possibility of doing the reverse, opening more holes, “arteries” of some sort that could lead from the heart to the lungs. These were the exciting boom years of heart surgery—the heart-lung machine was on the horizon—and Taussig was attuned to the possibility that some of the new approaches in surgery might be used to save the children dying all around her.

  Most of the heart surgeons racing toward progress focused on adults; adult hearts were bigger and easier to operate on, even if the gain would typically be years rather than, as might be the case in children, whole lives. With a few prominent exceptions, children were operated on only in special cases, when a new technique and a failing child happened to coincide. Taussig was not waiting for coincidence. She wanted to operate on tetralogy sufferers; she just needed to find someone to actually perform the surgery—someone who had the knife skills and could figure out what exactly the surgery should be.

  Taussig traveled to Harvard to consult with a surgeon who had done an operation in which he had closed the ductus arteriosus in the heart of a young patient. The ductus arteriosus is a small passage from the vessel carrying blood to the lungs, the pulmonary artery, to the vessel carrying blood from the left side of the heart to the rest of the body, the aorta; it is open in the fetus and helps to shunt blood away from the lungs (which serve no purpose in the fetus). But in some children, the ductus fails to close, and oxygenated blood coming from the lungs pours back into the pulmonary artery (and hence goes through the lungs again). In 1938, Robert Gross, a resident at Children’s Hospital in Boston, had performed a surgery in which he closed a ductus.8 Gross declined to help Taussig, accusing her of idiocy for even asking (which redoubled her resolve). Taussig returned to Johns Hopkins, where she persisted with her idea, so much so that other colleagues blamed her stubbornness on her deafness. Perhaps she literally didn’t hear the word no. It would take another two years before she found someone willing to try her new procedure.

  The man who would do it was Alfred Blalock. When he joined Johns Hopkins, it seemed like he had been sent by the Fates. He had the skills to try a surgery, and, inspired by Taussig, he had an idea about just which one. He could, he thought, take one of the arteries traveling from the heart to the body and reroute it to the lungs. But before he tried, he wanted to perfect the procedure on animals. For that, he turned to his assistant, Vivien Thomas, who developed the procedure, and then, over three long years, repeated it in dog after dog until it seemed just right. It was after these surgeries, after Taussig’s two years of finding no help, and then after three years of waiting for Thomas to perfect things, that Taussig, Thomas, and Blalock stepped into the room with the little girl, Eileen.

  Eileen’s parents wanted their daughter to live. The surgical team wanted the hundreds of thousands of blue daughters and sons to live. But a dog heart is not a human heart, and so the truth was that the team had no real idea whether their new procedure would work.

  On the operating table, Eileen Saxon was too little to want or even really understand. She suffered but had never known anything different. She stared up at the lights and then fell asleep as the ether kicked in. Standing above Eileen’s body, Blalock cut a smile-shaped incision, beginning below her right armpit into her chest and going up between her third and fourth ribs, before pushing apart her ribs. This alone was a challenge. She was so small that Thomas had had to devise not just one but several new tools and approaches to fit her body and the circumstances. Once her heart was exposed, he could clearly see it beating. The tiny heart was all wrong. Fortunately, thanks to Taussig’s work, it was precisely the kind of all wrong they had anticipated. Blalock clamped both ends of her pulmonary artery and then did the same for the subclavian artery traveling out toward the body. In the former, he cut a small hole into which he inserted the latter. He stitched the two arteries together with beaded Chinese silk. Suddenly, two riverbeds were joined, and twice as much blood flowed to Eileen’s lungs—hopefully enough to move more oxygen through her blue body. He then used the silk thread to reconnect the ribs, applied sulfonamides to the body cavity, and, layer after layer, stitched the tissues back the way they had been. After the surgery, when the three doctors stepped back, Eileen’s heart kept beating, and, as it did, her body began to turn from blue to pink. As Eileen’s mother would later say, “When I saw Eileen for the first time, it was like a miracle… I was beside myself with happiness.” So were Taussig, Blalock, and Thomas. The surgery had worked.

  Tragically, Eileen died three months later of other complications of her heart’s complex deformity. The surgery had prolonged her life, but not by nearly enough. Technically, though, the surgery did what it was supposed to do, and so it was tried again. The second surgery went well but again proved to be a relatively short-lived fix. It was on the third try that everything worked out the way everyone had hoped. The patient was, as Taussig commented, “an utterly miserable, small six-year-old boy… no longer able to walk.” Blalock performed the surgery on him, with the same drama as the first—the same anxious parents, the same trembling child. The boy turned a lovely color, with “lovely normal pink lips.”9 He woke up and looked at Blalock. He blinked his eyes and said, “Is the operation over? May I get up now?” He could. He got up in the next days and weeks that followed and became a happy, active child; he went on to have a full life—one given to him by Taussig’s idea, Blalock’s hands, Thomas’s practice, and Eileen’s tragedy.

