Finding out why exercise causes heart rhythm disturbances in long QT syndrome

Adrenergic regulation of IKs and impairment in the hereditary long QT syndrome (Dr Vrijraj Rathod)

People with a type of inherited heart condition called long QT syndrome are at risk of abnormal heart rhythms. These abnormal heart rhythms put people at risk of sudden cardiac death, particularly during or following exercise. Long QT syndrome can be caused by abnormalities in protein structures, called ion channels, which coordinate the flow of electricity in the heart. In around half of all cases of inherited long QT syndrome, there are faults in proteins called KCNQ1 and KCNE1. Professor Andrew Tinker and his team at Queen Mary University of London have shown that these faulty proteins alter the electrical properties of ion channels. When we exercise the body releases molecules that prime the heart and blood vessels for the stress of exertion: the so called fight-flight response. Professor Tinker believes that the way these molecules interact with the faulty KCNQ1 protein may explain why exercise causes abnormal heart rhythms in long QT syndrome. He will supervise a clinical research fellow to investigate this theory. Through their BHF-funded research, Professor Tinker and his team will develop a deeper understanding of the mechanisms underlying long QT syndrome. They hope to turn these understandings into new therapies to treat the disease.