Long QT Syndrome, Sudden Cardiac Death, Ventricular Tachycardia
Conditions
Keywords
Electromechanical Mapping, Risk Stratification
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.
Interventions
DIAGNOSTIC_TESTAdenosine and epinephrine, isoprenaline provocation
High-resolution electromechanical mapping at baseline and after provocative measures.
Sponsors
University of Freiburg
University of Bern
Maastricht University Medical Center
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
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. * Asthma. * Chronic obstructive pulmonary disease. * Left-main coronary artery disease. * Unstable coronary artery disease.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Differences in regional electromechanical dispersion between LQTS patients and controls | At day of investigation | Electromechanical dispersion in milliseconds |
| Differences in regional electromechanical dispersion between symptomatic and asymptomatic LQTS patients | At day of investigation | Electromechanical dispersion in milliseconds |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Correlation of electromechanical dispersion between LQTS type 1, 2, and 3. | At day of investigation | Electromechanical dispersion in milliseconds |
| Relation between global electromechanical window vs regional electromechanical dispersion in LQTS | At day of investigation | Electromechanical dispersion in milliseconds |
| Correlation between mechanical dispersion using TPM-MRI and cine-MRI | At day of investigation | Time-to-diastolic peak in milliseconds |
| Correlation between mechanical dispersion using TPM-MRI and speckle-tracking echocardiography | At day of investigation | Time-to-peak in milliseconds |
Contacts
Primary ContactRachel ter Bekke, MD, PhD
Backup ContactPaul Volders, MD, PhD
Outcome results
None listed