Heart--A History Page 7
In the dressing room, I pulled off my slacks and folded and snapped them under the bright lights. I hung up my clothes in a locker and put on green scrubs, like the kind I’d worn in anatomy lab in St. Louis six years prior. At the metal washbasin, Shah and I scrubbed our hands and arms to the elbow with brown iodinated soap. He spoke with a slightly oblique tone that seemed to intimate that there was more to what he was saying, even when he was saying something pretty obvious. “This man is sick,” he intoned gravely, kicking an aluminum panel to turn off the faucet. “If we don’t operate tonight, he will die.” I said nothing. It was my first heart surgery, and I wasn’t sure what to say or do. Should I ask questions and try to learn something? Or keep my mouth shut and stay out of the way?
Back in the OR, we donned sterile gowns and gloves and blue masks. Everything in the room was gray, beige, or blue, save for the wildly colored surgical caps. Shah put on tiny binoculars, like a jeweler’s spectacles. He was a handsome man, tall and lanky, with coiffed jet-black hair, like a Bollywood actor’s, only partially covered by his paisley beret. “Stand here, next to me, but don’t touch anything,” he said. He grabbed the sterile plastic cover on the ceiling lamp and adjusted the light just so. By now the patient was anesthetized and intubated, looking like just another cadaver. A mess of tubes and wires slithered menacingly across the table toward him. The operation was ready to begin.
With a scalpel, Shah cut through the skin over the breastbone. A necklace of dark red beads quickly appeared. With a buzz saw that looked like an ironing press, Shah made a linear cut along the entire length of the sternum. His assistant quickly poked at tiny bleeding vessels with a cautery, liberating tiny puffs of proteinaceous smoke. Using a stainless-steel retractor, they separated the two sides of the sternum, and the pink-and-yellow chest cavity came into view. Holding forceps and a scalpel, Shah cut open the silvery pericardium. The heart was dancing wildly, an incredible sight. I thought back to those dog days of summer in St. Louis, but what I beheld was so different from the dry brown heart of my nameless cadaver. This one was pink, like uncooked chicken; for a moment, it seemed to be the only thing in the room that was moving. Plastic catheters were quickly stitched into the right atrium and the aorta. They would be used to create a circuit for the heart-lung machine that was going to keep our patient alive.
The machine itself was a beige box, about the size of a small refrigerator, with a dizzying array of dials and tubes. It had already been primed with saline to purge it of air. Shah connected the machine via hoses to the catheters in the chest and told the perfusionist to turn it on. When he did, incredibly the heart began to shrink as blood, life’s fluid, was diverted into the plastic-and-metal apparatus. The heart nevertheless continued to beat, though weakly and more slowly. Much of my life I’d lived with the fear that the heart could stop at any moment and one’s life would be extinguished. And here it was, shrinking like a balloon with a small leak. It sent shivers through me. Never had the boundary between life and death seemed so thin.
With a metal clamp, Shah compressed the aorta, cutting off blood flow from the heart, thus isolating it. Then he injected ice-cold potassium solution into the main cardiac vein. Concentrated potassium is used to stop the heart in executions, and sure enough the patient’s EKG quickly flatlined. Shah poured iced saline directly on the heart to cool it further. Then, with the heart quarantined and the patient’s circulation and oxygenation being controlled by the heart-lung machine, he began to cut into the diseased organ.
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Many (mostly American) breakthroughs allowed that Christmas Eve surgery to take place, but perhaps none was bigger than the heart-lung machine. It has been described by the physician and author James Le Fanu as “among the boldest and most successful feats of man’s mind.” The machine was conceived by John Gibbon, a Philadelphia surgeon, in 1930, but it took almost twenty-five years to develop. One reason for the delay was the economic downturn during the Great Depression, and then there was World War II. But progress was also retarded by cultural fallacies. Though an artificial kidney was developed with relatively little fanfare, the heart occupied a special place in the popular imagination. How could a man-made machine replace the organ that houses the soul?
