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

NCT01271816 | Terminated DMC

• 2 other identifiers: R-10-53217390
• Study Type: observational
• Target: 100
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Keywords

genetics; sudden death; monitoring; loop recorder; cardiomyopathy

Outcome Measures

Primary Outcomes (1)

• Detection of ≥8 beats of wide QRS complex tachycardia considered ventricular tachycardia (HR>120 bpm*) by the ILR

  \* an algorithm to determine the ventricular tachycardia detection rate

  3 year monitoring follow-up

Secondary Outcomes (6)

• Comparison of ventricular arrhythmia burden between routine

  3 year monitoring follow-up

• Change in 2010 Task Force Criteria Score from enrollment to 3-year follow-up.

  3 year monitoring follow-up

• Detection of ≥30 seconds of wide QRS complex tachycardia considered ventricular tachycardia (HR>120 bpm*).

  3 year monitoring follow-up

• Proportion of patients that go on to receive an ICD

  3 year monitoring follow-up

• Proportion of patients that develop symptomatic sustained ventricular tachycardia

  3 year monitoring follow-up

Study Arms (1)

Presymptomatic ARVC gene carriers

ARVC gene positive patients without manifest ARVC after standard screening clinical testing.

Eligibility Criteria

• Age: 2 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

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

You may qualify if:

• Identification of a pathogenic mutation† categorized as associated or probably associated with ARVC
• 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.
• \< 200 PVCs / hour on screening Holter monitor
• Age \> 2 years

You may not qualify if:

• Implantable device in place (pacemaker, ICD)
• Age \< 2 years
• Mutation represents a variant of unknown significance with reasonable probability that it may not be disease causing
• Non-sustained ventricular tachycardia on screening Holter monitor (≥8 beats \> 100 bpm) and/or ≥ 200 PVCs / hour
• Previous syncope or palpitations attributed to ARVC by the site investigator
• 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.

Sponsors & Collaborators

• London Health Sciences Centre Research Institute OR Lawson Research Institute of St. Joseph's: lead
• Abbott Medical Devices: collaborator

Study Sites (1)

University of Western Ontario

London, Ontario, N6A 5A5, Canada

Location

MeSH Terms

Conditions

Arrhythmogenic Right Ventricular Dysplasia; Death, Sudden; Cardiomyopathies

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Death; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Andrew Krahn, MD FRCPC

  Western University, Canada

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: principal investigator

Study Record Dates

• First Submitted: January 4, 2011
• First Posted: January 7, 2011
• Study Start: December 1, 2010
• Primary Completion: October 1, 2013
• Study Completion: October 1, 2013
• Last Updated: January 3, 2014

Record last verified: 2014-01

Locations

CA (1)