Registry of Unexplained Cardiac Arrest
Long QT Syndrome
Catecholaminergi Polymorphic Ventricular Tachycardia
Early Repolarization Syndrome
Arrhythmogenic Right Ventricular Cardiomyopathy
|Study Design:||Observational Model: Cohort
Time Perspective: Prospective
|Official Title:||Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER)|
- Developing and Testing Algorithms for Diagnostics and Treatments in Survivors of Unexplained Cardiac Arrest [ Time Frame: 25 years ]Long Term follow up data on survivors of cardiac arrest Long term monitoring of high risk patients and familymembers with an Injectable Cardiac Monitor 24 hour holter monitoring during provocative testing with epinephrine infusion and ambulatory activities to detect subclinical repolarization
Biospecimen Retention: Samples With DNA
|Study Start Date:||May 2004|
|Estimated Study Completion Date:||January 2020|
|Estimated Primary Completion Date:||January 2019 (Final data collection date for primary outcome measure)|
Cardiac Arrest Survivors or Post Mortem Unexplained Cardiac
Probands - Unexplained Cardiac Arrest Survivors and Post Mortem Unexplained Cardiac Arrest Cases
First Degree Family Members
First Degree Family Members of those affected by Sudden Unexplained Cardiac Arrest
Arrhythmias caused by congenital or acquired abnormalities of cardiac K+ or Na+ channels are increasingly recognized as a cause of syncope and sudden death. Cardiac arrest in the absence of overt structural heart disease was previously considered idiopathic ventricular fibrillation (IVF). The list of causes of "unexplained" cardiac arrest (UCA) now encompasses K+ related abnormalities (Long and Short QT, Andersen's), Na+ related (Long QT3, Brugada), Ca++ related (Catecholaminergic Polymorphic Ventricular Tachycardia-CPVT), and latent cardiomyopathy. These underlying causes of cardiac arrest are overtly familial in 30-60% of cases. Clinical detection of the underlying phenotype is crucial to direct appropriate treatment, genetic testing and screening of family members.
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:
- 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.
- 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.
- DNA/plasma collection and biobanking for stratified whole exome sequencing.
Please refer to this study by its ClinicalTrials.gov identifier: NCT00292032
|Contact: Andrew D Krahn, MD||604-682-2344 ext firstname.lastname@example.org|
|Contact: Karen A Gibbs, RN||604-682-2344 ext email@example.com|
|Canada, British Columbia|
|University of British Columbia||Recruiting|
|Vancovuer, British Columbia, Canada, V6E 1M7|
|Contact: Andrew D Krahn, MD 604-682-2344 ext 63260 firstname.lastname@example.org|
|Contact: Karen A Gibbs, RN 604-682-2344 ext 63260 email@example.com|
|Principal Investigator: Andrew D Krahn, MD|
|Principal Investigator:||Andrew D Krahn, MD||University of British Columbia|