Risk factors, Treatment Options and More than a Dozen Genes Associated with Long QT Syndrome (LQTS)

Institution:

School of Medicine and Dentistry at the University of Rochester Medical Center

Researchers:

Arthur J. Moss, M.D., Bradford C. Berk, M.D., Ph.D. Distinguished Professor of Cardiology, University of Rochester School of Medicine and Dentistry  

Impact:

Long QT syndrome is a rare, inherited disorder that makes the heart particularly susceptible to arrhythmias. If untreated, individuals with the disorder are at high risk of sudden cardiac death.

Timeline:

Moss evaluated his first patient in 1970 and in 1971 published the details of a new surgical treatment called a left-sided cervicothoracic sympathetic ganglionectomy that turned out to be remarkably effective in limiting the dangerous arrhythmias associated with the disorder. A few years later beta blockers came on the market and Moss found that they were beneficial in patients with LQTS. He established the Long QT Syndrome Registry in 1974, which currently includes information from more than 1,000 LQTS families from around the world and more than 2,500 affected family members. Moss and hundreds of scientists and physicians have used this data to further our understanding of risk factors, identify genes connected with the disorder and personalize treatment for patients.