  Word of the surgery’s success spread. Just two years later, the procedure had been performed hundreds more times.10 One British cardiac surgeon, Sir Russell, called the operation “so outstanding that it altered the whole approach to cardiology.”11 Blalock had become famous, though at a time when fame for physicians was still regarded as unethical in the United States, just as it had been in Germany for Werner Forssmann and as it would later be, to a lesser extent, for John Gibbon. This fame simultaneously delighted and worried Blalock so much that he attempted to resign. Thankfully, his colleagues convinced him to continue working. As a result, thousands were saved. Children were brought and sent by parents to Johns Hopkins so that they might be born again with mended hearts. Hundreds of parents wrote Johns Hopkins, begging for their children to have the surgery, which would prove to be successful in roughly eight out of ten cases. Today, 90 percent of babies born with the tetralogy of Fallot will live out normal lives. Hundreds of thousands of adults walking among us are alive thanks to the team at Johns Hopkins.

  Taussig, Blalock, and Thomas would all become known for the tetralogy surgery. In every description of the events that led up to the new procedure, different emphasis tends to be put on the contribution of each of the three individuals (the approach is often called the Blalock-Taussig procedure, sadly omitting Thomas entirely), but the truth is that they were a team made up of individuals outside the mainstream of h
eart surgery who became great in the context of one another. Other surgeons would try to improve on the surgery (and name the “improved” versions after themselves), but the version that Taussig, Blalock, and Thomas pioneered persisted for years. It was replaced only when more elaborate procedures (in particular, the ventricular-septal defect patch repair and artificial shunt) were made possible thanks to Gibbon’s heart-lung machine.

  For Taussig personally, the surgery was one success among many for which she laid the groundwork. She studied children’s hearts when everyone else ignored them. She listened to children, reading lips when she had to. She valued diagnosis when others thought it useless, and she was willing to do the tedious work. This work acquainted her too well with the death of children, but because of it, she made possible the greatest successes in heart surgery—the tetralogy surgery, but more than that, an entire array of interventions so successful that if a child is born with a congenital heart defect today, he or she now stands an 80 percent chance of having a normal life span. Taussig’s success inspired others to establish pediatric cardiology wards all over the country. She helped to fund these centers through an appeal to the National Institutes of Health and the Children’s Bureau, and she trained the next generation of pediatric cardiologists and surgeons, nearly one hundred in total, many of whom were women.12 Taussig persisted as a scientist,13 as a leader (she would become the first female president of the American Heart Association), and as a mentor, but surely her most conspicuous legacy is her living one, the adults whom she helped heal as children.

  Most heart procedures gain patients a few years, at most a few decades; Taussig’s legacy has given whole lives. If there is a pinnacle to the story of the heart and our attempts to understand it, this is it: the hundreds of thousands of people born with heart defects who walk around us every day, looking no different, feeling no different—who are simply, miraculously, alive.

  But this is not the end of Taussig’s story. She did not, as one biographer put it, go gentle into the twilight. She did not know how. A 1968 painting by James Wyeth shows her at the age of seventy, white-haired, cloaked in a dark dress, and lit from the front as if traveling into the glow of yet another new discovery. Even after she moved into a retirement facility, she continued to work as a Thomas M. Rivers Research Fellow at the University of Delaware. Treatment of congenital disorders had advanced so much by then that most children born with congenital heart disorders survived. She saw them walking around her when she went out, individuals who would not have been alive but for her determination. With a life of successes at her back, Taussig could have enjoyed the fruits of her labors. Instead, she refocused. She studied adults who, as children, had been treated for congenital heart problems; she wanted to know their long-term fate. But she also became interested in the origin of the problems she had studied her whole life. She was convinced congenital heart problems could be understood in the context of evolution.

  15

  The Evolution of Broken Hearts

  Nothing in biology makes sense except in the light of evolution.

  —THEODOSIUS DOBZHANSKY

  In 1984, Helen Taussig moved to a retirement home. At night she slept in a building filled with other retirees, men and women living through the last phases of their lives. During the day she drove herself to the local Delaware Museum of Natural History. There, she pulled out birds that had been sent to her, plucked the feathers from their bodies, and cut into their tiny chests. One might be a warbler. Another a starling. Looking just beneath the skin, she found hearts, nearly all of which were ordinary bird hearts, four-chambered, perfect, and fascinating. She did ten a day or so before disposing of the feathers and writing down what she had found. Then she drove home, noticing en route the birds flying over her, birds on lines, birds even on her very own stoop. Mostly their hearts beat in rhythm, perfectly, moving blood to gut, beak, and wings. Sometimes, though, their hearts beat wrong. At least, that is what Helen Taussig desperately hoped.

