Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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COMPLETED
1529 participants
OBSERVATIONAL
2004-05-31
2020-08-30
Brief Summary
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Detailed Description
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Phenotype recognition of the range of these rare genetic conditions includes non-invasive and invasive testing to demonstrate the hallmarks of each individual condition, and exclude common causes such as ischemic or idiopathic forms of cardiomyopathy. The outcomes from this type of testing have not been assessed in a systematic fashion in patients with UCA or their family members. Phenotype-genotype correlation is necessary to develop optimal diagnostic testing in probands and screening techniques in their family members, which will result in disease-specific therapy. Genetic testing of patients with an overt phenotype demonstrates a potentially causative mutation in 50-75% of LQTS patients, and 20% of Brugada's Syndrome patients. Despite recognized mutations with phenotypic expression models, 30-80% of patients will have negative gene screening despite overt or latent clinical disease.
The proposed project is evaluating a systematic approach to clinical assessment and genetic screening of patients and families with UCA and suspected inherited arrhythmias involving:
1. A multicenter registry of UCA patients, their family members and referred patients with familial sudden death undergoing standardized testing for evidence of primary electrical disease (PED). The single center pilot experience at the applicant's institution has proven feasibility, and has been accepted for publication in Circulation, indicating novelty. Ten centers across Canada have agreed to participate. The target is to enroll 1500 UCA probands, 1st degree family members. 1st degree relatives of autopsy negative unexplained sudden death victims.
2. Long term cardiac monitoring for (3 years) in select high-risk patients with an injectable cardiac monitor to detect potential substrate and/ore triggers for sudden death.
3. DNA/plasma collection and biobanking for stratified whole exome sequencing.
Conditions
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Keywords
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Study Design
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COHORT
PROSPECTIVE
Study Groups
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Cardiac Arrest Survivors or Post Mortem Unexplained Cardiac
Probands - Unexplained Cardiac Arrest Survivors and Post Mortem Unexplained Cardiac Arrest Cases
No interventions assigned to this group
First Degree Family Members
First Degree Family Members of those affected by Sudden Unexplained Cardiac Arrest
No interventions assigned to this group
Eligibility Criteria
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Inclusion Criteria
* Syncope with documented polymorphic ventricular tachycardia felt to be responsible for the index event.
* First degree relative of an index case of UCA undergoing clinical testing.
* First degree relative of a family member with UCA or sudden death before age 35 with a negative autopsy for cause of death, presumed arrhythmic.
* First degree relative of a family member with UCA or sudden death with objective evidence of primary electrical disease, such as a diagnostic electrocardiogram (ECG), exercise test, drug infusion, or genetic testing.
Exclusion Criteria
* Reduced left ventricular function (left ventricular ejection fraction \[LVEF\] \< 50%)
* Event managed without an implantable cardioverter defibrillator \[ICD\] (for follow-up portion)
* Unwilling or unable to provide clinical follow-up (for follow-up portion)
* Comorbidity making survival of \> 1 year unlikely
* Persistent resting QTc \> 460 msec for males and 480 msec for females
* Reversible cause of cardiac arrest such as marked hypokalemia (\< 2.8 mmol/l) or drug overdose sufficient in gravity without other cause to explain the cardiac arrest
* Hemodynamically stable sustained monomorphic ventricular tachycardia with a QRS morphology consistent with recognized forms of idiopathic ventricular tachycardia (outflow tract or apical septal)
* Brugada's sign with e2 mm ST elevation in V1 and/or V2
* Unwilling or unable to provide consent
2 Years
ALL
No
Sponsors
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Kingston Health Sciences Centre
OTHER
Quebec Heart Institute
OTHER
The Queen Elizabeth Hospital
OTHER
St. Boniface Hospital
OTHER
Unity Health Toronto
OTHER
Vancouver Island Health Authority
OTHER
Provincial Health Services Authority
OTHER
Montreal Heart Institute
OTHER
Ottawa Heart Institute Research Corporation
OTHER
University of Calgary
OTHER
University of British Columbia
OTHER
Responsible Party
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Andrew Krahn
Principal Iinvestigator
Principal Investigators
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Andrew D Krahn, MD
Role: PRINCIPAL_INVESTIGATOR
University of British Columbia
Locations
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University of British Columbia
Vancouver, British Columbia, Canada
Countries
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References
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Krahn AD, Gollob M, Yee R, Gula LJ, Skanes AC, Walker BD, Klein GJ. Diagnosis of unexplained cardiac arrest: role of adrenaline and procainamide infusion. Circulation. 2005 Oct 11;112(15):2228-34. doi: 10.1161/CIRCULATIONAHA.105.552166. Epub 2005 Oct 3.
Krahn AD, Healey JS, Chauhan V, Birnie DH, Simpson CS, Champagne J, Gardner M, Sanatani S, Exner DV, Klein GJ, Yee R, Skanes AC, Gula LJ, Gollob MH. Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER). Circulation. 2009 Jul 28;120(4):278-85. doi: 10.1161/CIRCULATIONAHA.109.853143. Epub 2009 Jul 13.
