I was in the middle of a normal clinic day, seeing candidates for surgery, when a nurse told me that one of them had arrived with a diagnostic video. When I had a free moment, I walked over to a computer and put the CD into the drive. As the program booted up, I noticed that the video was a cardiac MRI (magnetic resonance imaging) study. I clicked through the images, and what I saw was frightening. A large mass was growing in the patient’s heart, in the back wall of the left atrial chamber, perhaps the worst possible place to have a problem like this. The right atrium and both ventricles are somewhat accessible to the surgeon’s knife. But the left atrium at the back of the heart next to the spine is a difficult, if not impossible, area to reach.
As I watched the video, more details emerged. As the left atrium attempted to pump blood, the wall opposite the growth ballooned out awkwardly instead of contracting with the rest of the chamber, its movement altered by the growth. The mass also took up a lot of space and was impeding blood flow. If it got just 5 percent larger, the chamber would be almost completely obstructed, resulting in a high risk of sudden death.
I called one of my cardiac surgery colleagues, Mike Reardon, and asked him to take a look.
“Oh, man,” Mike said, “that’s a tumor, all right—and in a bad place.”
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. 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.
“If we were to think about removing it,” I asked, “how would we approach it?”
“How old is the patient?”
“Thirty-seven,” I answered.
“Any history of coronary disease?”
“The transfer notes don’t mention anything.”
“Good,” said Mike. “There might be one way to remove this, but it is drastic. We can take his heart out of his body, remove the tumor, reconstruct the heart, and put it back in.”
“Okay…wow,” was all I could say.
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.
Mike and I went into the exam room to discuss the options with the patient, Mr. Johnson, and his wife. 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 said, “there will be a fair amount of risk.”
“How much risk?” Mr. Johnson asked quickly.
“There are three things to worry about,” I replied. “One is whether or not we can remove the entire tumor. The second is whether we can put the heart back together again so it will function normally. And the last thing is the overall risk of having to stop your heart, remove it, fix it, put it back in, and restart it.”
“We normally quote a 1 to 5 percent risk of dying for heart surgery,” I continued, “but your risk will be higher. The best-case scenario, if all goes well, is perhaps a 10 percent risk, and the worst case would be maybe five times that.”
There was no hesitation. “Let’s do this,” Mr. Johnson said.