The Open Heart Club

PART ONE

Near Encounters with the Real

1.

BY THE TIME his son Danny was born, Ludwig Spandau had lived three lives. He was fifty-three years old, with a young new wife, Sali, and a year-old daughter named Ruthie. He rented a walk-up apartment in Flatbush, Brooklyn, and worked on Long Island as a pharmaceutical chemist. He was a refugee, once divorced and once widowed. He spoke English with a thick German accent. Most of the people he had known had been murdered. On his forearm he had frightening scars where—so his son later suspected—Ludwig had removed his concentration camp tattoo.

Ludwig had been born to well-to-do Berlin Jews. His family had owned a paper business. He had served as a radioman in World War I, in a U-boat. After the war he had attended Humboldt University in Berlin, when Albert Einstein was on the faculty. Ludwig liked to dance. He met a girl named Friedel at a dance hall in Berlin in 1922 and got her pregnant. Their daughter, Inge, was born in 1923, and not long after her birth, the couple came to terms on a divorce. Soon after the divorce from Friedel, Ludwig married again, and his second wife, Lani, converted to Judaism.

They were assimilated, cosmopolitan people, raising two boys. The Nazis came to power, and Lani was murdered by the SS, and Ludwig’s father was killed at the concentration camp at Sachsenhausen. Ludwig, strong and able, was kept in Berlin, a slave on a work crew, repairing the city’s roads when they were bombed. After the war, he and his two boys—who had been in hiding in Bavaria—were reunited by the Jewish Agency in London, and all three came to the United States together in 1948.

Ludwig started a business formulating chemicals and trying to sell them. His English was poor. His business failed. He traveled across the country, looking for work. He returned to New York, and at a dance sponsored by a resettlement program, he met Sali. She was in her mid-twenties, almost half Ludwig’s age, the child of a refugee family from Hamburg; after years in Palestine, Sali had recently been reunited with her father in New Jersey.

War and calamity had erased whatever traces of religion Ludwig might once have had, but Sali was observant. In their Flatbush apartment, they kept a kosher home. Their building was busy with Yiddish-speaking immigrants, many with concentration camp tattoos. The crowded hallways smelled of onion and roasted garlic.

The Spandau’s second child, Daniel, was born in 1954. He would have been the final piece to cement their new American lives, but something was wrong with the baby. Little Danny was sickly and weak. He struggled for breath. He was prone to fainting spells. The more he was able to move around, the more frequently his lips and fingers turned blue.

At the local hospital, little Danny was X-rayed and given an electrocardiogram. Nurses fed the toddler vanilla pudding mixed with crushed radium pills and put him in a fluoroscopy machine: a kind of moving-image X-ray projected onto a screen. Their boy’s heart was deformed, the doctors told the parents, a cluster of four specific defects: (1) a hole in the center between the ventricles, the two largest chambers of the heart, (2) a narrowing in the pulmonary valve, the passage between the heart and the lungs, (3) a displacement of the aorta, the big artery that took the blood from the heart to the body, and (4) an enlarged right ventricle, the bottom right chamber. This complex of heart defects was called a tetralogy of Fallot, “tetralogy” for its four elements and “Fallot” for a nineteenth-century French physician, Étienne-Louis Arthur Fallot. Danny’s heart was shaped like a boot, and it was incapable of pumping sufficient oxygen to his body. In 1955 in Brooklyn, there was no cure for this condition, no such thing as open-heart surgery.

There was, however, a new kind of procedure that might alleviate his symptoms, one that had been invented a decade earlier. It was an operation not on the heart itself—an operation on the heart was impossible—but on the great arteries right above the heart. The newspapers called it a “blue baby operation.” The doctors called it a “Blalock shunt.” It had been invented by a remarkable team at Johns Hopkins University in Baltimore. Dr. Helen Taussig, a deaf, dyslexic pediatrician, had conceived of the procedure; Taussig, a rare woman in medicine in the 1940s, had been put in charge of these children doomed to die and figured out how to make their lives better. Vivien Thomas, an African American technician, had designed the tools and developed the surgical technique; Thomas, the only black man to wear a white coat at Hopkins in the Jim Crow South of the 1940s, could not use the main entrance to the building where he worked and was supposed to use only the water fountains and restrooms marked “colored.” Dr. Alfred Blalock, for whom the shunt was named, was the white southern surgeon who led the team and performed the first operations under Thomas’s careful guidance.

