ARRHYTHMIA STUDIES: Contracted Research, Canadian Inherited Arrhythmia Registries

Long QT

The NLQTS Research Network team aims to build a Canadian collaboration of dedicated investigators that will create a new paradigm in the modern investigation of patients with LQTS and in the description of a new disease modifier.

The project aims to describe the natural history of familial Long QT Syndrome to identify:

- Low risk patients that do not require protective beta-blocker therapy
- High-risk patients that require protective beta-blocker therapy and may benefit from a primary prevention ICD. This cohort would contain treated pre-symptomatic individuals effectively protected from harm.



The project will evaluate a standardized testing protocol in detecting the cause of cardiac arrest and familial sudden death in patients with apparently unexplained cardiac arrest. The testing is directed at the detection of rare genetic conditions that result in palpitations, blackouts and sudden death in patients and their family members. Genetic testing will be performed to validate the clinical findings.



Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited condition that may cause life threatening irregular heart rhythms that often manifest as unexpected cardiac arrest or sudden death in early adulthood. The condition is difficult to diagnose and often is not noticed until a family member suffers a cardiac arrest or death. The Canadian National ARVC registry will collect data from Inherited Heart Rhythm Clinics across Canada.


- To determine the natural history of ARVC (short/intermediate term), including risk of symptomatic arrhythmias and sudden death, for patients with the phenotype and those gene positive patients without phenotype evidence of disease.
- To understand risk factors for sudden death/appropriate ICD use in ARVC, including test characteristics/performance and their relationship to outcomes (ECG, Holter, signal averaged ECG, loop recorders, imaging, voltage mapping, T wave alternans, cardiac biopsy and biomarkers).
- To establish a phenotype genotype correlation, including comparison of patients with disease causing mutations, variants of unknown significance (VUS) and Task Force

Criteria (TFC) positive, gene negative patients


Local PI's: C Seifer/C Khoo
Co-investigators: A Khadem, A Tischenko, K Wolfe
Local contact:
Rebecca Medeiros
(204) 237-2380