University of Chicago surgeons perform fourth US heart-liver-kidney transplant

May 30, 2003

On Wednesday, May 21, 2003, after seven months of waiting, including nearly six weeks in the hospital, 40-year-old Michael Gaynor of Chicago received a new heart, liver and kidney at the University of Chicago Hospitals. He is the fourth patient in the United States to undergo this complex multi-organ transplant, and the third at the University of Chicago.

Gaynor's heart and liver damage were caused by a rare, inherited, metabolic defect called glycogen storage disease (GSD) type IIIa, also known as Forbes' disease, which gradually damages the liver and muscles, including the heart. The kidney damage resulted from insufficient blood flow, caused by his severe heart failure. Transplantation not only should give him healthy functioning organs but also eliminate some of the worst consequences of his GSD.

During his six weeks in the hospital, waiting for a transplant, "Mr. Gaynor made us pretty nervous," said Allen Anderson M.D., assistant professor of medicine and director of the heart-failure program at the University of Chicago. "He was really quite sick. He could have died from his heart failure, and would have if he had to wait a few more weeks. He required a lot of pre-operative attention, including multiple IV medications to support his circulation and control his pulmonary hypertension."

Although multi-organ transplants have become more common, they still present a logistical challenge. "To make it work, we had to transplant the heart in such a way that it could immediately withstand the stresses of major abdominal surgery," said Valluvan Jeevanandam, M.D., professor of surgery and section chief of cardiac surgery at the University of Chicago, who has performed more than 700 heart transplants.

Gaynor's operation was made even more difficult because the combined effects of high blood pressure associated with his heart failure and clotting problems caused by liver failure increase the risk of excessive blood loss. "We had to be super-meticulous about bleeding," Jeevanandam said.

The transplant operations began at about 7:30 a.m. The cardiac team performed the heart transplant first. A second surgical team, led by David Cronin, M.D., Ph.D., assistant professor of surgery, performed the liver transplant. Once the liver transplant was completed, the heart team returned to close the chest incision. Then Cronin's team closed their incision, and a team led by J. Richard Thistlethwaite, M.D., Ph.D., professor of surgery, performed the kidney transplant, which was completed by about 9:30 p.m.

Gaynor has recovered well and was awake, alert and breathing on his own the day after his 14-hour surgery. A few days later, he sat up in a chair and walked a few steps. He was transferred out of the intensive care unit on May 28 and is currently listed in serious condition, with all three organs working normally. "I feel better every day," he said.

If all continues to go well, he could leave the hospital as soon as Wednesday, June 4.

Gaynor, who worked for the Department of Information Technology at Northwestern University until his disease made him stop last fall, was diagnosed with GSD at birth, which was confirmed as type IIIa at age 12. His early symptoms were an enlarged liver, which permanently altered the shape of his spine, causing scoliosis, and low blood sugar levels. By age 14 he began to have symptoms of heart damage. At age 19 he had first cardiac pacemaker implanted. Despite two more pacemakers, his heart damage steadily progressed.

People with GSD IIIa are born with a mutation in the gene for an enzyme (amylo-1,6-glucosidase) involved in the metabolism of glycogen, which is the storage form of sugar in the body. Normally, during exercise, or between meals, the body breaks down glycogen and uses the stored sugar for energy. Due to the enzyme deficiency, people with GSD are unable to use stored glycogen. This results in damage to the liver, where glycogen is made and stored, and to muscles, that rely on it for energy. The muscle damage often involves the heart.

Since the transplanted heart and liver do not have the enzyme deficiency, they should function normally. Gaynor may experience some fatigue because his skeletal muscle will still be unable to use glycogen normally. No one has ever had a multi-organ transplant for this disease before.

The glycogen storage diseases are uncommon, affecting about 1 person out of 25,000. One fourth of those cases are GSD IIIa, which occurs in about 1 out 100,000. The disease is an autosomal recessive genetic disorder, so patients with GSD have received one defective gene from each parent. The severity of the disease can vary dramatically.

Despite the rarity of this disorder, two of Gaynor's older brothers also have GSD IIIa, but not as severe.

After a good start, Gaynor still must clear several hurdles. He has progressed well after a very large operation, but the drugs that patients receive to suppress the immune response to a transplanted organ leave the patient more susceptible to infections. He could also still develop an immune response to the transplanted organs, but this usually can be controlled by adjusting medications.

There have been four heart-liver-kidney transplants performed in the United States. The first patient, transplanted at the University of Pittsburgh in 1989, survived about four months. Patients two, three and four were transplanted at the University of Chicago. Although one of those patients (#3) died soon after surgery, Gaynor is off to a good start and, 69-year-old Kent Slater (#2) of Rockford, Mich., who was transplanted four years ago on May 7, 1999, is still alive and well.

Update

In May 2007, Michael Gaynor celebrated the fourth anniversary of his heart-kidney-liver transplant. Read about Gaynor's recovery and how he's staying active.