Without such a machine, cardiac surgeons were hamstrung. Once the heart is stopped to cut it open, a timer starts to tick. Deprived of oxygen-rich blood from the heart, the brain and vital organs will get irreversibly damaged within three to five minutes. However, most congenital heart malformations require at least ten minutes of circulatory stoppage to repair, too long to avoid brain injury. So, most surgeons believed such operations could never be performed, at least not until a machine was built to take over heart and lung function for those crucial minutes.
One surgeon who believed there was an alternative was C. Walton Lillehei, considered by many the most innovative surgeon of the twentieth century. Born and raised in Minneapolis, Lillehei grew up an inveterate tinkerer. As a teenager, when his parents refused to buy him a motorcycle, he built one out of spare parts. He brought that engineering mentality to his surgical research. His small research lab in the attic of Millard Hall at the University of Minnesota was nothing more than two operating tables, a sink, and a few oxygen tanks. But it was there, following his chief Owen Wangensteen’s edict to transform the department into a center of surgical invention, that Lillehei developed perhaps the most bizarre idea in the history of surgery: controlled cross-circulation.
Lillehei’s ideas were inspired by the circulation of blood between mother and fetus in mammals. Because the fetus is bathed in amniotic fluid, it cannot obtain oxygen by taking breaths. Its blood must be shunted into the mother, where it is oxygenated and cleaned of waste before returning to the fetus. Why, Lillehei reasoned, couldn’t the same scheme be employed in heart surgery? An animal (a “donor”) could be used to clean and oxygenate blood from another animal (the “recipient”), returning the blood to the recipient to nourish it while the recipient’s heart was stopped and isolated from the circulation. It seemed so simple, circumventing the need for a machine. In Lillehei’s early experiments, the circulatory systems of two anesthetized dogs were connected through a beer hose to a milk pump between them that pushed equal amounts of blood in opposite directions without introducing air bubbles. With the recipient dog’s chest splayed open, the inlet and outlet of its heart were clamped off, its lungs collapsed, and its blue venous blood propelled by the milk pump into the donor dog. Red, oxygenated blood was returned from the donor to the recipient through an artery in the recipient’s chest. In this way, the donor served as the recipient’s heart and lungs while the recipient’s heart was stopped and drained of blood.
At first, Lillehei and his team made mistakes in the complex hookup of the circuit, and the dogs, which they’d picked up from the pound, suffered brain damage. But after a few attempts, they were able to carry out their experiments successfully, with both recipient and donor dogs waking up unscathed. After the experiments, the dogs were euthanized and their organs examined under a microscope. There was no evidence that cross-circulation caused damage to either animal. The recipient was supplied with enough blood and oxygen to maintain basic function, and the donor’s ability to circulate its own blood wasn’t compromised either. A few months later, Lillehei tested his method on trained dogs, including the purebred golden retrievers of a cardiologist colleague. Even after thirty minutes of cross-circulation, the dogs were still able to follow commands and do their usual tricks.
In 1954, after years of experiments on some two hundred dogs, Lillehei and his team were eager to try their method on humans. They were interested in correcting congenital heart defects. At the time, fifty thousand or so American babies were born every year with such anomalies. (Even today, a baby is born with a heart defect in the United States every fifteen minutes.) Most defects are coin-sized holes in the wall between the atria or the ventricles, allowing mixing of oxygen-rich and oxygen-poor blood. These holes can result in stu
nted growth, oxygen deprivation, fainting, even sudden death. “Congenitals” were a fixture on hospital wards in the 1950s, often seen sitting on the edges of their beds, leaning forward to catch their breath, legs distended like tree trunks, seeping pale yellow fluid (the result of congestive heart failure) through their skin and into puddles on the tile floor. They frequently had facial deformities because heart defects often coexist with anomalies like Down syndrome. They suffered crippling infections, too; half died before the age of twenty. In short, they were cardiac cripples, their existence doomed, their prognosis worse than many childhood cancers. A leading surgeon asserted that it should be possible to fix some of the heart anomalies “as a plumber changes pipes around,” but such surgery was prohibitively long.
Though the need was great, Lillehei’s proposal to use one human as living circuitry for another was shocking, to some even immoral: the first operation in human history that had the potential to kill two people. The idea of anesthetizing a normal human being in the operating room to provide life support to another while the other’s heart was stopped, sliced open, and repaired was unacceptable to most doctors, a violation of their most fundamental oath. However, with no artificial heart-lung machine available, and despite the fervid opposition of his colleagues, Lillehei forged ahead.
