Surgery is First of its Kind in U.S.
In order to save the life of a woman with a rare recurring heart tumor, cardiac surgeons at the University of Maryland Medical Center have performed a first-of-its-kind operation by completely removing both of the heart's upper chambers (the left and right atria), then reconstructing them with animal and human donor tissue. To make this possible, the woman's heart was removed from her chest for several hours prior to being re-implanted. The operation was performed on August 19.
The patient, 46-year-old Sandra E. Lanier of Massachusetts, needed the operation to remove a rare form of a benign, but potentially deadly, tumor, called a myxoma, from her left atrium. She had already had three open-heart operations to remove other tumors. But two years after each operation, the myxoma returned like clockwork at slightly different locations. Safe removal of the tumors became more of a challenge with each new operation. To halt this seemingly endless cycle, the surgeons decided to remove all of the microscopic atrial cells that might evolve into a tumor.
The surgeons needed total access to the heart, so they performed what is known as an auto-transplantation, an unusual procedure that has been described in medical literature only a half dozen times, primarily to remove cancerous tumors. While she was connected to a heart-lung machine, they separated her heart from her body, placed it in an ice bath and worked on it for more than five and a half hours, before sewing it back into her chest. Despite the complex procedure, the doctors say Ms. Lanier has had an excellent recovery.
"We reasoned that in spite of the risks from this approach, our experience with other heart tumors, heart reconstruction and heart transplantation gave us confidence in this unique solution," says Bartley P. Griffith, M.D., chief of Cardiac Surgery at the University of Maryland Medical Center and professor and head of the Division of Cardiac Surgery at the University of Maryland School of Medicine.
Cardiac surgeon James S. Gammie, M.D., performed the 12-hour operation with Dr. Griffith. Dr. Gammie, who is an assistant professor of surgery at the University of Maryland School of Medicine, had previously operated on Ms. Lanier at the University of Massachusetts, in two of the earlier procedures to remove the tumors.
During the operation, after her heart was taken out, Dr. Gammie removed the remaining atrial tissue and used cow tissue to replace the back portion of the atria and line up the pulmonary veins so they could be reattached to the heart.
The heart with the rebuilt atria (top), which Dr. Griffith created from animal and human tissue.
Dr. Griffith, meanwhile, rebuilt the patient's atria with a combination of animal and human tissue, creating passive, bag-like receptacles to channel blood returning from the lungs into the ventricles, the heart's main pumping chambers. "The animal and human tissue knitted together nicely with the remaining half of her own heart," says Dr. Griffith. "Her newly constructed heart functions well and her recovery so far has been smooth."
Dr. Griffith says there is a risk that blood clots will form in the rebuilt atria. To minimize that risk, Ms. Lanier is taking blood thinners.
Removal of the atria also cut out the source of electrical impulses that cause the heart to pump. Those impulses start in the upper part of the atria and travel through the atrial tissue. To maintain adequate heartbeat, the surgeons implanted a permanent pacemaker.
A myxoma is a benign, jellylike tumor composed of soft mucous material and fibrous strands. Most myxomas are found in the left atrium, the heart chamber that receives oxygenated blood from the lungs. Myxomas can lead to the formation of blood clots and obstruct blood flow through the heart. In the left atrium, they often grow in the shape of a stalk and can swing about as the blood flows. This movement may cause intermittent obstruction of the nearby mitral valve and halt the flow of blood from the left atrium to the left ventricle.
About four years ago, Dr. Gammie diagnosed Ms. Lanier with a syndrome called the "Carney Complex," a hereditary disorder that involves recurrent atrial myxomas and may be associated with various endocrine tumors. There is one obvious sign of the syndrome: patients with the complex have very dark freckles. According to Dr. Gammie, "There were pictures of patients in the article that I was reading, and I realized that was what my patient had because of the very prominent freckles around her face." Researchers have identified only about 400 people in the world who have this complex.
Dr. Griffith says last week's surgery builds on his experience with a similar heart auto-transplantation procedure in Pittsburgh three years ago. In that case, a woman had developed cancerous tissue that was confined to her left atrium. After removal of her left atrium, he created a new one using cow tissue.
"Beyond the technically challenging and creative operative procedure, the remarkable thing about the Pittsburgh patient is that she continues to be free from a very aggressive form of heart cancer and has returned to her vocation as an opera singer," says Dr. Griffith.
Operations like this may open up possibilities for patients who have been told they have no option except a heart transplant, says Dr. Griffith. "A big operation is the old-fashioned way to treat cancer, but it's the only way that we think certain heart tumors can be cured, because cancers inevitably return and ultimately affect a transplant."
While a transplant may have cured the myxoma problem for Ms. Lanier, Dr. Griffith says, "A transplant comes with the possibilities of organ rejection, the need to take anti-rejection drugs, and the 50 percent average 10-year survival rate. She likely will have a better long-term chance with a correction of her own heart, even though it was a very complex operation."
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