A Blalock shunt, the Brooklyn doctors explained to the Spandaus, would not cure their child. After the operation, Daniel’s boot-shaped heart would still have a hole in its center, a blocked passage, and a misplaced aorta, but the shunt would reroute the blood so that more of it went to the lungs. If everything worked out, the blood that fed his body would have a higher oxygen content. The surgery was risky. There was a chance of infection, pneumonia, brain damage, or death. The long-term prospects were uncertain. The first procedures had been done in the mid-1940s, and the oldest surviving patients were mostly in their late teens.

The surgery took place in 1956, when Danny was two years old. The incision began at Danny’s left nipple and cut across his side, under his left arm, all the way to his back. The doctors entered the chest cavity between the third and fourth ribs, deflating the lung and sectioning membranes until they had a clear view of the beating heart.

A Blalock shunt (sometimes called a Blalock-Thomas-Taussig shunt) is not an implant but a suture, in medical terms an anastomosis, between the two of the largest blood vessels that run right above the heart. The doctors dug into Danny’s chest and located the pulmonary artery, the artery that brings blood to the lungs. They clamped the artery. They had to move fast. The operation had to be done quickly to reduce the chances of damage to the child’s brain. A second incision was made in the left subclavian artery, the big artery that fed blood to Danny’s left arm. The surgeons sewed the two arteries together so as to divert more blood from the subclavian artery to the lungs. Too large an anastomosis, and too much blood would flow through, overwhelming the lungs and the heart. Too small, and not enough blood would flow through, and the procedure’s effect would be negligible. The racing doctors completed their sutures. Quick as they could, they released the clamps. The blood from the subclavian artery joined the blood from Danny’s starved pulmonary artery. More blood than ever flowed to the boy’s lungs.

When a Blalock procedure is effective, the oxygen-rich blood feeds the body, and the results are visible right away. On the table, the child’s skin turns from blue to pink. But that didn’t happen to Danny Spandau. He lingered listlessly in the hospital. He came home small and weak and blue. He was two years old and could barely walk.

Back home in Brooklyn, the bloodless, blue-skinned little boy, skinny as a skeleton, cast a shadow across the building of traumatized refugees. Ludwig carried little Danny everywhere. The boy rode on his father’s shoulders up to the apartment, and if anyone asked what was wrong, why the father would not let the boy climb the stairs on his own, Ludwig would bark at them in his German-accent English, “None of your business!”

Danny’s sister, Ruthie, remembers those years after the first surgery as frightening and sad. The apartment was dark, and the mood was bleak. She thought—as everyone must have thought—that Danny was going to die. Danny thought more about his father.

“I never thought about me,” Danny remembers. “I thought about him, and how it made him feel when other people commented. I don’t think I ever thought of myself as sick.” But he was always terrified of going to the doctor. “I remember the blood drawings. I was all skin and bones and it hurt. They were always doing blood tests and X-rays. All sorts of crowds came to look at me.”

He was a curiosity, a learning tool for the doctors and medical students, who could not cure his condition but were nonetheless fascinated by its progression. Every time he was supposed to go to the hospital, little Danny hid. His mother would plead for him to come out. His parents had to hunt for him and drag him from under the couch.

2.

THE SPANDAUS WERE referred to Dr. Aaron Himmelstein, an amiable-looking, bald, round-faced man with glasses, who was beginning to perform experimental open-heart surgery at Babies Hospital, part of the Columbia Presbyterian Hospital in upper Manhattan, about a mile south of the George Washington Bridge and across the street from Harlem’s Audubon Ballroom. Dr. Himmelstein accepted Danny as a candidate for open-heart surgery. The boy must have been a desperate case. He was scheduled to be Himmelstein’s twelfth tetralogy of Fallot patient. Seven of the previous eleven had died.

Danny was tiny at four years old, fragile and skeletal. His sister Ruthie was his babysitter. Danny remembers the courtyard of the apartment building, where there were concrete walkways separated from grass lawns by low-slung chain barriers. He described for me his immobility: one day, he went for a walk, his sister on one side of him, her friend on the other. Two boys on bicycles came speeding toward them. The girls darted to either side of the path. Both called to little Danny, but he was too slow to join either of them. The bicycles knocked him down.

