March 8, 2012

Transplant eases heart patient’s complex problems

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Billie Jean Grace sports a T-shirt bearing the date of her heart transplant at Vanderbilt. (photo by Anne Rayner)

Transplant eases heart patient’s complex problems

Billie Jean Grace, 35, of Hopkinsville, Ky., wanted what every young mother wants: to be able to play with her children.

A congenital heart defect that required surgery during infancy and medical management throughout her life was taking its toll on her, eventually leading to congestive heart failure. It left her so fatigued that she could barely muster the energy to attend church or to take care of basic household chores.

Grace was born in 1976 with a condition known as transposition of the great vessels, whereby the two major vessels that carry blood away from her heart — the aorta and the pulmonary artery — were switched.

She underwent surgery at age three months to correct the defect and proceeded to have a relatively normal childhood. She steered clear of sports throughout middle and high school, opting for cheerleading instead.
After giving birth to her daughter in 2000, she developed an abnormal heart rhythm. She ran into real trouble, though, when she had her son in 2006.

“My placenta ruptured, and I ended up in cardiac arrest. I woke up three days later from drug-induced coma,” she said.

She received an implantable cardioverter-defibrillator (ICD) to provide monitoring and an electrical shock should she have a life-threatening heart rhythm. Still, she began a slow decline into congestive heart failure.

Fifty years ago, patients like Grace who were born with complex heart problems had a 10 percent chance of survival to adulthood. Now, thanks to surgical advances, care provided throughout childhood and programs like the Vanderbilt Heart Adult Congenital Program, these patients are living longer and receiving the continuity of care they need to survive, and thrive.

“Vanderbilt has supported a program to care for adults with congenital heart disease,” said Larry Markham, M.D., one of Grace’s physicians, and co-director of Vanderbilt's Adult Congenital Heart Disease program.

“Traditional teaching suggested that after an operation if all the problems in a patient's heart are fixed, then that would mean they were cured. As these patients have aged, we see that's rarely the case. It is unusual that there is a cure because there's a lifetime of problems that await.”

For Grace, the solution to her problem would be a heart transplant. Due to the complexities of her congenital heart defect, a team of Vanderbilt specialists from adult congenital, pediatric cardiology, electrophysiology and the adult cardiology heart failure team collaborated on her case.

Because of her complex congenital anatomy, her transplant was performed jointly by Simon Maltais, M.D., and the congenital surgical team of David Bichell, M.D., and Brett Mettler, M.D. Such collaboration is what sets Vanderbilt apart, Markham said.

“Billie Jean really is the epitome of what this program is able to do. It’s the only program in the state that has the resources and expertise to do all of this,” Markham said.

Patients like Grace represent a minority of the adult cardiovascular disease population. There are more than 24 million adults with cardiovascular disease in United States, and one million of those have congenital heart disease.

“It’s a small portion of adult cardiovascular care, but, if you look at the top tier adult cardiac programs in the country, in addition to other advanced specialty programs, they all support a robust congenital program as well. We’re no different in that respect.

“Our program is one of only 17 adult congenital programs in the United States that see more than 1,000 patients per year,” Markham said.

Grace received her heart transplant on Feb. 19, and is recovering nicely for the next month in Nashville before she returns home to Hopkinsville with her husband and children.

“I am feeling wonderful. Everybody has been awesome, every doctor, nurse, care partner, down to nutrition,” Grace said of the care she has received at Vanderbilt.

“I am looking forward to playing with my kids. I just want to be able to play with them. They like to go outside and run around. I want to go out there and kick that soccer ball around like they do.”