Heart

by Sandeep Jauhar

  In August 1969, Mirowski and Mower put a metal catheter inside a dog’s superior vena cava and a metal plate—a broken defibrillator paddle—under the skin of its chest. With weak current, they induced ventricular fibrillation by stimulating the heart during the vulnerable period. Then, with a single, much stronger twenty-joule shock, they terminated the fibrillation and brought the dog back to life. To publicize their achievement, they made a movie showing a dog first collapsing unconscious in cardiac arrest, then getting shocked by an implantable defibrillator, and finally standing up and wagging its tail. When observers suggested the dog had been trained to fall down and stand up, Mirowski filmed additional sequences with simultaneous EKG tracings to prove that his dogs’ hearts were indeed fibrillating. The film convinced many doctors that Mirowski was onto something with potentially great clinical benefits. In the spring of 1970, Earl Bakken of Medtronic visited Mirowski to inspect his apparatus. Mirowski performed a successful demonstration for his guest. Afterward, when Bakken asked what would have happened if the revived dog had not been defibrillated, Mirowski disconnected the defibrillator, put the dog back into ventricular fibrillation, and stood by as it quickly died.

  A dog collapsing in ventricular fibrillation and then standing up after successful defibrillation (Courtesy of Pacing and Clinical Electrophysiology)

  In a monumental blunder, Bakken decided that Mirowski’s device was not commercially viable. Since sudden death is essentially random, he wondered how Mirowski was going to identify patients most at risk. (Mirowski decided to focus on patients who had already survived cardiac arrest. Whether patients with heart disease but no history of cardiac arrest can benefit from an implantable defibrillator is a question that Mirowski was unable to answer and one that cardiologists are still grappling with.) Bakken also wondered how Mirowski was going to test his device. Would he have to put people into cardiac arrest to see if his apparatus worked? (The answer was yes.) Was that even ethical?

  So Mirowski and his team continued on their own, undeterred and largely unfunded. On February 4, 1980, they finally did their first human test. The fifty-four-year-old California woman had had multiple episodes of cardiac arrest. In the operation, surgeons at the Johns Hopkins Hospital implanted an electrode in her superior vena cava and sutured a patch electrode to the surface of her left ventricle. They inserted the generator into her abdomen. (As was the case with some medical school cadavers, early pacemaker and defibrillator generators were installed in the abdominal cavity.) Then, to test the device, they put her into ventricular fibrillation. The device did not activate at first. For fifteen seconds, Mirowski and his colleagues watched spellbound as the woman went unconscious. They were getting ready to apply an external defibrillator to her chest when the implantable defibrillator finally fired. One shock was all it took to revive her. Though The New England Journal of Medicine had rejected Mirowski’s first paper on his animal experiments, it quickly published his experience with his first three subjects in a paper called “Termination of Malignant Ventricular Arrhythmias with an Implanted Automatic Defibrillator in Human Beings.” Five years later, in 1985, the Food and Drug Administration approved commercial production of the device.

  •

  Seventeen years after FDA approval, Jack, my patient, was ready to become a reluctant beneficiary of Mirowski’s invention. Lightly sedated with midazolam and Ativan, he lay on a table in the cath lab, his head propped up on a foam wedge to help his breathing. He was relaxed and attentive. When I inserted a needle into his groin in preparation for the catheter, he seemed bemused, even tickled. “Oh my God, look, it’s my blood!” he said.

  I had a harder-than-usual time slipping the catheter into the right coronary artery. It turned out to be anomalous, originating from an unusual place. So Dr. Fuchs took over with a differently shaped catheter. “That’s the way it is with me,” Jack said, when I explained to him what was going on. “I’m an anomaly.” Fortunately, the right coronary was clean. The left coronary, too, was mostly normal. There was small plaque in the midportion, but it was unlikely to cause any trouble, so we decided to leave it alone. When I told Jack that we were done with the angiogram, he told us to keep on working. “You can go on for another hour if you want.” The scrub nurse laughed. Jack rather enjoyed being the center of attention. He seemed to appreciate an opportunity to be charming, even if it was on a surgical table.

  We transferred Jack to a stretcher and rolled him over to the neighboring electrophysiology suite, where the beeper-sized defibrillator was going to be implanted. Under the intense ceiling lights, his hospital gown was removed. I started off by cleaning his chest with three different antiseptic soaps. Then I pressed a clear antibiotic-impregnated film onto his skin. Defibrillator infections are rare, less than one in a thousand, but when they occur, the device must be surgically removed, so we had to be extremely careful to keep the operating field germ-free. Before long, Jack was getting a milky-white anesthetic, enough so he wouldn’t experience pain during the procedure but not so much that he could not breathe on his own.