  In retirement, Helen Taussig had embarked on an entirely new career. In the late 1970s, she began to imagine that if she studied the hearts of nonhuman animals—mammals and then birds—she might understand why so many babies were born with broken hearts. At the time, the prevailing idea was still that congenital heart defects were caused by mutations that arose when mothers exposed their unborn children to dangerous environments and mutagens (also called teratogens). Parents were blamed for the difficulties and even deaths of their children. Taussig disagreed. She wanted to understand the causes of the disorders of the heart in the hope that doing so might clear parents of the responsibility they so often felt. Her approach was unusual: she sought to study the heart’s deformities in light of evolution. For a physician, this was a novel endeavor. It required taking the detective’s approach she had used for decades when dealing with individual cases and applying it to a much bigger story, one that had unfolded over hundreds of millions of years rather than just the nine months of development. Taussig began to read what was known of the evolutionary biology of human hearts and those of other animals. What she found fascinated her and inspired big, bold ideas.

  Taussig approached the evolution of the heart with two guiding principles. The first was that an understanding of the evolution of the vertebrate heart would inform her (and others’) understanding of the problems of human hearts. To evolutionary biologists, this was an old idea, but to clinicians it was new, radical even. The second was that if one could understand which animals suffered from which congenital deformities, it might be possible to determine if the deformities were genetic and, if so, when they had arisen. Any congenital deformities present in both birds and mammals, for instance, must relate to genes older than either group. Conversely, deformities present in only mammals or only birds had to be more recent phenomena.

  In some ways, the difference between the culture of the surgeons and physicians with whom Taussig had spent her life and that of the evolutionary biologists whose field she was now entering could not have been greater, even in terms of something as simple as the number of practitioners. In the United States, a cardiology meeting such as the Transcatheter Cardiovascular Therapeutics Conference might attract as many as ten thousand cardiologists. In contrast, the main evolution conference in the United States has a big year when it attracts two thousand people, most of whom are students. Those two thousand faculty and students study not just human hearts or even humans but all of life. If we suppose there are about ten million species on Earth (no one really knows; I suspect a much larger number), this leaves about ten thousand species per evolutionary biologist. The cardiologists and other physicians study just one species, humans, and typically a single organ of that species. And whereas the physician wants to fix the problem, typically independent of big questions about why it arose, the evolutionary biologist is not concerned with problems. The evolutionary biologist, in other words, tends to focus on exactly the thing the physician is least focused on, and vice versa.

  But there is one thing that the physician and the evolutionary biologist share: the mind-set of a kind of detective. As Taussig started to look at the birds and the millions of years of evolution they implied, the sweep of time was new, but the love of the mystery reminded her of every mystery in every body of every child who had ever come into her office.

  In reading the papers of evolutionary biologists, Taussig was fascinated by the number of forms a functional heart could take, its beating diversity. Most vertebrates—those animals with backbones, like humans—do not have a four-chambered heart. The fish have two chambers, as do amphibians such as frogs, and turtles, snakes, and lizards have three; birds and mammals alone have four. But what was fascinating to Taussig about this state of affairs, other than the question of how all of these very different hearts work, was that the birds and mammals seemed to have come by their four-chambered hearts independently. Birds evolved from one group of four-legged reptiles; mammals from another. Their most recent common ancestor lived more than t
hree hundred million years ago. That both mammal and bird hearts have four chambers is a consequence of the predictable efficiency gained by having four chambers.

  Taussig knew that she was biting off more than she could chew with this project. She decided to focus on the simplest piece: comparing the heart deformities in birds and mammals. Were they the same? Initially, she imagined that she might be able to just look at what was already known of the bird deformities and compare that to what she had learned about humans and other mammals during her career. The challenge would prove greater than she had anticipated.

  Few scholars had studied the congenital deformities of hearts in nonhuman animals. When Taussig searched for examples, she found ancient anecdotes but few modern data. The Greek scholar Theophrastus, for instance, noted that all of the partridges of Paphlagonia seemed to have double hearts. This was fascinating, but she needed much more, which is what led her to begin to dissect birds herself.

  You might imagine that Taussig’s friends and colleagues would have greeted anything she did in her later life, after all the accolades and successes, with enthusiasm. She had earned enthusiasm. But little was forthcoming. Her colleagues and friends don’t seem to have appreciated the work she did in her final years; it would later occupy a brief pair of vague sentences in the key biographical paper about her. They gave her the begrudging respect of never telling her that they thought this evolution work she had begun to do—every day—seemed to be, at best, an eccentricity. They may have thought her a victim of her aging mind.