The odds were not good for his survival. In the late 1950s, birth defects of the heart ranked as one of the top ten causes of death in the United States. In March 25, 1957, Time magazine’s cover story was “Surgery’s New Frontier.” It was all about hopeful new breakthroughs in heart surgery: “rib-spreaders,” “rib-shears,” and a heart “red and purple with a greyish cast, glistening under the lights, squirming and rippling.” Alarming photographs showed patients plunged into ice water in preparation for surgery. One patient, according to the article, was put into a six-foot-long kitchen freezer until her body temperature dropped to seventy-five degrees. The article mentioned but misspelled Danny’s deformity, “the most famed of all congenital defects, Pallet’s tetralogy,” in which “the blue baby has an amazingly consistent pattern of four anomalies combined.” The magazine noted grimly that “the death rate inevitably is high in heroic operations on patients already in poor condition.”

The cover illustration showed Dr. Charles Bailey of Hahnemann Hospital in Philadelphia, one of “the most daring innovators in heart surgery,” pushing against “the limits set by nature” far beyond anything that had previously been thought possible. In the front-cover portrait, Bailey’s comb-over was concealed by his white surgical cap; his shoulders were drawn as broad as Superman’s, and even his glasses looked like futuristic armor—a vision of the doctor as warrior, scientist, spaceman, and explorer of the unknown, “mummified,” as the article put it, “in sterile gear.”

The magazine issue, with its neo-Frankensteinian cover, hung on every newsstand in every train and subway station in New York City. It piled up on tables in doctors’ waiting rooms. In breathless prose, it recounted the history of heart surgery. Since 1944, Blalock and Taussig in Baltimore had put over 1,000 shunts into the great arteries above the hearts of blue babies, with an 85 percent long-term survival rate—a rate that could have given no cheer to Ludwig Spandau, as the procedure had not helped his son. In 1948, according to the article, Charles Bailey had performed the first- ever cardiac surgery on a human being by punching through the wall of a beating heart and clearing a clogged valve with his gloved finger. That remarkable feat was only prelude to the era of bypass surgery, when the heart was disconnected from the patient’s body and repaired.

Open-heart surgery was a postwar American invention, as miraculous as space travel and as bloody as the Battle of the Bulge. It began with children. The first series of successful surgeries happened in the mid-1950s in Minnesota, and the patients were all kids, blue babies like Danny who had been born with defective hearts. The advances in pediatric cardiac surgery led to operations on adults. In 1954 at the University of Minnesota in Minneapolis, Dr. C. Walt Lillehei had begun performing bypass procedures by laying out two operating tables: one for the patient and one for the patient’s parent. Lillehei connected the parent’s and child’s bloodstreams to each other. The parent’s heart filled the child’s body with oxygenated blood, while Lillehei cut into his patient’s little heart and repaired its deformities, sewing shut holes in the walls between the chambers. The parents risked their lives for their kids. A mother had gone brain-dead. Once, when Lillehei could not get a parent to participate, he had used the lung of a dog to oxygenate his patient’s blood.

By 1957, Lillehei’s two-patient bypass procedure had given way to use of the heart-lung machine. Three hundred miles south of Minneapolis at the Mayo Clinic, Dr. John Kirklin’s heart-lung machine procedures had progressed to the point where the majority of his patients with simple defects survived. In 1957, complex conditions like tetralogy of Fallot were just beyond medicine’s reach, but operations with these new machines were now being performed around the country. Time magazine described Charles Bailey’s heart-lung machine as “an odd-looking device”:

Not long after the magazine came out, the date for Danny Spandau’s surgery drew near. In preparation for the operation, Danny had a cardiac catheterization. The inventors of the cardiac catheter were two Columbia doctors, Andre Cournand and Dickinson Richards, and they had won the Nobel Prize for their invention just two years before. Danny was strapped down on the operating table, while the doctors cut a hole in his thigh and guided a wire up inside him. Through the catheter, the doctors released dye into his bloodstream. Neither Danny nor I remember our early catheterizations, but I have spoken to other patients who do, and they recall feeling the dye as it released and how that was painful and terrifying. The masked doctors gathered around Danny’s gurney, put on lead vests, and with giant X-ray guns shot images of his chest from two different angles, one from above and one from the side, a dozen X-rays per second, to get a sense of the flows and dynamics of his heart.

Not long after the catheter exam, the hospital called the Spandaus with bad news. Dr. Himmelstein had taken sick. He had brain cancer. He would not be able to operate. The surgery would have to be postponed. The family had to wait another year, with their fainting blue baby at death’s door. Columbia was not able to find a replacement heart surgeon. Danny’s case was taken over by Himmelstein’s young chief resident, Dr. James Malm.

