The Heart Healers (24 page)

Each year we do about a hundred thousand heart valve surgeries in the United States, about a quarter of a million worldwide. Today we operate on patients in their nineties. Despite accepting people who were inoperable in earlier years, we have reduced the operative mortality from 50% in Starr’s first year to about 3% today. In otherwise healthy patients, our surgical mortality rate is less than 1%. For this reason it is often a fool’s errand to compare mortality rates among surgeons. In major medical centers, a surgeon’s mortality rate is more a reflection of the number of desperate, high-risk patients she accepts than her surgical skill. Yet despite all these advances, we are poised to enter a brilliant new era of treating valve disease. As we will see in our chapters that deal with the future, many procedures that began in the operating rooms of cardiac surgeons will be performed in the catheterization laboratory.

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ALBERT STARR AND
the cardiac surgeons had proven that we could completely reverse one form of heart failure. Restoring valve function allowed a grateful heart to return to work without facing an insurmountable resistance from a stenosed (narrowed) valve or volume overload from an incompetent valve. The impact of artificial heart valves on the thinking of surgeons was profound. With the heart-lung machine, it seemed, anything they could imagine, they could do. Some began to imagine the inconceivable: that one day an intrepid surgeon might replace a heart itself, not with an artificial one, as Lowell Edwards had imagined, but with a real one. Was the heart-lung machine so effective, so mind-bendingly potent, that it could sustain life in a patient who had no heart at all?

14

“THE SHIP HAS WEATHER’D EVERY RACK, THE PRIZE WE SOUGHT IS WON”

It is infinitely better to transplant a heart than to bury it to be devoured by worms.

—CHRISTIAAN BARNARD, SOUTH AFRICAN CARDIAC TRANSPLANT PIONEER

AS WE ENTERED
the mid-1960s, buoyed by Al Starr’s spectacular valve surgery at the beginning of the decade, a few hundred miles south at Stanford University, surgeon Norman Shumway focused on the other major cause of heart failure: damage to the heart muscle, most commonly caused by CAD. After a third heart attack, the heart often has so little remaining muscle that no amount of supportive treatment is possible. In those years, the patient was doomed. We had nothing more to offer. Shumway aspired to the most impossible surgical feat of all time: replacing the whole heart in patients with severely damaged heart muscle. For those of us who lived through it, there is no more shocking, more memorable story in cardiology than the development of the cardiac transplantation.

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WHEN THE SANTA
Ana winds of October suddenly arrive in Los Angeles, you do not need a weatherman to tell you. The winds that blow off the desert to the east tear relentlessly at every window and door, demanding entry; dry and hot, they scorch your face like a suddenly opened oven. Riding on the gusts of the Santa Ana is an unspoken fear. Is this wind the killer, the one that brings wildfires that man cannot control? It was on such a day in the mid-1990s that I received a call from a valued friend from across the country, the past president of the American College of Cardiology. The call had an unusual provenance. In academia, an “endowed chair” refers to a philanthropic donation, which supports in perpetuity the salary or the research of the so-honored professor. Our patient comedian George Burns created the George Burns and Gracie Allen Endowed Chair in Cardiovascular Research, and I was its first recipient. My cardiology friends around the country referred to me simply as “the George and Gracie.” So I was not surprised to learn that the patient was one of George’s professional colleagues.

“Jim, I’ve been taking care of a Hollywood personality.” I recognized the patient’s name immediately. I will call him Marcus Stuart. “He’s seventy years old, but he is still working. He has severe congestive heart failure, which I’ve managed for a few years. He is in otherwise very good health, but his heart has brought him to the end of the line. It’s sad because he’s over sixty-five, so we cannot consider him for a cardiac transplant. He is in bad shape and I expect that he’ll die in the next year. But between now and then he is going to require multiple hospitalizations. He and his wife are going to need someone to shepherd him through the final days. He has homes in both Manhattan and Los Angeles, and he’s returning to LA now. Can you help?”

