long-QT syndrome Romano-Ward syndrome long-QT syndrome , LQTS
Conditions
Interventions
Pharmacological (adenosine, epinephrine) provocation, ECG-imaging and
tissue-phase mapping using magnetic resonance imaging (TPM-MRI).
Sponsors
Maastricht Universitair Medisch Centrum +
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
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
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 (LVEF
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Cardiac event risk prediction in LQTS patients using regional electromechanical dispersion. | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary study points consist of: • difference in electromechanical dispersion (in ms) between symptomatic and asymptomatic genotyped LQTS patients and controls. • comparison of electromechanical dispersion (in ms) between LQTS type 1, 2, and 3. • relation between global electromechanical window vs regional electromechanical dispersion in LQTS. • Comparison of mechanical dispersion using TPM-MRI and cine-MRI. • Comparison of mechanical dispersion using TPM-MRI and speckle-tracking echocardiography. | — |
Countries
Netherlands, Switzerland
Contacts
Public ContactRMA Bekke
Maastricht Universitair Medisch Centrum +
Outcome results
None listed