Adapted and reprinted, with permission, from a Discover Magazine article, appearing in “Vital Signs” – April 2012 issue
By Will Smythe
I was in the middle of a normal clinic day when the nurse placing patients in the exam rooms told me that someone had arrived with a diagnostic video.
“A video?” I asked, “a video of what?”
“I don’t know,” she replied, “but I set the CD next to the computer in the work room for you.”
As a general thoracic surgeon, I was used to viewing chest X-rays, CT scans and other similar radiographic tests, but not videos, as I was primarily a lung surgeon. Adult cardiac surgeons – those that operate on the human heart – review these video studies much more often when planning for what they do each day.
I walked over to the computer, sat down and put the CD into the drive. I watched as the program booted up, and the patient’s name appeared at the top of the study information on the screen.
“Hmm,” I said to myself, “a real-time cardiac MRI study – why would I be looking at this?”
I began to click through the images, first in still frames, and then with the heart contracting. This is where the “real-time” name comes from – seeing the heart anatomy, and function in a “moving Xray”, over time.
What I saw was frightening.
There was a large mass growing in the patient’s heart, in the back, or posterior wall of the left atrial chamber – this was simply the worst place in the heart to have a problem like this.
My own heart sank. Primary tumors, which originate in tissues rather than spreading there from some other place in the body, are uncommon in the heart. They occur in less than 0.05 percent of autopsies, or less than 5 in 10,000. Seventy-five percent of them are benign, but this one did not look harmless. Benign tumors typically grow out from the surface of the cardiac wall like a mushroom on a stalk; malignant tumors look more like a bulge of varying thickness in the wall. Most cardiac surgeons will encounter only a few benign primary tumors in a career, and many will never deal with a malignant one.
Imagine the heart as a large hollow bell pepper, divided into four chambers – two small ones on the top, and two larger ones on the bottom, with a small and a large chamber to the left, and one of each to the right. Now, rotate it forty-five degrees counter-clockwise, so that the two left chambers are mostly behind the two right ones. Next, lean the pepper back about forty-five degrees, so that the right upper and lower chambers are at about the same height, and the bottom of the heart where the two large chambers end is pointing out, termed contradictorily the “apex”. This is how the heart lies in the chest of most people.
The right atrium collects blood from the entire body once it has been depleted of oxygen, and pumps it into the larger right ventricle, which in turn pumps it into the lungs to get more oxygen. Once the blood leaves the lungs, it is collected into the left atrium, which in turn pumps it into the left ventricle, which pushes oxygenated blood throughout the body.
The right atrium and both ventricles are somewhat accessible to the surgeon’s knife. However, the left atrium, on the back of the heart next to the spine, is in a difficult area to reach. The lateral walls can be seen easily, but the anterior wall is in the center of the rotated and tilted heart, and the back wall is next to the spine, behind everything.
I watched the heart contract on the images. Blood in motion is much brighter than the structures surrounding it, which are depicted in varying shades of grey. The moving fluid is actually beautiful to watch, like white syrup saturated with white glitter, flowing in some areas smoothly, and in others with small whorls and eddy currents. These images were indeed beautiful, but what they indicated was anything but.
One of my partners, also seeing patients in clinic, wandered over and looked over my shoulder.
“You doing cardiac surgery now?” he asked.
“Not exactly,” I replied, but hey… take a closer look at this.”
The left atrium anterior wall, opposite the tumor, ballooned out awkwardly as it contracted. The blood didn’t flow smoothly into the area, and some was forced back out into the lung vessels. The mass was taking up a lot of space in the chamber, and impeding the normal flow pattern.
“I don’t know what that thing is,” my partner said, shoving his hands into his lab-coat pockets definitively, and leaning back away from the computer screen, “but if it gets about five percent larger, you’re going to find out on autopsy – that chamber is close to being completely obstructed, and if that happens….”
“Agreed,” I said.
I decided to call one of my adult cardiac surgery colleagues in the medical center, Dr. Mike Reardon, and ask him to take a look with me. I rebooted the study when he arrived.
“Man,” Mike said, “that’s a tumor alright, and in a bad place.” He was transfixed. I looked over at him as he flipped through the images, the grey/white/black rectangular representation of the computer screen reflecting back at me from his glasses.
“If we were to think about removing it,” I asked, “how would we approach it? Do you think its possible?”
The reclining office chair Mike was sitting in squeaked as he leaned back, and put his hands behind his head – his brow furrowed, and his expression searching. He was looking for additional data to begin to formulate his plan.
“How old is the patient?”
“Thirty-seven,” I answered.
“Any history of coronary disease? Is his heart normal? Does he have any other medical problems… lung disease? diabetes?…”
“I haven’t spoken with him yet, but the transfer notes from the outside doctor don’t mention anything.”
“Good,” Mike answered. He leaned forward now, and shook his head, as if agreeing with himself about what he was thinking, “there might be one way to remove this, but it is drastic.”
“What’s that?” I asked.
“We can take his heart out of his body, remove the tumor, reconstruct his heart, and put it back in.”
