The Open Heart Club

by Gabriel Brownstein

  Any damage to the muscle of the heart, Dr. Mehta explained, risks damage to the heart’s conduction system. In my 1999 surgery, something had been severed or disturbed in the circuit of the heartbeat, and so my heart would not beat on its own. This is known as heart block. After approximately forty-eight hours of electrical stimulation in the cardiac ICU in Boston, my heart had recovered from its block, and the sinus node reasserted control. But damage had been done; my heart was continually reconstituting its conduction system, and there were some glitches where the heart had been cut. Sometime after I came back to Brooklyn, a new electrical conduction circuit had asserted itself—the stressed-out pattern of atrial flutter. Electricity ran in a loop around a scar, and the top chambers of my heart added some frivolous beats to their usual march-time dance. Back in Boston, through cardioversion, the electrical shock had eliminated that problem, but then, in the months after, the heart—adapting and readapting to the damage and scarring and recovery of the myocardium—had developed a new circuit yet again, a circular charge running around the bottom of my heart, a new electrical pattern that resulted in my ventricular tachycardia, or V-tach, my new arrhythmia.

  The scar on the skin of my chest, so narrow and stylus fine after surgery, had thickened too. It was now a quarter of an inch wide, and I imagined, as the doctor talked, that similar changes had happened inside me.

  Dr. Mehta explained everything smoothly and suavely, and I listened like an eager student. The V-tach in itself would not necessarily kill me, he said. I had tolerated it well. But V-tach can easily flip to ventricular fibrillation, or V-fib, the rhythm of death, where the big bottom chambers of the heart flap fast as a humming bird’s wings, as fast as three hundred beats per minute (bpm), so fast that the chambers become useless and no longer move the blood at all. That was invariably fatal. My machine, said Dr. Mehta, would monitor every beat of my heart. If my heart rate became accelerated, the device would give me treatment.

  “You mean I’ll get a shock?” I said.

  “Not necessarily.”

  The ICD had three modes, Dr. Mehta continued, and the modes were determined by the speed of my heart in beats per minute: there was a first threshold at which the device simply recorded the heartbeat; a second threshold at which it delivered antitachycardia pacing (ATP), little gentle pulses akin to a pacemaker’s; and a top threshold at which the machine would fire, delivering forty joules of energy into the muscle of the heart. If the device sensed an accelerated heartbeat (first threshold), it would begin recording. If that heartbeat exceeded a preprogrammed rate (second threshold), the device would give me ATP. The ATP pulses would be mild enough that I would not feel them—the hope was that the pacing beats would trick the heart out of arrhythmia. But if the ATP didn’t work (third threshold), I would get the shock. The shock would be quite painful.

  “What does it feel like?” I asked.

  “I’ve never experienced it,” he reminded me, “but they say it’s like getting kicked in the chest by a horse.”

  I should be careful when I exerted myself, he warned. “The machine,” said Dr. Mehta, “is an idiot.”

  The ICD did not always recognize the difference between sinus tachycardia and ventricular tachycardia. It could distinguish between fast and slow beats but not between healthy and sick ones. My device gauged its thresholds based on the speed of the heart. It would react the same way if I was racing up a hill or if I was in V-tach. Because my V-tach ran at 150 bpm, said Dr. Mehta, the threshold for firing was relatively low. I would have to be careful when working out. I would get a shock anytime the machine sensed a sustained heart rate over 150 bpm. This was the disadvantage of the device.

  The advantage was that my arrhythmia would not kill me. So long as I had the device in my chest, I was safe.

  “A hundred percent safe?”

  Dr. Mehta smiled. Nothing was 100 percent, but genuinely safe. I was not going to die of arrhythmia when I had the ICD inside me.

  Marcia and I left the hospital, and we went home. The phone rang in the morning. It was Astrid. I thanked her for trying so hard to save my life. I apologized for not listening to her. She was relieved. She was happy, she said, so happy to hear my voice again.