I thought about congestive heart failure (CHF), a disorder as smothering and potentially lethal as that Santa Ana wind. CHF has many causes, but the most common are valve disease and death of cardiac muscle, the latter from a heart attack or from an infection. The volume of blood that the left ventricle ejects on a single beat, called ejection fraction, is normally 60 to 70%, whereas in CHF it falls to 40% or less. In severe heart failure the ejection fraction is much less than half-normal. The resulting symptoms are weakness and fatigue because of diminished flow to vital organs, and shortness of breath because blood backs up into the lungs.

CHF is now one of society’s most pressing public health concerns. About 5 million Americans now have CHF and over half a million new cases appear each year. About a quarter of the patients with CHF are less than sixty years of age. CHF is a high-risk disease, with a mortality rate over 50% within five years of first diagnosis. The sudden death rate in CHF is about nine times that of the general population. CHF ranks at the very top of the nation’s health-care issues, because it is an exceptionally high-cost illness and patients require repeat hospitalizations—now more than all forms of cancer combined.

The medical treatment of a patient dying of CHF has much in common with caring for terminal cancer. Today, a very good specialist can reduce emergency room visits and hospitalizations by careful adjustment of potent drugs with different modes of action. A great doctor goes a step further, recognizing that the primary goal of treatment subtly morphs from prolongation of life to maximizing the quality of remaining life. Doctors who are able to do this focus equally on the physical and the metaphorical heart, supporting the patient on every step of the downhill descent to death. Because this requires both time and empathy, many doctors are not able to find that exquisite and ever-changing balance between honestly answering a question and never relinquishing hope. In my medical school years, when we had virtually no effective therapy to offer a patient with terminal heart failure, my professors quoted the great nineteenth-century physician Oliver Wendell Holmes, who taught us to “beware of how you take away hope from another human being.” It is a life lesson that I pass on to my own students in an era of statistical probabilities.

A few days later Mr. Stuart stood in the doorway of my office. Tall, slim, and distinguished in a black open-neck sweater, with a gray beard and mustache, he cut the perfect image of a distinguished Hollywood personality. We chatted amiably about our mutual friend who had referred him to me. In the process we discovered that we shared the same beach in Malibu. He lived about a ten-minute walk north along the Pacific. In his medical history I clicked off all the causes of congestive heart failure. He seemed to have none of them … certainly none of the most common causes like CAD, hypertension, or valve disease. I diagnosed Mr. Stuart’s problem as idiopathic cardiomyopathy. Although it sounds profound, the term disguises abject ignorance. Idiopathic cardiomyopathy simply means heart muscle malfunction of unknown origin.

As I examined Marc, I focused on assessing the severity of his heart failure. His thin frame was due to muscle wasting. His mouth opened slightly with each breath. His neck veins were distended, reflecting the inability of the heart to pump out all the returning blood. The muscles between his ribs pulled in hard with every breath. Listening to his lungs I heard the cardinal finding of congestion, the crackling sound of fluid in his airways. By tapping on his chest to outline the border of his heart and lungs, I found that Marcus’s heart was markedly enlarged, the result of its inability to effectively pump blood to the rest of the body.

The echocardiographic image spoke a thousand words: his heart looked more like it was twitching than contracting. My assessment confirmed every detail I had been given on the phone. Marcus Stuart had a heart so damaged that he very likely had less than a year to live. He had passed the point at which we had an effective medical therapy to offer. He needed a new heart. I asked him to get some routine lab work and to come back to see me again in about a week. I got the lab results back the next day. The function of his other organs, including his liver and kidney, was remarkably normal. I sat looking at the results for a few minutes. Marc was a man with a body too good to die, but with a heart beyond repair. I walked across the hall to plop down in a comfortable chair in the office of our cardiac surgeon Alfredo Trento, who years before had found a way to treat Maria, our child with tetralogy. Alfredo is also one of the nation’s leading transplant surgeons.

“Alfredo,” I said, “I have a seventy-year-old man, otherwise highly productive, with end-stage idiopathic cardiomyopathy. He’s otherwise in good health, and I feel that he is physically able to withstand the trauma of heart surgery. If you were willing to consider an age exception, is it even possible?”

