Heart--A History

by Sandeep Jauhar

  * * *

  A couple of weeks later, I saw Jack in the clinic. He was wearing his bowler and a vintage blazer, looking even more stylish than usual. He said he was feeling better. There was more color in his face. He had gained some weight, too.

  He had given up his magnets, which he could no longer apply to his body because they interfered with his defibrillator. (This was probably the reason he had resisted the device for so long, I realized.) I inspected the implantation site. It was red but dry and intact. There were small bandages covering the incision.

  “The visiting nurse suggested more diuretic to treat the swelling in my legs,” Jack said, hopping onto the exam table. “What do you think?” I couldn’t help but smile. I had been recommending this for months. “I think that would be a good idea, Jack,” I said.

  He reminded me that before he left the hospital, he’d agreed to increase fosinopril, one of his heart medications. “But sometimes it makes me dizzy,” he said. “Would it be all right if I cut the dose in half?” I started to laugh. Jack, once my most noncompliant patient, had become a convert to modern cardiology. To think that all it took was to bring him back from the dead.

  But before I could say anything, Jack reminded me that doctors in the hospital had stopped his herbal supplements. “They did give me magnesium, but in the gluconate form, which you can’t absorb,” he said with irritation. As soon as he got home, he’d gone back to taking his usual nutraceutical cocktail. “I’m finally feeling better,” he said. “I won’t ever let that happen again.”

  The first total artificial heart (Gift of Dr. Denton Cooley, Division of Medicine and Science, National Museum of American History, Smithsonian Institution. Reproduced by permission)

  11

  Replacement Parts

  For a dying man [a heart transplant] is not a difficult decision … If a lion chases you to the bank of a river filled with crocodiles, you will leap into the water, convinced you have a chance to swim to the other side.

  —Christiaan Barnard, South African surgeon

  The mother had on red lipstick, thickly and haphazardly applied. Her eyes were swollen, her hair tied up in a bun. Tears had left tracks on her brown, pitted cheeks. When she saw me, the tears started up again.

  Ravindra, her twenty-five-year-old son, was dying, and we both knew it. I’d been dreading the conversation we should already have had, and so apparently had she. Whenever I’d tell her that we needed to discuss her son’s condition, she’d tell me to speak with her husband, Ravindra’s father. He was a simple man, a salesman, who would sit quietly, square-jawed, even as his wife wailed in grief. When he could bear it no longer, he would put his arm around her and gruffly say, “Come on, woman, come on.”

  Curled up on a stretcher in the emergency room, their son was gulping air. His breathing had quickened over the past several days; that is why they had brought him in. Crusted mouth, sunken eyes, wasted temples: he rested unnaturally on the bedsheet, his body almost folding in on itself, a consequence of Friedreich’s ataxia, a hereditary nerve disease that robs motor function of the arms and legs and, in its final stages, destroys the heart, too. On an echo, his heart wasn’t so much beating as twisting, trying to expel its contents. Though he was an adult, he looked no older than a teenager. A tiny wisp of a mustache was all that distinguished him from the adolescent patients down the hall. At Christmas, I’d put up the money for an Xbox for him, as I’d done for my own son, Mohan. It was the only thing Ravindra wanted, but the family could not afford to buy one. Sadly, he never got to play with it. By the time the holidays rolled around, he was too debilitated, confined to a motorized wheelchair when he wasn’t lying in bed. I remembered the embarrassed look on his face when his mother showed me pictures of him when he was younger. In one photograph, he was standing on a pier, a large body of water behind him, broad-shouldered in a red tank top. When I asked him if he liked the picture, he nodded without looking up. When a nurse asked if the pictures were really of him, he loudly grunted yes.

  Now he was back in the hospital. He’d been admitted the previous month, too. When patients with heart failure begin to have frequent hospitalizations, it means their condition has taken a turn for the worse. It is a sign that the end is near.

  I asked Ravindra to sit up so I could listen to his back. His father jumped out of his chair before I could correct myself. “He can’t sit, Doc,” he said apologetically.

