January 18, 2008

Center focuses on severing heart failure’s family ties

Featured Image

Rebecca Hung, M.D., talks with Christy Woodard, second from right, and family members, from left, Kendra Johnson, holding daughter Keondra Johnson, and Kendell Johnson, 14, who is holding Kendrick Johnson Jr., son of patient Kendrick Johnson. (photo by Neil Brake)

Center focuses on severing heart failure’s family ties

Congestive heart failure has plagued three generations of Christy Woodard's family, and with Vanderbilt's help, she is hoping to alter the fate of its youngest members.

Woodard, her children and grandchildren are enrolled in Vanderbilt's newly established Center for Inherited Heart Disease, trying to piece together their genetic puzzle in order to change their family picture.

Woodard, 36, works in Environmental Services and was only 6 when her father received a heart transplant. He suffered from dilated cardiomyopathy, which causes the heart to become weakened and enlarged, and unable to pump blood effectively. He lived for 10 years following his transplant.

Woodard had no reason to believe that she might be susceptible to the same disease.

Then, at age 22, when she was expecting her third child, she became ill. A series of tests determined that she, too, had dilated cardiomyopathy. On July 22, 2000, she received a heart transplant at Vanderbilt — and a second chance at life.

“I have been feeling great and feel like nothing was ever wrong,” she said.

When Woodard's 19-year-old son, Kendrick, was diagnosed with DCM last year, she was devastated.

“It's scary because my son was healthy and all of a sudden it's like, in the blink of an eye, he was sick,” Woodard said.

Kendrick, like his mother and grandfather, needs a heart transplant. An artificial heart pump, known as a left ventricular assist device, supports his damaged heart in the interim.

Now, Woodard is hoping earlier diagnosis and treatment will prevent the onset of cardiomyopathy in her younger son, 14-year-old Kendell.

“This case illustrates the devastating impact of an inherited cardiomyopathy on three — and possibly four — generations of a family,” said Rebecca Hung, M.D., assistant professor of Medicine and co-director of the Center for Inherited Heart Disease along with Vernat Exil, M.D., assistant professor of Pediatrics.

The new center is a joint venture of the Vanderbilt Heart & Vascular Institute and the Monroe Carell Jr. Children's Hospital at Vanderbilt, which provided an initial grant for the clinic.

“Having completed a highly detailed family history for the Woodards, the number of at-risk family members is astounding, even to me,” Hung said.

Testing has confirmed that Woodard and both of her sons have a large deletion in the dystrophin gene, which is often associated with mild cases of Becker muscular dystrophy and the DCM that may accompany it. Woodard's sisters are also suffering from heart failure.

“The Woodards obviously have had this long family history. Although they knew heart trouble ran in their family, they didn't know what gene caused it. Without knowing what gene, it's hard to predict who is going to be affected,” Hung said.

Hung estimates that 20 percent to 30 percent of young adults with DCM have a relative with the disease although they may not have been diagnosed or have symptoms.

The clinic will offer patients and their families clinical and genetic screening, education about their specific disorder, and counseling about their risks of progressing to symptomatic disease.

“Genetic testing isn't routinely performed” Hung said. “I think, in general, we take a family history and ask if there is anyone else in the family with heart disease. It's a very vague question.”

Hung and Exil work with a team of cardiologists and geneticists to treat otherwise healthy individuals who are at genetic risk for the development of potentially devastating cardiac disorders such as congestive heart failure and sudden death.

Research will be a secondary component of the center.

“We hope to build a large database to preserve clinical and biological data in hopes of altering the natural history of the disorder for future patients and their offspring,” Exil said.

The Inherited Heart Disease Clinic is a collaborative effort that includes Charles Hong, M.D., Ph.D., assistant professor of Medicine; Larry Markham, M.D., assistant professor of Pediatric Cardiology; Doug Sawyer, M.D., Ph.D., associate professor of Medicine; Dawood Darbar, M.D., assistant professor of Medicine and Pharmacology; Prince Kannankeril M.D., assistant professor of Pediatrics, Dan Roden, M.D., director of the Institute of Experimental Therapeutics, and clinical fellow Cheri Silverstein, M.D.