  We went to the Brooklyn Botanical Gardens, Marcia, Eliza, and I. The last roses were in bloom, and the trees were changing color. As I lay back on the lawn, with my baby daughter crawling around and my wife beside me and the fat white clouds moving across the sky, I felt the terrible luck of my life, the stupid happenstance by which I was there at all, despite all my idiocy, my seeming attempts to destroy myself. The green of the grass, the blue of the sky, the wet smell of earth—I felt it all so keenly, the miracle of everything.

  I still can’t believe it: that I was born where Jim Malm was practicing, right after he mastered the art of tetralogy repair; that my heart held on just long enough, while the blood leaked backward and my ventricle expanded, for imaging technology and medical consensus to form around surgical intervention in 1999; and that I survived that crazy train ride somehow and was met in the hospital by newfangled technology that would guarantee my life. Had these arrhythmias come in the 1980s, when I was wearing my Holter monitor to high school and being tested for them, the doctors would have had no way to contain the threat to my life. But now they did.

  Baby Eliza put her face over mine, huge and smiling. Then she climbed up on top of me, her little foot and chubby weight right on the incision, and her poor father yowled in laughter and pain.

  38.

  THE FIRST IMPLANTED defibrillator was placed in a human chest in 1980 at Johns Hopkins. The machine was a half-pound brick, five inches long. Dr. Levi Watkins Jr. performed the operation. Watkins was the first African American surgeon on staff at Hopkins and a protégé of Vivien Thomas, who was still working at the hospital, now as a professor. Thomas himself helped Watkins design the surgery and prepare for it, running experiments in his dog lab. The patient was a desperate case: a fifty-seven-year-old woman who had had a heart attack and bypass surgery and whose frequent spells of arrhythmia were causing her to faint and putting her life at risk. She needed more than a pacemaker. She needed something that would reset her heart when the sinus node lost control of its beating. The doctors cracked her chest, laid the big device into her abdomen, and attached it via patches to the exterior of her heart.

  In the operating room, watching the procedure, was Michel Mirowski. A short, thickset, balding, and bespectacled research scientist in his mid-fifties, Mirowski wasn’t used to wearing scrubs. He was a researcher, not a surgeon. He had conceived of the implanted defibrillator in 1966 and had spent his career wandering—from Israel to Mexico to Baltimore, then back to Israel, then back to Baltimore again—obsessively working on the problem. Mirowski had received virtually no grant money in his quest to build the machine and minimal corporate support. He and his partner, Dr. Morton Mower, had been obliged to finance their work on their own, even purchasing their own lab dogs at $1 apiece.

  In the early 1970s, the implanted defibrillator was seen as an unwieldy, unnecessary, even sadistic device, enormous, uncomfortable, and as likely to go off unnecessarily as to save a patient. An early critic called it “an imperfect solution in search of a plausible and practical application.” Others described it as a bomb sewn into the human body. Some compared its use to Nazi experiments on prisoners, a particularly cruel comparison, given Mirowski’s history.

  He was born in Warsaw in 1924. As a boy, his name had been Mordechai Frydman. He’d changed it when he was fleeing the Nazis. His early childhood had been happy—he was the bookish son of a man who ran a profitable delicatessen. “I had a liberal view of the world which was in conflict with the Jewish reality in Poland,” he remembered. “I thought I was, first of all, Polish—obviously a mistaken impression.” He was an assimilated, middle-class child. But with the rise of fascism in Poland, his placid early life vanished. “Even the police were sympathetic to the right-wing trends,” he remembered. “They stood
aside when fascistic toughs beat and even killed Jews, and this was before the Nazis arrived.”

  In September 1938, German planes dropped bombs on Warsaw. Mirowski walked streets where the sides of buildings were torn open and the rooms exposed like dollhouses. In November, his mother died of heart failure—probably as a result of scars from childhood rheumatic fever. On December 1, all the Jews in Warsaw were ordered to wear yellow stars. “The schools were closed, and the persecution began. I remember German officers cutting off the payess,” the long sidelocks worn by Hassidic Jews, “to humiliate them. I told my father that I wouldn’t wear that yellow star and that it seemed foolish to stay in Warsaw.”