  Shapiro, the colorful electrophysiology attending, entered with flair. “Honey, I’m home,” he boomed to the nurses. Together, we gowned, masked, and gloved. Then I tipped the table downward to put Jack’s head below his legs so blood would fill his chest veins to make them more visible. Shapiro injected Novocain into the skin and soft tissue. “That hurt,” Jack mumbled, and Shapiro told him to stop talking. “It’s dangerous for you,” he said, winking at me before increasing the rate of the anesthetic drip.

  With an electrical knife, Shapiro made a two-inch incision on the left upper chest, close to the shoulder. He dissected through the layer of yellow fat with the blunt end of a pair of scissors, down to the glistening white fascial plane and then below the pectoral muscle, where he burrowed a pocket for the defibrillator. Because Jack was so thin, we wanted to put the device below the muscle so it wouldn’t create too much of a bulge. I stood to one side, mostly watching. Occasionally, I was asked to cauterize a tiny bleeder, and so I’d pull out the electrical knife, releasing a thin wisp of blood smoke. Every few minutes, Shapiro would step back from the table and dance wildly to the song (“Roxanne,” “Rock Lobster”) on the radio.

  Before long, Shapiro inserted a twenty-two-gauge needle into a chest vein, pulling back on the hub of the syringe until it suddenly gave way, filling the clear plastic column with maroon blood, a sign of low oxygen tension. He threaded a slippery guide wire, like a guitar string, through the bore of the needle and into the vein. When he knew it was safely in, he pulled out the needle. “Never let go of the wire,” he said, and I nodded nervously. Shapiro threaded a plastic catheter over the wire and pulled the wire out of the vessel, leaving the catheter in place. Then he inserted a thin electrode through the hollow catheter and inched it forward into the heart. On the X-ray screen, it curved into the organ like a snake ready to strike. It buckled ever so slightly when it made contact with the inner surface of the right ventricle. Out came the catheter, leaving the electrode in place. Shapiro then placed a second wire through a large vein and onto the surface of the left ventricle. He slipped the generator, the size of a credit card but about a centimeter thick, into the pectoral pocket and connected it to the wires.

  We were done. All that effort over the past several months, and Jack, my magnetic patient, had finally gotten his defibrillator. It was time to test the device by fibrillating Jack’s heart. The Medtronic rep, a courtly, graying man who was there to help with the testing, called for me from across the room. “Step right up,” he said, standing in front of a small computer. “Now you’re going to kill your patient.”

  I was supposed to deliver stimuli to the heart during the vulnerable period to induce ventricular fibrillation. I pressed a few buttons on the keyboard to pace the heart three times and then deliver an extra impulse at a variable delay, trying to time the extra impulse into the vulnerable period to cause cardiac arrest. The stream of electrical pulses made cartoonish soun
ds, like Pac-Man gobbling dots. I started with an extra stimulus at 330 milliseconds. A few squiggles appeared on the screen, denoting a burst of disordered electrical activity, but the rhythm returned to normal. I repeated the test at 320, 310, and 300 milliseconds, with a similar result. But the next beat, at 290 milliseconds, did what we wanted. On the monitor, Jack’s picket-fence heartbeat transformed into a sine wave oscillating at several different frequencies. It was ventricular fibrillation, the rhythm of death. “Here we go,” the rep said excitedly. He started counting: “Five … ten … fifteen.” The defibrillator was programmed to shock after eighteen cycles of the sine wave. Though Jack had been awake this whole time, when I looked over he was now unconscious. I heard a dull thump, as if someone had driven a fist into Jack’s bony chest, and his body jumped ever so slightly off the table. The defibrillator had fired. On the screen, there was a spike and a pause, and then the EKG returned to normal. A nurse lightly slapped Jack’s face. “Wake up,” she said. “It’s all over.”

  Afterward, I asked Shapiro what we would have done if the implantable defibrillator hadn’t worked and external defibrillation was also ineffective. “It’s happened before,” he said. “You get these floppy hearts, and you induce fibrillation, and you can’t always shock them out of it.” He paused and started wiping down his hands. “It doesn’t make us happy,” he said, as if recalling a bad memory. He glanced at me once more. “It doesn’t make us happy.”

  •

  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 parttime 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 ret
ain 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.