Jason Roberts is behind one of seven McMaster-led research projects that have received a total of $61 million from the Canadian Institutes of Health Research (CIHR) to advance medical research, training and innovation.

McMaster University researcher Jason Roberts is tackling a lethal heart condition with a potentially revolutionary treatment, helped by $2.7 million in federal funding announced on Jan. 19.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disease that causes fat and scar tissue to replace healthy heart muscle cells, triggering irregular heartbeats, heart failure and sudden cardiac death.

However, a drug called tideglusib could prevent ARVC’s onset in people carrying the gene, having proven effective when tested using animal models.

“ARVC affects thousands of patients and families across Canada. It can be a helpless feeling to carry the gene for ARVC and know that no matter how you live your life, you may some day develop a deadly form of heart disease,” said Roberts, an associate professor of the Department of Medicine and scientist at the Population Health Research Institute.

“The goal of our research program is to develop an effective medical therapy that is able to prevent the onset of ARVC and effectively treat patients in whom the disease is already established. There is presently no medical therapy that targets ARVC’s underlying pathophysiology.” 

Currently, the only treatment for ARVC that has been proven to save lives is the insertion of an implantable cardioverter-defibrillator (ICD) in people with the condition.

Roberts said that while ICDs prevent cardiac arrests and save lives, the shocks they deliver are painful and patients suffer multiple device-related complications over their lifetimes.

He added that Canadian researchers have already made important contributions in the field of ARVC. Canada has established the Hearts in Rhythm Organization (HiRO) to improve care for people with ARVC through collaboration and research.

To date, HiRO’s ARVC registry has enrolled almost 1,000 people with the condition, as well as their family members. Many of them will be eligible for enrollment in the upcoming tideglusib clinical trial.

Roberts said researchers from Newfoundland have also pinpointed the gene responsible for a highly aggressive form of ARVC that affects large numbers of Newfoundlanders, commonly known as the ‘Newfoundland curse’.

“We are hopeful that our clinical trial will show that our treatment is effective for all genetic subtypes of ARVC, including the Newfoundland curse,” said Roberts.

“Working together with centers across the country and leveraging the expertise of the Population Health Research Institute, a world class clinical trials program based at McMaster University, we believe that we are in a great position to move forward with this clinical trial and hopefully usher in a new era in the treatment of ARVC.”

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