June 27, 2008

Collaboration at center of adult congenital heart disease program

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Robin Patton, here with her son, Thomas, 9 months, is among those helped by improvements in addressing congenital heart disease. Photo by Neil Brake)

Collaboration at center of adult congenital heart disease program

Due to improvements in pre-operative diagnosis, pediatric surgery, and post-operative care, children with congenital heart disease (CHD) are not only surviving, but thriving as adults.

From 1940 to 1959, only 10 percent of patients born with complex heart defects survived to adulthood. By comparison, from 1980 to 1989, nearly 80 percent have survived. Today about 1 million adults are living with CHD and they have unique problems that require specialized care from cardiologists with expertise in CHD.

The Vanderbilt Heart Adult Congenital Program is poised to be a leader in providing such care.

“Our program has a rich history of caring for congenital heart disease,” said Benjamin Byrd III, M.D., director of the program.

In fact, Alfred Blalock, M.D., Vanderbilt's first surgical resident, helped develop the Blalock-Taussig shunt, which led to the first blue baby operation in 1944 at Johns Hopkins.

CHD encompasses a broad range of defects from a simple hole in the heart to the more complex arterial switch and single ventricle lesions. Because many defects are corrected in early childhood, adult patients sometimes believe they are cured and no longer need the care of a physician.

“We have developed a program to care for adults with congenital heart disease because the traditional teaching dictated that 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 not really the case,” said Larry Markham, M.D., co-director of Vanderbilt's Adult Congenital Heart Disease program. “There is very rarely a cure because there's a lifetime of problems that await.”

Robin Patton, 26, a former child life specialist at the Monroe Carell Jr. Children's Hospital at Vanderbilt, was born with a ventricular septal defect. She was diagnosed as an infant when her pediatrician detected a heart murmur and was referred to Vanderbilt pediatric cardiologist Thomas Graham, M.D., who monitored her yearly for 18 years, during which time she lived a normal, healthy life.

When she was in college, however, she developed bacterial endocarditis and was hospitalized for 10 days. Graham discovered that Patton had a bicuspid aortic valve instead of a tricuspid valve and that blood was leaking around it — a condition known as regurgitation. Patton underwent the Ross procedure, open-heart surgery that involves moving the pulmonary valve to the aorta and putting a cadaver valve on the pulmonary side.

“I noticed a big difference in my health after the surgery,” she said. “I was able to go back to college, be with my friends, and keep up with them. I went back to dancing, which was something I used to do.”

Patton gave birth to her first child nine months ago.

“We weren't sure if my heart was strong enough or healthy enough to handle that pressure, but my doctors felt I had a good healthy heart for seven years, and they gave me the go ahead,” Patton said.

According to the Adult Congenital Heart Association, 50 percent of CHD patients born with complex problems face a high risk of developing new heart problems as they age. Common long-term complications include rhythm problems, valve problems, heart failure, heart infection and stroke.

Many adults with heart defects require additional cardiac surgery.

Vanderbilt has one of only a few centers dedicated to treating adults with CHD. Byrd, Markham and Frank Fish, M.D., director of the congenital electrophysiology program, have a collaborative relationship with pediatric and adult cardiac surgeons and interventional cardiologists that allows for continuity of care over the patient's lifetime.

“Patients with CHD know within five minutes of their physician walking in the door whether or not he has a grasp of the complexity of congenital heart disease,” Markham said.

Markham, for example, is an assistant professor of Pediatrics and Medicine and has completed a fellowship in congenital heart disease. Advanced training in the specialty is necessary, said Byrd, who, as the director of the adult echocardiography lab, has treated adult CHD patients for 20 years.

“Pediatric cardiologists are not trained in taking care of adults and their acquired diseases. Adult cardiologists have not traditionally been trained in congenital heart disease as seen in children,” Byrd said. “The thing that makes us special is we are trained and have focused for many years on CHD.”