Thursday October 18, 2001

Money will help establish registry of heart disease victims

A global research project headed by the UA will be awarded $7.4 million over the next five years to study a rare heart disease.

The grant was awarded by the National Institutes of Health to research the disease known as arrhythmogenic right ventricular dysplasia, a heart disease that can cause sudden death. The research project involves 10 research centers - nine in the United States and one in Canada.

Kathleen Gear, a research nurse Sarver Heart Center, said the disease known as ARVD occurs when muscle along the wall of the heart's right ventricle is replaced by fatty tissue, causing a rhythm disturbance of the heart.

Dr. Frank Marcus, a professor in the College of Medicine who worked with the doctor who originally discovered the disease, said the grant money will establish a registry in which each research center will enroll its patients.

Researchers would then be able to obtain information on which patients are at risk for sudden death and which medications would work best for the patient.

"The major thing we hope to accomplish is to identify and learn more about the genetic aspects of the disease," Marcus said. "We hope to identify the gene or genes responsible and what form of therapy would be most effective to direct at the disease."

The disease primarily affects people between the ages of 20 and 40 and accounts for up to 4 percent of deaths in young athletes.

Gear said that when fatty tissue replaces muscle in the heart, it can cause an abnormally fast heartbeat.

She said the disease can affect young athletes because only the left side of the heart is worked through physical activity. This makes the right side of the heart more vulnerable to the disease.

The disease is found worldwide and can be hereditary. Researchers have identified it as an autosomal recessive gene, which means an individual can only inherit ARVD if both parents carry the gene that causes the disease.

Gear said some people may carry the disease but never be affected by it if it remains dormant.

"We're looking for the autosomal dominant gene which is inherited from one parent and the individual has a 50 percent chance of inheriting it," Gear said.

She said that within the next five years, researchers will identify people diagnosed with ARVD and take blood samples from them and their families.

"A family that has two members with the disease gives us a better chance of locating the gene responsible," Gear said. "It makes the search for the gene much easier."

She said once the dominant gene is identified, medications can be determined that would work more effectively to treat the disease.