By studying the sick hearts removed from four patients undergoing heart transplants, researchers have identified a protein and a signaling pathway that may contribute to sudden death in an inherited form of heart disease.
The patients were receiving new hearts to treat Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), a rare genetic disease that causes abnormal heart rhythms (arrhythmias) and increases the risk of sudden cardiac death, particularly during exercise or emotional excitement. ARVC affects between 1 in 1,000 and 1 in 1,250 people and is a leading cause of sudden death among young athletes who have no prior symptoms or cardiovascular disease diagnosis.
“We have identified a new pathway in heart cells that explains how arrhythmias occur in patients with ARVC,” says Long-Sheng Song, MD, professor of internal medicine at the University of Iowa Carver College of Medicine and one of the leaders of the study team that included researchers from the UI and the Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College in Beijing, China. “We hope that new drugs targeting this pathway can now be developed, which might help us treat this devastating, progressive disease more effectively.”
The team led by Song at the UI and Shihua Zhao, MD, at the Chinese Academy of Medical Sciences and Peking Union Medical College in China published their findings March 3 in the journal Circulation.
Read more at University of Iowa Health Care
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