In 1960, six-year-old Danny Spandau was listless and weighed just twenty-seven pounds. When he rested, he tended to draw his legs up against his chest, a squat typical of children with tetralogy of Fallot; it relieves some of the pressure on their chest. The Spandaus met Dr. Malm up in Babies Hospital. He was tall and graceful, precise in his speech, elegant, and well-mannered. His hair was parted neatly. He had high cheekbones. His features were small, feminine, almost Asiatic, and his skin strangely flawless. In blue and red pens, Malm drew careful pictures of Danny’s heart and described to the Spandaus the surgery he would perform. He showed the four chambers of the heart, the big ventricles below and the small atria above.

In a normal heart, the blood flowed in through the vena cava, down into the small chamber on the right side, the right atrium. From the right atrium, it descended into the right ventricle, the big powerful pumping chamber that sent the blood up through the pulmonary artery into the lungs. From the lungs, the blood came back into the left atrium, then down into the big left ventricle, which shot the blood out through the aorta to fill the body with life. Danny’s heart, Malm explained, had four defects, but Malm focused on two of them. There was a big hole in the ventricular septum, the wall between the two big beating chambers of the heart, so the oxygenated and unoxygenated blood comingled and went out the aorta together. There was also a narrowing, a stenosis, in the valve between the left ventricle and the pulmonary artery—that stenosis limited the amount of blood that could get to Danny’s lungs. Malm was going to patch the hole in the middle of the heart, and he was going to widen the gap between the pulmonary artery and the left ventricle.

He called this operation a “complete repair.” What the Spandaus did not know in that first meeting was that Jim Malm had never in his life performed open-heart surgery.

3.

HEART DEFECTS ARE the most common of all life-threatening birth defects, affecting 1 in every 110 babies. Roughly one in every three kids born with a heart defect requires surgery, which means (more or less) that there’s now a kid with a surgically repaired heart at every school in the affluent world. Pediatric cardiologists and pediatric heart surgeons are good at what they do: these days, in the United States, over 85 percent of kids with deformed hearts survive surgery into adulthood. Still, congenital heart defects remain, of all birth defects, the number one cause of infant death.

Congenital heart disease is among the most common chronic diseases in the United States. People with repaired hearts are living long lives—healthy, give or take, but in need of constant monitoring and attention. You probably have a neighbor, a relative, a friend, or a coworker with a repaired heart defect. Most of us look entirely ordinary, even when we’re in deep trouble. We are invisible. Approximately nine in ten Americans with moderate to severe heart defects do not get the lifelong care and monitoring that they need.

Tetralogy of Fallot is the most common complex congenital heart defect. Fifteen years before Jim Malm, all the kids with tetralogy died—most died in infancy, but the healthiest lived crippled lives into their teens. Fifteen years after his breakthrough surgeries, almost all the tetralogy kids in the United States survived surgery, and many of us have thrived—snowboarder Shaun White, a three-time Olympic gold medalist, was born in 1986 with a tetralogy of Fallot.

In 1960, when Danny was rolled into surgery, a good portion of tetralogy patients didn’t make it through the operation. Mortality rates varied from hospital to hospital, but they were so bad that Helen Taussig—the most prominent pediatric cardiologist of all—argued that corrective surgery on “tet” patients wasn’t worth attempting. At Columbia under Himmelstein, more than half of the patients died.

In 1960, blood could not be stored safely long term. The donors had to be on hand for surgery, so Danny was accompanied by his older brother Stevie, one of the two sons from Ludwig’s first marriage—as a child during World War II, Stevie had hidden from Nazis. Now he was a US marine. He came to the hospital with twelve of his Marine Corps buddies, all of them ready to donate blood.

In those days at Columbia Presbyterian, there were no operating rooms specifically designated for cardiac cases. “Every operating room was occupied every day,” Malm remembered. “Gall bladders, colon cancer, breast surgery, thyroids.” He had to fight for operating room space. “There was no room at the inn. If I increased my volume, then one of the other surgeons couldn’t do his thyroid, couldn’t do his breast.”