  “Yes, of course,” I said, berating myself silently. I’d forgotten.

  We pulled him up. His lung sounds were crackly. When I pressed on his distended belly, the veins in his neck popped out like straws. Typical symptoms of end-stage heart failure include shortness of breath, fatigue, nausea, and mental lassitude. Ravindra had all of them.

  I put away my stethoscope and stepped back from the stretcher. His parents stared at me. “Don’t let him die,” his mother whispered, as if reading my thoughts. “We are not ready to say goodbye.”

  I asked Ravindra’s father to step outside. In the hallway, we faced each other squarely. He had a trim beard. He worked part-time as a Hindu priest. Traces of red powder were still on his forehead.

  “His heart is getting weaker,” I said, not sure how to begin.

  “It gets weaker and weaker until it finally stops?” he said. I nodded, not having the energy to disabuse him of this misconception. I could feel his desperation. I had a son, too.

  I remembered the story he’d told me of how Ravindra got sick. “He used to pull his hair, bite his clothes,” the father had recounted. “Schoolteacher said something not right with him.” They took him to a pediatrician, who did a blood test. “I don’t know where he sent it. Then we went to seven more places with seven more tubes of blood, and then they come up with this. They told us he will end up in a wheelchair. We didn’t believe them, but they were 100 percent right. Everything what they say, we see today. Only thing they got wrong was they said he would live for fifteen years. He lived for twenty-five.”

  Now, facing me outside his dying son’s room, he asked me the question I’d been dreading. “Can you give him a new heart?”

  * * *

  Many diseases have a common, final pathway. For heart disease, that final pathway is heart failure. In the most common form, the heart’s contractions weaken because of damage—heart attacks, chemical, viral—resulting in a drop in blood flow and blood pressure. Because blood pressure determines oxygen delivery to vital organs, the body does all it can to keep the pressure up. Hormones are released that signal the heart to beat faster and the kidneys to retain water to increase blood volume (and therefore blood pressure). These hormones are a temporary fix, however. Cardiac output and blood pressure often do return to normal levels, but at a great cost. The body becomes congested as fluid accumulates and leaks into tissues. As patients become weaker and malnourished, protein levels also drop, keeping even less fluid inside the veins. Soon, water is everywhere, filling up soft tissues in the legs, abdomen, and lungs. The French writer Honoré de Balzac had congestive heart failure. According to Victor Hugo, his close friend, Balzac’s legs resembled “salty lard.” They were so waterlogged that doctors tried draining them by puncturing the tight, congested skin with metal tubes, resulting in gangrene, from which he died.

  Though patients with heart failure are often literally drowning in their own fluids, their kidneys continue to limit water excretion, incorrectly perceiving a low blood volume because of inadequate blood flow. Treating congestive heart failure is a Sisyphean struggle. The more fluid that is removed with diuretic drugs, the more fluid-retaining hormones get activated. In the end, the therapy becomes its own enemy. Half of all patients with heart failure die within five years of receiving the diagnosis. For the most severe cases, like Ravindra’s, the average survival is only a few months.

  The definitive treatment for end-stage heart failure is a heart transplant. The field has progressed quickly over the past several decades. Today the survival rate after cardiac transplantation is a
bout 85 percent at one year, nearly four times better than the average survival of patients treated with medications alone.

  But as recently as the early 1960s, heart transplantation seemed like a pipe dream. Organ rejection and life-threatening infections posed prohibitive risks. By the second half of the decade, however, animal research had pointed a path toward human transplantation.

  In the end, the race to transplant the first human heart was primarily between Dr. Christiaan Barnard at Groote Schuur Hospital in Cape Town, South Africa, and Dr. Norman Shumway at Stanford. The two surgeons had been residents under Walt Lillehei at the University of Minnesota. By many accounts, they’d had a frosty relationship. Shumway scorned Barnard’s showmanship, his aggressiveness, his willingness to cut corners. Barnard, for his part, resented the way his Minnesota colleague viewed him as a foreigner born into poverty in a pariah country. However, they did share the inspiration of their great surgical mentors, who guided them throughout their careers. It was Owen Wangensteen, the surgical chief at Minnesota, who arranged for Barnard to get his first heart-lung machine at Cape Town in 1958. Before Barnard used it—in the first open-heart surgery in apartheid-era South Africa—he received a letter of encouragement from Lillehei. “Nice and simple,” Lillehei advised his protégé, describing the kind of surgery Barnard should attempt first. “Nothing too fussy, nothing too flashy. I have every confidence in you.”

