BC Inherited Arrhythmia Program

Click here for more information on the BC Inherited Arrhythmia Program

Description: Andrew-Krahn_articleTragedy strikes like lightning when young people collapse and cannot be saved. These tragedies are often not as random as one might think, with warning signs including stories of similar tragedies in the families of the victims. In recent years, genetic technology has drastically changed the ability to diagnose individuals at risk for premature sudden cardiac death due to a heart arrhythmia.  These advancements allow physicians to identify patients at risk much earlier and develop and implement strategies to help prevent the consequences of this devastating health tragedy within families.

A new multi-disciplinary clinic focused on inherited heart diseases opened in the Fall of 2012 with my recruitment as the Paul Brunes UBC Professor in Heart Rhythm Disorders and Professor and Head, Division of Cardiology, the Faculty of Medicine. The clinic consists of experts from cardiology, medical genetics, pediatrics, genetic counseling, and nursing, and is supported by hospital leadership, researchers, and administrative staff, led by myself at St. Paul’s and VGH Hospital.

Clinical and Research Program

I am collaborating with Dr. Laura Arbour, Professor in the Department of Medical Genetics, and Dr. Shu Sanatani, Associate Professor, in the Department of Pediatrics on a patient-focused clinical and research program to better understand the diagnosis and treatment of inherited heart rhythm diseases that predispose individuals to sudden cardiac arrest such as Long QT syndrome, Right Ventricular Cardiomyopathy, Catecholaminergic Polymorphic Ventricular Tachycardia, and Brugada syndrome, while identifying and treating patients and their families at the clinic. This innovative research program will focus on the prevention of fainting and sudden death, a preventable tragedy that snatches life from youth far too often. Thirty-thousand Canadians die suddenly every year, and a family history of sudden death increases the risk of sudden death by a factor of 2. It is estimated that 7000 British Columbians are affected by these inherited conditions that carry a lifetime risk of sudden death as high as 20%, much of which is completely preventable with simple inexpensive measures.

The major objectives of the program are to create a provincial program targeting the detection and treatment of inherited heart rhythm diseases, educate patients and health care professionals about the prevention, diagnosis, and treatment of these conditions, and establish effective response systems to assess and identify warning signs before tragedy occurs. I also lead a research project to explore the entire genetic code of sudden death victims and their families, and to characterize new mutations that are detected. By looking at DNA stored by the coroner and from individuals who have had a life threatening arrhythmia, we aim to discover new genes that may put living family members at risk, leading to more effective and targeted prevention and treatment.

Successes to Date

This provincial cardiogenetics clinical and research team has received funding from the Specialty Service Commission (SSC) for $498,832 for 3 years to create and support a program (BC Inherited Arrhythmia Program, BCIAP) that is delivering state of the art care to patients and their families to identify risk and prevent sudden death. The Program officially began in March 2013, and sees 400 patients each year at risk for sudden death related to these conditions. Five-hundred of these patients have been enrolled in a research registry, with many of these patients also participating in national registry studies hosted at UBC. The most common reason for referral is for family evaluation after a tragic sudden death, and the most common diagnosis is Long QT syndrome, an inherited cause of sudden death that is virtually cured by a single daily pill that costs cents a day (beta blocker). The research team lead by myself has funding to conduct research from the Heart and Stroke Foundation, CIHR and the CANNeCTIN network. The Program also hosts 3 National registries in sudden death and unexplained cardiac arrest (CASPER), Long QT Syndrome (LQTS) and Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

Currently the clinic is transitioning from every other week to every week, with recruitment of an expert clinician in combination with the existing team lead by Dr. Krahn. The “genius” of the clinic is cradled in the multidisciplinary nature of the team that brings expertise from several dimensions, but the incremental step is actually the communication tools that provide patient and caregivers tools to avoid harm. We communicate with family members (standard family letter) and create screening standards of care that are taught to satellite team members in Prince George, Terrace, Kelowna and at the Royal Columbian Hospitals. Simultaneously, research staff paid through peer reviewed funding enroll patients in multiple studies that include local, national and international collaboration. The BCIAP program has ethics approval for database formation, with 505 enrolled patients. The impending access to the entire genetic sequence of patients with attendant immense patient specific datasets is highly anticipated. The BCIAP is seeking sustainable funding to create an efficient, electronic management system that will link with research in a single managed “Big Data” strategy that has the potential to be transformative both locally, and as a leading site to roll out nationally.