Danny was rolled in. The anesthesiologist put a mask over the boy’s face and told him to count down from ten. Something extraordinary happened as he passed out. “I’m a scientific guy,” Danny told me. “I’m an analytic chemist by training. So I’ve explained it not as an out-of-body experience, but maybe it was. Who knows? It may have been the reflection in an overhead lamp. All of a sudden I wake up and see the reflections of all the doctors around the table. I see the top of the doctors and me lying on the table. It was so clear. It was literally like being with my back on the ceiling of the OR looking down at the operation. I saw the doctors in all their scrubs. And I saw myself lying on the table, being worked on.” He felt completely removed from his body, a spirit looking down at the surgery. “And that’s a memory I’ve had essentially all my life.”

Carefully, Malm sectioned the little boy’s chest. The heart-lung machine was of the new disc oxygenator type, a device about the size of a big fat salami, stored in something the shape of an industrial-sized sewing machine. It sat on a rolling cabinet, and from it extended long clear tubes. The machine was primed with a mixture of blood and blood thinners. One tube’s canula was inserted into Danny’s aorta, the other into his vena cava above his right atrium. Danny’s blood came into the machine and coursed over a series of discs. Each bloody disc was perfused with oxygen, and the oxygen-rich blood was sent back into Danny’s circulatory system. The heart-lung machine regulated the speed of blood flow, and by slowing down the blood, Malm lowered Danny’s body temperature to eighty-two degrees. The induced hypothermia slowed Danny’s metabolism and gave him some protection from damage that might come from oxygen deprivation during surgery. The heart was drained entirely.

Malm cut right through the pericardium, the rough sack of protective tissue that contains the heart. Now he had to attack the heart muscle itself. Other surgeons had favored a “median,” or horizontal, slice across the right ventricle, but instead of going side to side, Malm made a “vertical right ventriculotomy incision.” He had devoted himself to study of the heart’s electrophysiology—the system of electrical currents that control the heartbeat—and this seemed less likely to interfere with the circuitry of the heartbeat. It also gave Malm a better view of the hole in the septum.

The interior of the right ventricle wall is not smooth but, in doctors’ terms, trabeculated, which means it’s made of a crisscross web of muscle fibers. Even with today’s powerful imaging technology, it can be difficult to locate the boundaries of each ventricular septal defect. Malm found Danny’s by feel. He took a Teflon patch and stopped the gap in the middle of the heart, between the muscular wall at the bottom of the ventricles and the soft tissue at the top. He used mattress sutures to attach the patch, the kind of big, railroad-track loops that are inelegant but extremely secure. Once Malm was sure there was no leak at all, he attacked the stenosis of the pulmonary valve, the blockage on the right side of Danny’s heart.

He made a generous hole between the right ventricle and the pulmonary artery, slicing any tissue that was in the way and putting in an outflow patch to smooth the passage of blood toward the lungs. He wanted to get as much blood moving as he could, and he wanted the space wide open. Where a normal heart would have a pulmonary valve, impeding blood flow backward from the lungs, Danny would have an open passageway.

Malm shut the hole in the ventricular septum. He relieved the pulmonary stenosis. He sewed Danny’s heart shut, doing as little damage to the muscle as he could, and he stitched up the pericardium. The boy was taken off the heart-lung machine. Blood filled his heart and the chambers began to beat. The boy’s temperature returned to normal, and his blood pressure did too. The whole operation had taken about an hour and a half. The crucial thing, according to Malm, was to get the blood flow as near normal as possible. “No leaks!” he barked at me over the phone when I asked him his secret to keeping a child alive.

There was no special recovery room for cardiac patients in 1960. Danny recalls a big room with lots of other kids. He woke up under a clear plastic oxygen tent. For the first two days after surgery, he lived in a high-humidity bubble. His parents hovered close by but could not touch the top of his body. IV tubes replaced his lost blood and body fluids and gave him the necessary sugars and electrolytes. Nurses came by with shots of penicillin and streptomycin and delivered the shots to the muscle of Danny’s legs.

“I pinked up right after surgery,” Danny told me.

For a time, after the oxygen tent was removed, Danny was confined to a wheelchair. “I remember my hands were so dirty from wheel-chairing, and it was so disgusting that I never sucked my thumb again.” After six days, he was allowed to walk. When Danny came home from the hospital, everything had changed. He could climb the stairs to his apartment. He began to put on weight and to grow. The darkness was lifted from the Spandaus’ lives. When Danny was strong enough, his brother Stevie took him to a marine ball to celebrate his resuscitation, and Danny was shown off in front of all the young men who’d given their blood to him.