Lillehei had one attribute that set him apart from most other doctors: he was a cancer survivor. In his final year of residency, he had been diagnosed with a usually fatal lymphosarcoma of the neck. No less than Wangensteen, his department chief, operated on him in a ten-and-a-half-hour surgery. Lillehei’s biopsy results had actually come in several months prior, but Wangensteen had waited until a few days before Lillehei graduated to tell him so the young surgeon would finish his residency. In the operation, Wangensteen and his team excised the tumor, lymph nodes, and much of the surrounding soft tissue of Lillehei’s chest and neck. Exploratory surgery a few months later showed no trace of cancer.
With such a close brush with mortality, Lillehei seemed to have a better acquaintance with death than most surgeons, rendering him less fearful of it. He had been given a 25 percent chance of surviving five years, so for much of his early career he was running along the edge of a precipice, waiting for the inevitable slip that would send him into the abyss. His tenuous hold on his own life conferred courage, perhaps even a kind of foolhardiness. He was going to spend whatever time remained for him tackling the conundrum of open-heart surgery. He was willing to try new things, experimental procedures with low chances of success, despite the up-front costs. For his part, Wangensteen gave Lillehei the time and resources to perform his innovative work. He was protective over him like a father over a vulnerable child. He was also convinced that Lillehei had the best chance of any of his protégés to be awarded a Nobel Prize.
There was another alternative to the heart-lung machine besides cross-circulation, at least for simple heart surgeries: cooling the body down to freezing temperatures to slow the metabolism and thus reduce the need for oxygen. A ten-degree drop in temperature halves the rate of most chemical reactions, including cellular processes, which is why people have been known to survive submerged in a frozen lake for up to forty minutes. The first use of surgical hypothermia was presented by Wilfred Bigelow, a Canadian surgeon, at a conference in Denver in 1950. Bigelow anesthetized laboratory dogs, cooled them in an ice bath, opened their chests, clamped off their hearts to stop blood flow, and then de-clamped, stitched, warmed, and woke them up with no permanent brain damage. He later found that monkeys were even better than dogs at tolerating hypothermia. At sixty-eight degrees Fahrenheit, they could have their circulation stopped for almost twenty minutes with no damage to their brain function.1
The first successful human demonstration of Bigelow’s “frozen lake” strategy took place on September 2, 1952—more than half a century after Ludwig Rehn’s first myocardial stitch—when Dr. John Lewis, a slightly senior colleague of Lillehei’s at the University of Minnesota, used hypothermia to fix an “atrial septal defect,” a hole in the wall between the left and right atria, in a five-year-old girl named Jacqueline Johnson. Though the girl’s heart was enlarged, she herself was frail and underweight. She had been sick for most of her life with recurrent pneumonia, and doctors had written her off as having only a few years to live. Faced with such a grim prognosis, her parents gave Lewis and his team the go-ahead to operate.
Using a rubber blanket that circulated a cold alcohol solution, Lewis cooled Jacqueline’s core body temperature over many hours, the thermometer reading steadily dropping from normal, around 98.6 degrees Fahrenheit, down to 79 degrees. He quickly pinched off her major veins and arteries with tourniquets so that no blood could get into or out of her heart, achieving an almost bloodless organ. At this point, no blood was circulating in her frozen body. Then, with a scalpel, he cut through the wall of her right atrium, being careful to avoid the coronary arteries and essential electrical structures. It took about three minutes for him to find the dime-sized hole. Within two minutes, he had sewn it shut. To test the repair’s integrity, he injected the heart with salt water to make sure there was no leak. When it looked as if the repair was intact, he released the clamps on the major blood vessels. The heart began to beat sluggishly. With his hands in the open chest, Lewis massaged it, willing it by hand to do its job, and within a few minutes the heart began to speed up. After a few minutes, Lewis warmed up the little girl in room-temperature water, filling a trough that had been purchased from Sears, Roebuck. Though there were a few small bumps in her postoperative course, Jacqueline did well. Eleven days after the operation, she went home. By the end of the month, she was just another girl at school.