Alfredo gave me a big smile. “Sometimes people get lucky, Jim. It turns out I’m initiating a heart transplant program for otherwise healthy patients over sixty-five. Mine will be one of the first in the country. My idea is that since people over sixty-five have a shorter natural lifespan, we could use hearts from older potential donors that would be rejected for a young person. So yes, I’d consider him. But he’s gotta have very good insurance. A heart transplant costs about three hundred thousand dollars.”

When Marcus came to see me the following week, I gently told him my honest assessment of his prognosis. “I knew that you would say that, Jim,” he said. “My body tells me that, too. I have no strength and I can barely breathe.”

“Marc, I want to offer a ray of hope,” I replied. “Would you consider a heart transplant?”

Marcus Stuart’s reaction was as profound as any I can recall in any patient over my forty years of cardiology. I was looking at a man who had recently passed through the stages of denial, anger, depression, and acceptance of death. In the space of a single sentence, he was being asked to discard those months of wrenching adaptation. Marcus sat there, self-consciously searching for an emotionally safe place within himself where he might be allowed to hope. His eyes turned glassy. His mouth opened as if to reply but he couldn’t speak. He rubbed his eyes. He held up his right hand in the universal stop sign. For a moment I thought he was telling me to stop, until I realized he was signaling that he needed a few moments before he could speak.

I reached out to rest my hand on his arm. “I understand what you’re going through,” I said. “I have as much time as you need.” I waited quietly. Marcus did not need to hear my words. He needed to listen to his own heart.

Finally he cleared his throat and said, “Of course I want a transplant. But you forget I am seventy. Everyone knows I am seventy years old. I can’t just claim I am sixty-five and get a transplant. Hearts are so scarce. They have to go to younger people.” Reassurance had to precede hope.

I explained Alfredo’s new over-sixty-five transplant program, which used hearts that were deemed too old for younger patients. After he realized the reality of potential transplant, I tried to caution him about the ordeal of multiple cardiac biopsies and the side effects of immunosuppressive drugs. It was not an easy road, even for a young man. I had the impression I’d scarcely been heard as he brushed aside these concerns. The white-hot radiance of reprieve from death blinded him to lesser concerns. Then I delivered the sobering reality. Even if both he and Alfredo decided to proceed, he still had to have a willing insurance company. The company could easily refuse to pay, arguing that the current standard of care was that transplants were not done in people over sixty-five years.

“I have insurance through the Guild,” Marc replied. “They take very good care of us. But this is a huge, one-of-a-kind request that no one has ever made before. So I have no idea what they’ll say. Plus they may take a month or two to decide and I might not live long enough to find out. So don’t let the cost slow us down. Madeline and I will find the money,” he said, “and I will deal with the Guild.”

Alfredo saw Marcus and agreed that he was a suitable candidate for his over-65 transplant program. Marcus had one stipulation: “I want all my records to be in an assumed name. In the movie business the people who finance the movie require a completion bond on the stars, to protect them from loss if the movie does not get made. If everybody knows I had a transplant, insurance companies might refuse to issue a completion bond, and I would never work again.” I arranged the assumed name, and promised I would tell no one his secret. I passed the baton of Marcus’s care to the cardiologists who worked with the transplant team. It typically takes about six months to a year to find a heart. Selection of a heart depends on a waiting list stratified by both risk of imminent death and duration of waiting. The donor heart is then tested against each potential recipient’s profile until a compatible match is found.

Marc’s ordeal had just begun. His would have an agonizing wait, knowing that he had to be constantly available to race to the hospital on short notice, and that about a third of those on the recipient list die before their match is found. Then in the ninth month after his name was entered on the transplant list, we received the call. His new heart would arrive by plane from a northern city in an ice container hardly more sophisticated than the one used for soft drinks on the beach. Marc swallowed hard in both anticipation and fear as he raced from Malibu to the hospital for his gamble with destiny. Would this be the end of life, or a new beginning?