Extended monitoring for cardiac arrhythmias in Dravet syndrome

Mary Connolly, MB, BCh, FRCPC(C), FRCP(I), FRCP(Edin) – The University of British Columbia
Shubhayan Sanatani, MD, FRCPC – The Universty of British Columbia
Extended monitoring for cardiac arrhythmias in Dravet syndrome
Clinical Research Grant – $250,000, 2 years

Grant Summary:

Dravet syndrome (DS) is a rare serious condition associated with early onset of seizures that typically do not respond to anti-seizure medications. Development and learning are affected in most patients. Individuals with DS have a higher chance of dying compared to those with other forms of seizures, and one common cause of this is sudden unexpected death in epilepsy (SUDEP) which occurs in 15-20% of individuals and is a great source of worry for all who care for individuals with DS. It is not fully understood how SUDEP occurs in DS, but seizures can cause rapid or very slow heart rates or rarely the heart may stop beating for a period of time. In a mouse model of DS, seizures affected the part of the brain that controls the heart and breathing and this suggests that abnormal heart rhythms triggered by seizures could be a major factor in causing SUDEP. In this study, we will place a heart monitor under the skin in 20 individuals with DS to measure their heart rhythms during seizures over a period of two years or longer. We will analyze information obtained from these monitors and seizures diaries to identify any serious abnormal heart rhythms that occur during seizures. This study aims to help us better understand how seizures affect the heart and if they can lead to life threatening heart rhythms. This knowledge could help lead to ways to prevent SUDEP in people with DS.

About the Investigators:

Dr. Mary Connolly is a Clinical Professor of Pediatrics (Neurology) at The University of British Columbia (UBC), Director of The Comprehensive Epilepsy Program and a Clinician Investigator at BC Children’s Hospital Research Institute. She completed Medical School at Trinity College Dublin and trained in internal medicine and pediatrics in Ireland. She completed residency in pediatric neurology at UBC and epilepsy fellowship at The Children’s Hospital Boston. She joined the faculty at UBC in 1993 and was head of the Division of Pediatric Neurology from 2004-2023. Her clinical and research interests include epilepsy surgery in children, tuberous sclerosis complex and Dravet syndrome and she is site PI for many studies of novel therapies in epilepsy.

She is President of the Canadian League against Epilepsy (CLAE), Past President of the Canadian Association of Child Neurologists (2009-2011) and past Co-Chair of the Canadian Pediatric Epilepsy Network (2013-2017). She received the Clinical Practice and Advocacy Award from the CLAE in 2015 and the Academic Leadership Clinician Practitioner Award from the Pediatric Chairs in Canada in 2021.

Dr. Sanatani
obtained his MD from UBC in 1993, completed a Pediatric Residency at Queen’s University and a Pediatric Cardiology fellowship at UBC with advanced training in cardiac electrophysiology at Toronto’s Hospital for Sick Children and UBC. Dr. Sanatani joined the faculty as the Director of Cardiac Pacing and Electrophysiology at BC Children’s Hospital in 2001. He was promoted to Associate Professor in 2008 and full Professor of Pediatrics in 2017. Dr. Sanatani assumed the role of Division Head and Medical Director of the Children’s Heart Centre in 2015, and is the Section Head of the Heart Rhythm Service. Dr. Sanatani’s academic focus includes sudden unexpected death in the young, as well as supraventricular arrhythmias in the pediatric population. He served as the VP of Research for the Pediatric and Congenital Electrophysiology Society (PACES) prior to becoming President in 2016.

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