Davies B, Bartels K, Hathaway J, Xu F, Roberts JD, Tadros R, Green MS, Healey JS, Simpson CS, Sanatani S, Steinberg C, Gardner M, Angaran P, Talajic M, Hamilton R, Arbour L, Seifer C, Fournier A, Joza J, Krahn AD, Lehman A, Laksman ZWM. Variant Reinterpretation in Survivors of Cardiac Arrest With Preserved Ejection Fraction (the Cardiac Arrest Survivors With Preserved Ejection Fraction Registry) by Clinicians and Clinical Commercial Laboratories. Circ Genom Precis Med. 2021 Jun;14(3):e003235. doi: 10.1161/CIRCGEN.120.003235. Epub 2021 May 7.
Cheung CC, Davies B, Gibbs K, Laksman ZW, Krahn AD. Patch monitors for arrhythmia monitoring in patients for suspected inherited arrhythmia syndrome. J Cardiovasc Electrophysiol. 2021 Mar;32(3):856-859. doi: 10.1111/jce.14917. Epub 2021 Feb 15.
Mellor G, Laksman ZWM, Tadros R, Roberts JD, Gerull B, Simpson CS, Klein GJ, Champagne J, Talajic M, Gardner M, Steinberg C, Arbour L, Birnie DH, Angaran P, Leather R, Sanatani S, Chauhan VS, Seifer C, Healey JS, Krahn AD. Genetic Testing in the Evaluation of Unexplained Cardiac Arrest: From the CASPER (Cardiac Arrest Survivors With Preserved Ejection Fraction Registry). Circ Cardiovasc Genet. 2017 Jun;10(3):e001686. doi: 10.1161/CIRCGENETICS.116.001686.
Steinberg C, Padfield GJ, Champagne J, Sanatani S, Angaran P, Andrade JG, Roberts JD, Healey JS, Chauhan VS, Birnie DH, Janzen M, Gerull B, Klein GJ, Leather R, Simpson CS, Seifer C, Talajic M, Gardner M, Krahn AD. Cardiac Abnormalities in First-Degree Relatives of Unexplained Cardiac Arrest Victims: A Report From the Cardiac Arrest Survivors With Preserved Ejection Fraction Registry. Circ Arrhythm Electrophysiol. 2016 Sep;9(9):e004274. doi: 10.1161/CIRCEP.115.004274.
Herman AR, Cheung C, Gerull B, Simpson CS, Birnie DH, Klein GJ, Champagne J, Healey JS, Gibbs K, Talajic M, Gardner M, Bennett MT, Steinberg C, Janzen M, Gollob MH, Angaran P, Yee R, Leather R, Chakrabarti S, Sanatani S, Chauhan VS, Krahn AD. Outcome of Apparently Unexplained Cardiac Arrest: Results From Investigation and Follow-Up of the Prospective Cardiac Arrest Survivors With Preserved Ejection Fraction Registry. Circ Arrhythm Electrophysiol. 2016 Jan;9(1):e003619. doi: 10.1161/CIRCEP.115.003619.
Krahn AD, Healey JS, Chauhan VS, Birnie DH, Champagne J, Sanatani S, Ahmad K, Ballantyne E, Gerull B, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Klein GJ, Gollob MH, Simpson CS, Talajic M, Gardner M. Epinephrine infusion in the evaluation of unexplained cardiac arrest and familial sudden death: from the cardiac arrest survivors with preserved Ejection Fraction Registry. Circ Arrhythm Electrophysiol. 2012 Oct;5(5):933-40. doi: 10.1161/CIRCEP.112.973230. Epub 2012 Sep 3.
Krahn AD, Healey JS, Simpson CS, Chauhan VS, Birnie DH, Champagne J, Gardner M, Sanatani S, Chakrabarti S, Yee R, Skanes AC, Leong-Sit P, Ahmad K, Gollob MH, Klein GJ, Gula LJ, Sheldon RS. Sentinel symptoms in patients with unexplained cardiac arrest: from the cardiac arrest survivors with preserved ejection fraction registry (CASPER). J Cardiovasc Electrophysiol. 2012 Jan;23(1):60-6. doi: 10.1111/j.1540-8167.2011.02185.x. Epub 2011 Sep 28.
Derval N, Simpson CS, Birnie DH, Healey JS, Chauhan V, Champagne J, Gardner M, Sanatani S, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Ahmad K, Gollob MH, Haissaguerre M, Klein GJ, Krahn AD. Prevalence and characteristics of early repolarization in the CASPER registry: cardiac arrest survivors with preserved ejection fraction registry. J Am Coll Cardiol. 2011 Aug 9;58(7):722-8. doi: 10.1016/j.jacc.2011.04.022.
Other Identifiers
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10076
Identifier Type: OTHER
Identifier Source: secondary_id
R-04-099
Identifier Type: -
Identifier Source: org_study_id