THE BC INHERITED ARRHYTHMIA PROGRAM: A MULTIDISCIPLINARY APPROACH TO FAMILIES AT RISK FOR SUDDEN DEATH

INTRODUCTION: Inherited arrhythmia (IA) disorders predispose to syncope, sudden cardiac arrest (SCA) and sudden death. Establishing a diagnosis is critical to implementing treatment and enabling cascade screening of at risk family members. An expert, multidisciplinary approach to this patient population is a guideline-based recommendation. The BC Inherited Arrhythmia Program (BCIAP) combines the expertise of cardiology and medical genetics in a provincial, multi-site, family-centered program targeting at risk patients and family members. Launched in April 2013; 400 referrals were expected in the first year, with 38 multidisciplinary clinics planned. METHODS: The BCIAP assesses patients and first-degree relatives for IA, SCA, and history of familial sudden death. An adult electrophysiologist (EP), genetic counsellor and research nurse attend patient evaluations. Families with children under 18 attend clinics that also include a pediatric EP and nurse. Medical Geneticists provide indirect patient care and case review. Patients undergo clinical assessment, investigations, and genetic counselling. Consent to contact for research is sought for all; the majority of patients in the Vancouver clinic are approached for voluntary research registry participation and bio banking. RESULTS: In the BCIAP’s first year, 576 referrals were received. The most common indication was Long QT syndrome (23%), followed by a family history of sudden death (14%). In total, 209 (46%) of referrals were for a family history indication, and 61 (67% excluding outreach) of the families referred to the program stemmed from cascade screening. Thirty-six multidisciplinary clinics were held at the program’s two main sites, (Vancouver and Victoria); a pediatric EP attended 39%. Additionally, 8 intake and 9 outreach clinics were held in Vancouver and Northern BC respectively. At the program’s two main sites, 398 patients were seen during 343 patient visits; 142 (41%) were family visits. Of families seen, 59% underwent assessment by both adult and pediatric EPs. In patients with available data, 113/398 (28%) underwent genetic testing. Predictive familial mutation testing results are available for 37 patients; 46% tested negative and were discharged. In Vancouver, 317 patients were invited to enrol in research. Of these, 294 provided consent (93%) and are in one or more registry. CONCLUSION: The BCIAP surpassed its target referral numbers and clinics by 44%, emphasizing the need for the service in BC. The number of referrals received for a family history indication and families referred suggests that the BCIAP supports a multidisciplinary family based approach. The potential of research is accepted broadly by this patient population.