I hesitated, and muttered under my breath, “okay…, wow.”
He was describing an extremely rare procedure: a cardiac auto-transplant. We would operate on the heart outside the chest cavity and use cardiopulmonary bypass to support his body while we worked. The first successful auto-transplant to remove a cardiac tumor was performed in the 1990s. Since then, the procedure has been undertaken fewer than 50 times worldwide.
We went into the exam room together, and discussed the options with Mr. Johnson, an executive for a well-known company headquartered in another state, and his wife.
He told us that he had become increasingly short of breath with exertion, and now would experience the symptoms when just simply walking.
We told him the mass was probably a cardiac sarcoma, a malignant tumor originating in either the heart muscle or the blood vessels located there. We also told him that he faced significant risk of sudden death if the chamber became completely blocked, and that chemotherapy was usually ineffective for larger tumors of this type. Surgery was the only reasonable option, and we needed to move fast.
“However,” I related, “it will be complex, and there will be a fair amount of risk.”
“How much risk?” the patient asked, sitting wringing his hands together tightly.
This was a familiar site to me, as a cancer surgeon. I had learned over time that you can tell a lot about what a patient is thinking not only from the expression on his or her face, but also from the hands – were they relaxed? Were they waving above his head? Was she grabbing her thighs so tightly that the blood flow was no longer able to reach the fingers – blanched white? Were they shaking, or sweaty? The hands, unlike the eyes, may not be the window to the soul, but they may well serve at times as a reasonable road-sign to the subconscious.
His wife was sitting next to him, her own left hand resting on top of both of his. Her other hand, palm down on her lap, was visibly trembling, as well as her lower lip.
She spoke up, her eyes moist and wide with alarm, “we have children, doctor – young children.”
Doctor Reardon reached over and placed his hand on top of theirs.
“Let’s talk about it,” he said, softly, but confidently, “I think we can do this.”
He went on to describe the auto-transplant procedure. We would use cardiopulmonary bypass to support his body while we worked on the heart, outside his body. His wife looked horrified.
Bypass was literally that – collecting the blood in tubes inserted into the large veins which return spent blood to the heart, then sending it to a pump where it is also supplied with oxygen. The system then bypasses the heart and lungs, and delivers blood to the body via tubing inserted into the large vessel leaving the heart – the aorta.
“Have you ever done this before?” the patient asked.
“Yes,” Mike replied, confidently, but with no hubris, “I have done this two other times – and thats as many as anyone else, anywhere in the world, actually.”
“And the risk?” he asked.
“There are three things to worry about,” I replied, “one is whether or not we can remove the entire tumor, the second is whether or not we can put the heart back together again so that it will function normally, and the last thing is the overall risk of having to stop your heart completely, fix it, put it back in and make sure that it is able to start beating on its own again. We normally quote between a one and five percent risk of dying for heart surgery, but your risk will be higher.”
“How much?” he asked, his expression unchanged.
Mike responded, “best case scenario, or if all goes well in other words, perhaps a ten percent risk. Worst case? Hard to tell, maybe four to five times higher than that. In one of the cases I have done, it went very well. The other one did not.”
“Let’s do this,” the patient said, without hesitation.
His wife put her head on his shoulder, sobbing quietly, and daubing her eyes with a tissue.
ώ ώ ώ ώ ώ ώ ώ ώ
Two days later we were in the operating room, where I would assist Mike. As the anesthetizing medications were administered, Mr. Johnson’s blood pressure dropped dangerously. The anesthesia team quickly gave him epinephrine, a drug that increases the pumping action of the heart, and constricts the blood vessels, to bring it back up. “Not much blood getting into that left side,” the anesthesiologist said as he looked at the monitor. “That’s why his pressure dropped. Good thing you didn’t wait too much longer.”
We divided Mr. Jones’ sternum with the electric saw designed for this purpose. We are often asked by patients if we are going to “crack their chests”, but there is actually no “cracking” or breaking involved, just the smooth, low buzzing of the saw as it moves through the outer and inner rigid “tables” of bone – the hard part of the sternum, and the soft, trabecular marrow space that lies between. The marrow is loaded with fat, and in older patients, takes on the consistency of reddish-yellow butter. Interestingly, some heart surgeons start at the bottom of the breastbone and move upward, and others come from the top down. In this use of the terminology, I was a top-down man.
We placed a retractor to distract the two sides of the bony shield apart from one another, and open up the chest, exposing the soft organs beneath that evolution had engineered the now breached bony surface to normally protect. The sternal retractor is a metal device with two grasping arms, and a toothy ratcheted crank that is turned to move the arms apart once it is inserted into the narrow space left by the saw. I had always thought that it looked somewhat like a polished modern version of some sort of ancient torture device.
The outer sac in which the heart is bathed in fluid and protected, the pericardium, was opened carefully using an electric knife, and we inserted the tubing into the large vessels entering and leaving the heart so to establish bypass.
“Go on bypass!” Mike stated, loud enough for the technicians, called perfusionists, managing the pump behind us to hear, as well as the other team members. The heart began to empty of blood.
