Canadian Genetic Heart Rhythm Network

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CGHR Network Studies

CASPER: Cardiac Arrest Survivors with Preserved Ejection Fraction Registry

CASPER in the news: Article in London Free Press July 28, 2009

A young woman was attending her first day of University in London, Ontario when she experienced a cardiac arrest – a serious medical emergency in which a person stops breathing and his or her heart stops pumping blood, affecting approximately 40,000 Canadians each year.  Thanks to speedy emergency care, including CPR performed by a bystander, the young woman’s life was saved. Although 70 per cent of cardiac arrests occur in homes and public places, such as a school or the sports field, less than five per cent of people who experience cardiac arrest outside of a hospital survive.

Today this young woman is healthy and thriving, and her participation along with her family in a unique Canada-wide program based at London Health Sciences Centre (LHSC).

Paper that outlines the CASPER study, and reports the outcome of the first 63 patients with unexplained cardiac arrest.

CASPER: an overview

CASPER is a national study that, through a standardized testing protocol, aims to detect rare genetic conditions in children and adults with an unexplained cardiac arrest, as well as their family members.  Such conditions, which can go undetected, can lead to cardiac arrest and sudden death.

“CASPER allows us to better understand what causes people to have a cardiac arrest, and to identify the cause within families and therefore prevent further cardiac arrests in family members,” said London Arrhythmia Cardiologist Dr. Andrew Krahn, a researcher investigating the genetic causes of heart rhythm problems who heads the team of Canadian physician-researchers involved in CASPER.  “In most cases, when a genetic condition is identified, it can be treated – and lives are saved.”

Specifically, accurate testing after a cardiac arrest is needed in order to detect inherited heart conditions in children and adults; identify these conditions in family members who have not experienced symptoms; direct genetic testing; and deliver effective treatment.  This process involves standardized clinical testing, such as Holter monitoring, echocardiography and MRI, as well as genetic testing, in which a tablespoon of blood is analysed for one of the 11 different genetic causes of cardiac arrest that researchers have identified.

Through her involvement in CASPER, the young woman who survived a cardiac arrest was able to have her heart abnormality identified and to begin receiving the appropriate treatment, allowing her to live a normal life.  This program aims to detect such conditions in family members as well, although they may appear to be completely healthy. The young woman’s mother, father and sister were tested – resulting in the identification and subsequent treatment of the same heart abnormality in one of her parents.

CASPER by the numbers

Approximately 160 children and adults are currently enrolled in CASPER.  Of the 160, approximately half are patients who have experienced a cardiac arrest and half are family members.  CASPER is the only study of its kind in Canada and is relatively unique in the world.  Ontarians are particularly fortunate, as our province has shown leadership in Canada where genetic testing is available to patients for some of these conditions.

In addition to LHSC, eight Canadian academic health care centres are involved in the program, including centres in British Columbia, Alberta, Ontario, Quebec and Nova Scotia.  Through CASPER, Dr. Krahn and his colleagues are establishing a significant research network across the country to study these genetic conditions in children and adults.

“Ten years ago many of the cases now involved in CASPER would have been unexplained cardiac arrests,” said Dr. Krahn.  “Now, because of CASPER, we’re able to explain and identify inherited conditions in half of cases, and detect and treat the problem in their families before they can get in harm’s way.”

The CASPER study and ongoing genetic research in arrhythmia has recently received encouraging news of ongoing funding through 2013 by the Heart and Stroke Foundation and Boston Scientific, permitting ongoing discovery of important inherited causes of arrhythmia.

CASPER is sponsored by the Heart & Stroke Foundation of Ontario, as well as Boston Scientific and generous donations by individuals and families. This also includes the development of a web site to increase awareness about CASPER among the public and potential study participants, enabling the program to reach and help even more heart patients and families.