The acclaim for Wangensteen and his department was widespread. “‘Deep Freeze’ Heart Girl Making Rapid Recovery,” announced a headline in The New York Times. The Minneapolis Tribune gushed that the operation “seems to give surgeons a method, long sought, of putting the knife to the live human heart in plain sight.” Though many who opposed animal experimentation were aghast, a newspaper editorialist, referring to the number of dogs that had been sacrificed in developing the technique, wrote that “one child at the price of fourteen dogs is a remarkable bargain.”
Still, hypothermia wasn’t the blanket answer for all congenital heart defects. It afforded surgeons only a fraction of the time they needed because it protected the non-perfused brain for only a relatively short period. While five minutes was sufficient time to repair a simple lesion like an atrial septal defect (ASD), more complex defects, such as ventricular septal defects (VSDs), the most common type of congenital heart abnormality, in which holes in the wall separating the two ventricles allow blood to flow abnormally, required more time, at least ten minutes. And so, these patients continued to be labeled “inoperable.”
Lillehei proposed using cross-circulation on these children. He appealed to Wangensteen, expecting his full support, but the chief refused to grant him permission. The technique was too new, Wangensteen said, too risky to use on a frail child, and he correctly predicted the furor that would erupt if a child not facing imminent death—or his donor parent—succumbed on the operating table. Lillehei pushed on, presenting Wangensteen with published reports showing poor outcomes of experimental VSD repairs using the hypothermia method, but Wangensteen was unmoved. He gave permission to Lewis, Lillehei’s rival, to perform the first VSD repair using hypothermia. Only after Lewis quickly failed in his first two attempts, resulting in two deaths, did Wangensteen relent and give Lillehei the opportunity he had been waiting for.
Lillehei’s first patient was a thirteen-month-old boy with a VSD. Gregory Glidden, as he was named, lived with his parents and eight siblings in the north woods of Minnesota about a hundred miles from Minneapolis. His father, Lyman, a mine worker, and his mother, Frances, were tragically familiar with congenital heart disease. Gregory’s older sister had also been born with a VSD and had died unexpectedly in her sleep three and a half years earlier. (Frances found her dead in bed one
morning.) Like his sister, Gregory had spent most of his young life in the hospital. His first words and first steps were spoken and taken on a lonely patient ward. In December 1953, Gregory’s pediatrician sent an urgent referral to the University of Minnesota. The little boy was having frequent fevers and could not easily draw breath. He weighed just eleven pounds, no more than his stuffed animals. His heart was enlarging, too, at an alarming rate; it was more than double the normal size, a sign of impending circulatory failure.
Cardiologists in Minneapolis admitted Gregory to the Variety Club Heart Hospital at the University of Minnesota. After performing tests to confirm the presence of the VSD, they arranged for a consultation with Lillehei. They had heard about the innovative research he was doing in the attic of Millard Hall. Perhaps this maverick would be the one to finally fix the dreaded VSD and prevent another baby’s death. After meeting Gregory, Lillehei proposed an operation in which he would fix the boy’s VSD using cross-circulation, with Lyman Glidden, who had his son’s blood type, serving as the donor. Lillehei made it clear to the Gliddens that he had used cross-circulation only on dogs, but he told them that if a child of his needed open-heart surgery, he would not hesitate to use the technique. Desperate, the Gliddens gave the go-ahead. The consent form they signed in March 1954 was a single sentence: “I, the undersigned, hereby grant permission for an operation or any procedure the University staff deems necessary upon my son.”
Today, patient autonomy and shared decision making are mantras in the hospital, ethical imperatives that supersede all others, including beneficence. But the situation was very different in the 1950s, when doctors were more apt to act without what we would consider informed consent. Medical paternalism was rampant, but it would be a mistake to think of Lillehei as authoritarian. By all accounts, he was an unusually compassionate physician, having been a patient himself. As a patient, he knew the vulnerability that comes with illness. He knew on a visceral level how patients look to their doctors for guidance and protection. But as a surgeon, he also understood that his young patients had no chance for a normal life and that there were no other procedures available to help them. Desperate parents did not want to hear that there were no options. They wanted a doctor to do—try—something.