  Barnard was up against a great challenge. In the 1960s, the United States—Stanford in particular—was the mecca of transplant cardiology. Plus, Shumway had a great deal more experience with animal transplants, which he had helped pioneer. In 1959, he and Richard Lower, a Stanford resident, performed the first dog heart transplant. The recipient lived for eight days, demonstrating that an organ could be transplanted from one animal to another and continue to function. By 1967, about two-thirds of Dr. Shumway’s research dogs were able to live for a year or more. In late 1967, he announced in an interview in The Journal of the American Medical Association that he was going to start a clinical trial at Stanford to perform the first heart transplant in a human. “Although animal work should and will continue,” he said, “we are none the less at the threshold of clinical application.” At that point, he had transplanted hearts into nearly three hundred dogs. Barnard had done about fifty.

  But Shumway was at a disadvantage when it came to finding a human donor. American regulations at the time prohibited organ harvesting from brain-dead patients if their hearts were still beating. The heart had to stop completely before organs—including the heart—could be collected.1 Barnard, on the other hand, was governed by more liberal South African laws—legislation that he himself had presciently advocated for—that allowed a neurosurgeon to confirm death if a patient showed no response to light or pain, a much lower bar than the one for his American counterpart.2 By South African standards, once family consent was obtained, a transplant team could quickly harvest organs, including the heart, while they were still being perfused with blood.

  It was a close race, but Barnard broke the transplant tape first, on December 3, 1967, thirty-four days before Shumway. His first patient, Louis Washkansky, a fifty-five-year-old grocer, received the heart of a young woman who had suffered brain damage after being hit by a car while crossing the road. He lived for eighteen days after the procedure, succumbing to a lung infection after his immune system was weakened by drugs to prevent organ rejection. Shumway had to content himself with doing the first adult heart transplant in the United States a month later, on January 6, 1968. His patient, a fifty-four-year-old steelworker, lived for two weeks before surrendering to what Shumway described as “a fantastic galaxy of complications,” including gastrointestinal bleeding and sepsis.

  Today, with the development of antirejection drugs, the long-term outcomes following heart transplantation are excellent. The median survival is probably greater than twelve years (fourteen, if the patient survives the first year). The success has been a mixed bag, however. Though many lives have been saved, many more have been lost while patients have waited for a viable organ. Only about 3,000 Americans receive a heart transplant each year, though roughly 4,000 are on the transplant list and perhaps ten times that number would benefit from a transplant if an organ were available. Despite public campaigns to increase organ-donor awareness, the number of available organs has remained relatively constant over the years (in part because of seat belt and motorcycle helmet laws that have resulted in fewer road fatalities). For this reason, heart transplants will never be the answer for the 250,000 or so patients in the United States with advanced heart failure. As the Vanderbilt University cardiologist Lynne Warner Stevenson put it, “Relying on transplants to cure heart failure is a bit like relying on the lottery to cure poverty.”

  Therefore, replacing the human heart with an off-the-shelf mechanical device has been the great ambition of cardiologists and cardiac surgeons for the past half century. At first glance, the obstacles seem insurmountable. Blood quickly coagulates when it encounters plastic or metal. Without adequate blood thinning, clots can be expelled from an artificial heart and course through the body, blocking arteries and causing strokes and other damage. An artificial heart also can never stop pumping, even temporarily, so without an internal battery to drive the device, power lines must travel into the body, posing the risk of infection. Moreover, even as recently as the late 1960s, a mechanical device had never been housed inside a human body in direct contact with the bloodstream. It was impossible to predict the consequences. And so, even a generation ago, building an artificial heart appeared preposterously wishful. But that didn’t stop some from trying.

