Electromechanical Profiling of Arrhythmogenic Substrates and Triggers in the Long-QT Syndrome
Trial Snapshot
- Phase
- Not Applicable
- Enrollment
- 150
- Primary Endpoint
- Differences in regional electromechanical dispersion between LQTS patients and controls
Study Overview
Brief Summary
High-resolution, non-invasive electromechanical mapping in genotyped long-QT syndrome patients and healthy controls at baseline and during smart provocation.
Detailed Description
Using simultaneous ECG-imaging, speckle-tracking analysis and tissue-phase mapping with MRI we will assess electromechanical dispersion at rest. Regional electromechanical elasticity will be investigated during adenosine and epinephrine, isoprenaline infusions and is postulated to increase sudden cardiac death risk prediction in the individual patient.
Study Design
- Study Type
- Observational
- Observational Model
- Case Control
- Time Perspective
- Prospective
Eligibility Criteria
- Ages
- 18 Years to — (Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- Yes
Inclusion Criteria
- •LQTS group (Group 1):
- •Diagnosis of LQTS according to the ESC guidelines.
- •Genetic testing either already performed or consent to genetic testing (at least 5 major LQTS-related genes tested: KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2).
- •Control group (Group 2):
- •> Control subjects with structurally normal hearts.
Exclusion Criteria
- •Pregnancy, nursing or planning to become pregnant.
- •Known allergy or strong reaction to skin electrodes or contrast agent.
- •Inability to give informed consent.
- •Presence of metal objects in or attached to the body.
- •Dialysis.
- •Cardiomyopathy.
- •Second-degree heart block or higher degrees of block.
- •Sick sinus syndrome.
- •Chronic obstructive pulmonary disease.
- •Left-main coronary artery disease.
- •Unstable coronary artery disease.
Outcomes
Primary Outcomes
Differences in regional electromechanical dispersion between LQTS patients and controls
Time Frame: At day of investigation
Electromechanical dispersion in milliseconds
Differences in regional electromechanical dispersion between symptomatic and asymptomatic LQTS patients
Time Frame: At day of investigation
Electromechanical dispersion in milliseconds
Secondary Outcomes
- Relation between global electromechanical window vs regional electromechanical dispersion in LQTS(At day of investigation)
- Correlation of electromechanical dispersion between LQTS type 1, 2, and 3.(At day of investigation)
- Correlation between mechanical dispersion using TPM-MRI and cine-MRI(At day of investigation)
- Correlation between mechanical dispersion using TPM-MRI and speckle-tracking echocardiography(At day of investigation)