Registry of Unexplained Cardiac Arrest

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details. Identifier: NCT00292032
Recruitment Status : Recruiting
First Posted : February 15, 2006
Last Update Posted : May 2, 2018
Heart and Stroke Foundation of Ontario
Information provided by (Responsible Party):
Andrew Krahn, University of British Columbia

Brief Summary:
The CASPER will collect systematic clinical assessments of patients and families within the multicenter Canadian Inherited Heart Rhythm Research Network. Unexplained Cardiac Arrest patients and family members will undergo standardized testing for evidence of primary electrical disease and latent cardiomyopathy along with clinical genetics screening of affected individuals based on an evident or unmasked phenotype.

Condition or disease
Cardiac Arrest Long QT Syndrome Brugada Syndrome Catecholaminergi Polymorphic Ventricular Tachycardia Idiopathic VentricularFibrillation Early Repolarization Syndrome Arrhythmogenic Right Ventricular Cardiomyopathy

Detailed Description:

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:

  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.

Study Type : Observational
Estimated Enrollment : 2500 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER)
Study Start Date : May 2004
Estimated Primary Completion Date : January 2025
Estimated Study Completion Date : January 2027

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

Primary Outcome Measures :
  1. 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

Information from the National Library of Medicine

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Ages Eligible for Study:   2 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Unexplained cardiac arrest patients, and first degree relatives of 1) UCA patients 2) patients with known primary electrical disease or 3) unexplained sudden death before age 60

Inclusion Criteria:

  • Cardiac arrest requiring cardioversion or defibrillation.
  • 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:

  • Coronary artery disease (stenosis > 50%)
  • 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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00292032

Contact: Andrew D Krahn, MD 604-682-2344 ext 63260
Contact: Karen A Gibbs, RN 604-682-2344 ext 63260

Canada, British Columbia
University of British Columbia Recruiting
Vancouver, British Columbia, Canada, V6E 1M7
Contact: Andrew D Krahn, MD    604-682-2344 ext 63260   
Contact: Karen A Gibbs, RN    604-682-2344 ext 63260   
Principal Investigator: Andrew D Krahn, MD         
Sponsors and Collaborators
University of British Columbia
Heart and Stroke Foundation of Ontario
Principal Investigator: Andrew D Krahn, MD University of British Columbia

Publications of Results:
Publications automatically indexed to this study by Identifier (NCT Number):

Responsible Party: Andrew Krahn, principal investigator, University of British Columbia Identifier: NCT00292032     History of Changes
Other Study ID Numbers: R-04-099
10076 ( Other Identifier: REB )
First Posted: February 15, 2006    Key Record Dates
Last Update Posted: May 2, 2018
Last Verified: April 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

Keywords provided by Andrew Krahn, University of British Columbia:
cardiac arrest

Additional relevant MeSH terms:
Long QT Syndrome
Heart Arrest
Tachycardia, Ventricular
Brugada Syndrome
Arrhythmogenic Right Ventricular Dysplasia
Ventricular Fibrillation
Pathologic Processes
Heart Diseases
Cardiovascular Diseases
Arrhythmias, Cardiac
Heart Defects, Congenital
Cardiovascular Abnormalities
Congenital Abnormalities
Genetic Diseases, Inborn