  Mirowski’s father made him take a new name so he could pass among the German invaders. And he gave his son two final pieces of advice before they parted. “Be a physician,” he said, and “be a Jew.” On December 5, the boy left the city. With a friend, he headed east, walking through woods and fields, hitching rides in the backs of trucks, sleeping in railroad stations, suffering asthma attacks. The boys traveled two hundred miles, down the Vistula river, past the suburbs and the resorts where bourgeois Jews had once spent their summers, through the German territories, to Lvov, which was held by the Soviets.

  He tried to enlist in the Russian army but was too young, so he ran further eastward, across Ukraine to Kiev, sleeping in parks. The Nazis entered Kiev in September 1941, and Mirowski and his friends fled ahead of their advance, riding on the tops of trains, eating whatever they could scavenge. They headed north, into Russia. In Rostov, the boy found work on a building crew, before he had to run again, 1,000 miles southward, to Krasnodar, where he hustled, rolling loose tobacco into papers and selling cigarettes. From Krasnodar, Mirowski went further east, ahead of the advancing Germans, all the way to Baku, the great oil-rich city on the Caspian Sea. In the Uzbek city of Andijan, he worked up to eleven hours a day at an airplane factory.

  He was now 2,500 miles from home, fighting off tropical fever, going to the library every day to study. He found work with a traveling group of singers, storytellers, and musicians, hanging signs and posters, a job that gave him coupons for bread. In 1944, finally, he enlisted in the Polish army and so survived to the end of the war and returned to Poland with the liberators. Everything he had known was gone, his family dead, his neighborhood rubble.

  “I saw the camp near Gdansk where the Germans had converted human corpses into soap, and there was a lot of it still available,” he remembered. “As far as Poland was concerned, it had become a cemetery for me.”

  Mirowski arranged with some fellow refugees to flee illegally to Palestine and arrived in 1947 only to find there were no medical schools there. He ended up studying medicine in France. “I knew I wouldn’t be staying,” Mirowski remembered. “Only in Israel or in the US could someone like me be a first-class citizen.”

  In 1954, he traveled back to Palestine. He found a job in Tel Aviv, in Tel HaShomer Hospital, working under Dr. Harry Heller, whose erudition and professionalism engendered a kind of worship in Mirowski. In Heller, Mirowski encountered the kind of doctor and mentor that he might have studied with had the war never happened, had he remained Mordechai Frydman, the clever deli man’s son, and had he gone off to Berlin for school.

  “A typical German professor,” Mirowski said of Heller. “Every day that we made rounds together was a holiday for me.” But Heller was suffering from bouts of tachycardia. “My wife asked me why I was so concerned,” Mirowski remembered. “‘Because he will die from it,’ I told her. And he did, two weeks later, at dinner with his family.”

  Mirowski had seen so much death—but here at last was one cause he could combat. He devoted the rest of his life to building a device that could save a person from sudden cardiac death due to arrhythmia. In the early 1970s, external electric defibrillators existed—Paul Zoll had built the first, and then another Boston doctor, Bernard Lown, had adapted the machine to alternating current—but defibrillators were large and unwieldy, weighing thirty to forty pounds, and the idea of putting one in a person’s chest seemed absurd. In Baltimore, Mirowski worked with Helen Taussig and met his partner, Morton Mower. Despite the lack of grant money and the widespread doubt that their idea was plausible, Mirowski and Mower persisted, and by 1980 they had built their big, bulky, but initially successful device.

  The principle of Mirowski and Mower’s machine is the same as the principle of the device that was put in my chest: when the heart beats too fast, it gets recalibrated with a shock. But my ICD is something quite different from the big, heavy brick Mirowski and Mower invented. My ICD weighs just a few ounces, and its sensors and memory are computerized; it varies treatment according to symptoms and can be adjusted to respond to the conditions of different hearts. ICD technology continues to improve: now patients don’t get wires put in their hearts. The latest models are smaller and nestle on the side of the patient’s chest, monitoring and treating arrhythmias. I’ll get one of those someday or maybe something even more futuristic.

  But for now, I am satisfied. Like so much else in cardiology, my ICD seems to have emerged through a haze of chaos and impossible history, just in time to rescue me.

  39.

  LET’S PAUSE IN this discussion of progress to remember the patients who did not survive. Not everyone has been as lucky as I have been.