I had always imagined, as it emptied. and the movements became more exaggerated due to the associated loss of volume in the hollow chambers, that the heart looked as if it were struggling anxiously, perhaps desperately sensing that it’s vital function – to deliver blood, was threatened.
A very cold solution heavily laden with potassium was injected into the coronary vessels, which normally supply blood laden with oxygen and important nutrients such as glucose – the heart muscle’s fuel. In a few moments, the beating stopped. Cold saline slush was placed in the surrounding sac, to cool it further and maintain a state of suspended animation, and Mike and I went to work dividing the vessels entering the heart. Once they were divided, Mike lifted the organ up and out of the body.
I looked down into the patient’s open chest as Mike placed the his cold, now flaccid heart gently on the table a few feet away. The room had become eerily quiet save the sound of softly clanking instruments as they were moved around on the tables nearby by the nurses, and the rhythmic rotation of the rotor bypass pump. Where his heart should have been was literally an open space, a void, with tubes leaving it from the margins. It garishly reminded me of a spider without a head, or a body – only legs. The central part that should have been there simply wasn’t. It was an unnerving, unnatural sight. The only other time you got this particular view was during a traditional heart transplant procedure, immediately before you placed a donor heart into the recipient’s chest, after the removal of the diseased one. The difference in this situation; however, was critical. During donor organ transplants, we never take out the diseased heart until we had the new one in the room. In this case, there was no normal donor and a chance that we wouldn’t be able to fix one we took out, and put it back in.
I wrested my eyes away and looked over at the bypass pump a few feet away, like a mother’s body powering the movement of blood via a clear plastic umbilical cord – coming from, and leading back into the patient’s body. I looked, mesmerized for several seconds, at the smooth, steel rotary wheels turning quietly and dispassionately, compressing the tubing and moving, moving, moving the red fluid of life.
I wonder which of us will tell the perfusionist to just turn the machine off if this doesn’t work out?
I focused again on the task at hand. Mike worked quickly at the back table with my assistance. The heart was cradled in a cold saline-soaked cloth, to try to continue to keep it metabolically silent and sleeping as long as possible – to protect the heart muscle which was obviously not being supplied with blood.
“The tumor is bigger than it looked on the images,” Mike said, as he made a final pass with a pair of sharp scissors across a tiny peninsula of muscle attached to the mass, “but that looks like all of it.”
The fleshy heart wall and tumor were placed in a small blue plastic bucket to transfer to a waiting pathologist. We had to check the margins of the specimen to make sure that we had removed the entire tumor. Out of the body, the small, pink fleshy piece of tissue looked so harmless, but we knew better.
We waited, between furtive glances at the normally invisible standard institution-issue clock, with its unadorned black frame, white face, and black hands. The pathologist called into the room a few moments later, “there is some microscopic tumor at the margin.”
Mike sat there for a long moment, staring down at the opening in the back of the heart he had created. His head was bowed, and he was completely motionless – it almost looked as if he were praying. He remained this way for what seemed like several minutes.
It has been suggested by research studies that some surgeons have an unusual ability to focus on challenging tasks, and to completely eliminate outside “noise” in the process – at times for hours at a time. Some have called this the state of “flow” – whereby the conscious mind lets the subconscious take the wheel, and others have noticed this ability, paradoxically, in individuals suffering with attention deficit disorder. In the latter group, it seems that at some level of complexity they are able to shed the inability to focus, and actually become unusually focused on a task – as if they had been saving it up in a central nervous system bank account to withdraw only occasionally, and under the right circumstances.
He cleared his throat to speak, but did not look up. “If I take any more,” he said, “I may not be able to reconstruct it.”
“We can give chemotherapy,” I replied, “it has a better chance of working with microscopic disease only… better for him to have at least a chance, than not leave the OR, what do you think?”
“I agree,” he said, and went immediately to work.
“We don’t have much time,” Mike said, “we need to get this organ back in there.”
He sewed a piece of bovine pericardium—the heart sac from a cow which has been sterilized and preserved for this purpose—into the large opening left where the tumor had previously been. We then carried the heart back over to the patient and placed it in the void.
“Looks better already,” I said.
We then began sewing the large vessels back together, and allowed the heart to gradually re-warm. We knew from donor transplantation that a healthy heart will often start to beat once it is warmed, even without a blood supply re-established. We waited, without speaking of it, for the familiar and comforting signs of reanimation.
But this time… nothing happened.
We continued to work, completing the suture lines, and began to allow blood to flow back into the coronary vessels of the heart as we finished up.
“No action here,” the anesthesiologist said, watching the electrocardiogram.
Mike inserted a small needle into the muscle to measure the temperature.
“Should be working, the temp is good,” he said, “and, it’s a normal heart.”
Several more seconds passed.
Then, we noticed a little quiver down near the apex of the heart…, then another, and then the heart sprang back to life, beating vigorously. We removed the tubes, and closed the patient’s sternum in the usual fashion, using thick wires on the bony structures, and suture on the muscle and skin.
Mr. Jones recovered well, and was discharged from the hospital several days later.