  Willem Kolff, a Dutch physician, would be the first to succeed. The inventor of the artificial kidney, he moved on to a more vital organ when he performed the first artificial-heart replacement in an animal at the Cleveland Clinic in 1957. Kolff’s organ held two balloon-like sacs filled with blood inside its plastic ventricles. Pressurized air filled the ventricles and compressed the balloons, thus forcing blood out in much the same way as from a beating heart. Kolff’s subject, a dog, survived for approximately ninety minutes. A few years later, at a congressional hearing in 1963, Michael DeBakey, a distinguished surgeon at Baylor College of Medicine in Houston, called for federal investment to support research like Kolff’s. “It is possible to completely replace the heart with an artificial [device], and animals have been known to survive as long as thirty-six hours,” he told the legislators. This idea could reach “full fruition,” he predicted, if there was funding to support more research, especially in bioengineering. DeBakey’s appeal fell on receptive ears. American cardiovascular research had produced a steady stream of life-prolonging innovations over the previous decade, including the heart-lung machine, implantable pacemakers, and external and implantable defibrillators. Despite this progress, heart disease remained the number one killer in the country. Critics, such as Congressman John Fogarty, chairman of the House Appropriations Health Subcommittee—and a cardiac patient himself; he died of a heart attack in 1967—noted that millions were being spent to put a man on the moon. Why couldn’t more money be invested to help Americans dying at home?

  And so, in 1964, the National Institutes of Health started the Artificial Heart Program “with a sense of urgency,” as an ad hoc committee advised, with the goal of putting a man-made heart into a human by the end of the decade.

  On April 4, 1969, just before the decade ran out, the surgeon Denton Cooley, DeBakey’s great rival at St. Luke’s Episcopal Hospital in Houston, implanted the first artificial heart, made of polyester and plastic and powered by compressed air, into Haskell Karp, a forty-seven-year-old Illinois man suffering from end-stage heart failure. After the implant, which was supposed to provide only a few days of support, a frantic search for a donor heart commenced. A compatible organ was identified three days later in Boston. The donor was put on a chartered Learjet outfitted with a full medical team from Houston, but on the flight home the plane’s hydraul
ic system failed, and the pilot was forced to make an emergency landing. Another jet was dispatched, but by the time the donor arrived in Houston, they had a problem; his heart was damaged. On the ambulance ride to the hospital, the donor heart fibrillated, requiring electrical shocks and chest compressions to keep it pumping. It was transplanted successfully, but Karp died thirty-two hours after the operation.

  Though $40 million of federal money had been spent over almost a decade, many considered Cooley’s attempt premature. More research was needed to design surfaces that would not create blood clots, as well as to develop an internal generator so patients would not have to be hooked up to an external power source. Through the 1970s, many refinements were made to artificial-heart design, including changing the shape of the organ and developing more blood-compatible materials. In 1981, Cooley tried again. This time, the artificial heart provided thirty-nine hours of support, but again the patient died shortly after heart transplantation.

  Cooley’s artificial hearts were intended as interim therapy, a temporary bridge to heart transplantation. Neither was designed to be a long-term replacement. However, many patients with end-stage heart failure do not qualify for transplantation because of advanced age or coexisting medical conditions. Such patients require permanent support or “destination therapy,” a bridge not to transplant but to eventual death.

  The concept of permanent mechanical support was put to the test a year after Cooley’s second implant when a retired dentist named Barney Clark was wheeled into an operating room at the University of Utah Medical Center. Clark, who was sixty-one, had end-stage heart failure caused by a viral infection. He had originally been scheduled for surgery on the morning of December 2, 1982—coincidentally, almost exactly fifteen years after Christiaan Barnard’s first heart transplant—but when his condition acutely worsened on the night of December 1, in the middle of a heavy snowstorm, his doctors decided to press ahead with the world’s first permanent artificial heart. By the time the seven-hour operation was over, it had unleashed a blizzard of a different kind.