  I met Doron Weber on a spring day in New York at a diner on the Upper East Side of Manhattan. A bald, gregarious man—a New York intellectual type quite familiar to me—Doron is the author of one of the few great books about congenital heart disease, Immortal Bird. It’s a memoir of the death of his son Damon.

  A decade had passed since the book’s publication, but I felt I could still see the grief over his lost boy on the man’s body. On a sunny spring day, he looked worn, like he’d walked to the diner through a storm. We chatted over breakfast. I got a bagel and a fruit cup. He ordered eggs, bacon, home fries, and toast. We did a little writerly shoptalk: books we liked, agents we’d worked with, editors we knew. When I say my heart went out to Doron, I hate the cliché, but that was the feeling, an ache in my chest moving toward him.

  The title of Weber’s book comes from Keats’s “Ode to a Nightingale”: “Thou wast not born for death, immortal Bird!” And that’s how I feel about my daughters, Lucy and Eliza. Everyone is born to die, but not them. For Doron in his memoir, his son Damon is a powerful spirit fastened to a dying animal. Damon’s defective heart is not, for Doron, Damon. And Doron’s ambivalence about doctors and hospitals is evident right from the start, when Damon is diagnosed and taken to the neonatal ICU:

  Three-day-old Damon lays inside a clean bubble, separated from us by modern technology, a pint-sized astronaut marooned in his own world. We slip our hands through the little portholes to touch him but this only brings home how distant and sealed-off he is… a captive of the medical profession.

  The Webers need the physicians to save their child, but ultimately they want to free their son from the prison of the hospital.

  Though he is born with just one ventricle, though he undergoes several surgeries, including a Fontan operation, which reroutes the blood around the failed parts of his heart, for thirteen years Damon is successful. He hikes up mountains. If he’s ever winded or having trouble, he hides it. The Webers’ life is eerily similar to my family’s. They live in the same neighborhood we do. Damon goes to Brooklyn Technical High School, where my daughter goes to school. He’s a patient at New York–Presbyterian, just like me. Mike Freed makes an appearance, consulting from Boston. Damon does well at Brooklyn Tech. He makes friends. He stars in plays. But life is not easy. A decade after his childhood surgeries, and though his heart seems to be pumping adequately, his blood begins leaking protein.

  “Yeah, just give me a minute.” Damon retrieves from the closet a big box with all his “fixings.” First, he disinfects the injection site with alcohol swabs. Then he applies a yellowish smear of anesthetic cream to his abdomen and covers the cream with a thin patc
h of cellophane, which he fastens with a makeshift belt.… We watch together until the cream has done its work. Then Damon unwraps a fresh syringe and needle and inserts the needle into a small ampoule of heparin. He retracts the plunger and fills the syringe with the clear, straw-colored liquid to just the right level, flicking it to ensure there are no bubbles or floating particles.

  Then, with a determined sigh, Damon jabs himself.

  His condition is a mystery. Protein-losing enteropathy (PLE), it’s called, and no one knows how or why it occurs in post-Fontan kids. About half the children who get PLE die, but again, the numbers are small, and the diagnosis is imprecise, so the statistics are difficult to parse. In his first year of high school, even though his liver is so swollen he looks pregnant, Damon wins a part in The Importance of Being Earnest. The moment he steps on stage with his slicked-back pompadour, twinkling eyes, and pompous thumbs hooked into his Victorian lapels, Damon commands attention.” But he also looks small and frail at curtain:

  My son bends at the waist with panache, draping his belly with one deft arm, while his red locks tumble forward. Temporarily buoyed by the energy on that stage, he’s the picture of actorly decorum, yet he’s barely holding it together. I know from his friends he’s been falling asleep during rehearsals, and even now, I can see him husbanding his meager resources to stand there and look normal.

  Damon loses energy. His skin turns swollen and pasty, distorting his once beautiful face. The doctors are confused. The great surgeon Jan Quaegebeur says to Doron, “I think you’re overreacting and things may not be as bad as you’re making them.” But things are awful. Eventually, Damon needs a heart transplant, the aftermath of which is a horror show. Doctors are absent when they ought to be present.