Prolonged Monitoring to Detect Ventricular Arrhythmias in Presymptomatic Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Patients (PREPARE)

This study has been terminated.
(inadequate recruitment)
Sponsor:
Collaborator:
St. Jude Medical
Information provided by (Responsible Party):
Andrew Krahn, Lawson Health Research Institute
ClinicalTrials.gov Identifier:
NCT01271816
First received: January 4, 2011
Last updated: January 2, 2014
Last verified: January 2014
  Purpose

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited condition characterized by life threatening heart racing, presenting with palpitations, cardiac arrest (collapse requiring an ambulance) or sudden death. The disease affects the right ventricle, the part of the heart that pumps blood to the lungs. ARVC is diagnosed with a wide range of tests that focus on the pumping function and the electrical signals from the right ventricle. These factors are summarized in a score that forms the ARVC Task Force Criteria. Genetic testing has identified 5 different genes that lead to ARVC, which are detected in about 60% of patients with ARVC. This allows doctors to test family members of the patient with ARVC to determine if they are at risk of developing the condition. Currently, family members undergo testing that includes imaging and electrical tests such as a 24-hour monitor to determine if they have evidence of ARVC. With increasing frequency, family members are found to have the gene that may lead to ARVC, but little or no evidence that their hearts are affected. This may be because the family member is too young to develop the condition, or that other factors that we do not understand have protected them from developing it.

The PREPARE study will study 100 patients that carry a gene that can lead to ARVC, but do not have anything more than minor evidence that the condition is present. These patients will not have heart racing on their initial 24-hour monitor. These patients will undergo long term monitoring with an implanted heart monitor that is inserted with a minor surgical procedure, which will detect abnormal heart rhythms that may provide a clue that heart racing from ARVC is present that is not detected with a 24-hour monitor that is performed on an annual basis (St. Jude Confirm implantable loop recorder). These patients will be enrolled in 10 adult and pediatric centers across Canada, and followed for 3 years after their heart monitor is implanted. If heart racing is detected, patients will discuss these results with their doctor to discuss what it means to them.


Condition
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Official Title: Prolonged Monitoring to Detect Ventricular Arrhythmias in Presymptomatic ARVC Patients

Resource links provided by NLM:


Further study details as provided by Lawson Health Research Institute:

Primary Outcome Measures:
  • Detection of ≥8 beats of wide QRS complex tachycardia considered ventricular tachycardia (HR>120 bpm*) by the ILR [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
    * an algorithm to determine the ventricular tachycardia detection rate


Secondary Outcome Measures:
  • Comparison of ventricular arrhythmia burden between routine [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
    * an algorithm to determine the ventricular tachycardia detection rate

  • Change in 2010 Task Force Criteria Score from enrollment to 3-year follow-up. [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
  • Detection of ≥30 seconds of wide QRS complex tachycardia considered ventricular tachycardia (HR>120 bpm*). [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
  • Proportion of patients that go on to receive an ICD [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
  • Proportion of patients that develop symptomatic sustained ventricular tachycardia [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]
  • Proportion of patients that develop sustained ventricular tachycardia, cardiac arrest or sudden death [ Time Frame: 3 year monitoring follow-up ] [ Designated as safety issue: No ]

Estimated Enrollment: 100
Study Start Date: December 2010
Study Completion Date: October 2013
Primary Completion Date: October 2013 (Final data collection date for primary outcome measure)
Groups/Cohorts
Presymptomatic ARVC gene carriers
ARVC gene positive patients without manifest ARVC after standard screening clinical testing.

  Show Detailed Description

  Eligibility

Ages Eligible for Study:   2 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Probability Sample
Study Population

ARVC gene carriers with minimal or no evidence of being clinically affected

Criteria

Inclusion Criteria:

  1. Identification of a pathogenic mutation† categorized as associated or probably associated with ARVC
  2. Failure to meet definite revised Task Force Criteria for ARVC. Mutation carriers by definition have a major criterion, so included patients may have 1 minor criteria, but would meet Task Force Criteria for ARVC with 2 minor criteria or 1 major criterion.
  3. < 200 PVCs / hour on screening Holter monitor
  4. Age > 2 years

Exclusion Criteria:

  1. Implantable device in place (pacemaker, ICD)
  2. Age < 2 years
  3. Mutation represents a variant of unknown significance with reasonable probability that it may not be disease causing
  4. Non-sustained ventricular tachycardia on screening Holter monitor (≥8 beats > 100 bpm) and/or ≥ 200 PVCs / hour
  5. Previous syncope or palpitations attributed to ARVC by the site investigator
  6. Meets definite revised Task Force Criteria for ARVC (≥2 minor criteria and/or ≥1 additional major criterion). These ARVC patients who do not have an implanted device (ICD or pacemaker) will be included in a parallel voluntary registry separate from the study.
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01271816

Locations
Canada, Ontario
University of Western Ontario
London, Ontario, Canada, N6A 5A5
Sponsors and Collaborators
Lawson Health Research Institute
St. Jude Medical
Investigators
Principal Investigator: Andrew Krahn, MD FRCPC University of Western Ontario, Canada
  More Information

Additional Information:
No publications provided

Responsible Party: Andrew Krahn, principal investigator, Lawson Health Research Institute
ClinicalTrials.gov Identifier: NCT01271816     History of Changes
Other Study ID Numbers: R-10-532, 17390
Study First Received: January 4, 2011
Last Updated: January 2, 2014
Health Authority: Canada: Ethics Review Committee

Keywords provided by Lawson Health Research Institute:
genetics
sudden death
monitoring
loop recorder
cardiomyopathy

Additional relevant MeSH terms:
Arrhythmias, Cardiac
Arrhythmogenic Right Ventricular Dysplasia
Cardiomyopathies
Heart Diseases
Cardiovascular Diseases
Pathologic Processes
Heart Defects, Congenital
Cardiovascular Abnormalities
Congenital Abnormalities

ClinicalTrials.gov processed this record on July 26, 2014