Electromechanical Profiling of the Long-QT Syndrome (LQTS)

Electromechanical Profiling of Arrhythmogenic Substrates and Triggers in the Long-QT Syndrome

High-resolution, non-invasive electromechanical mapping in genotyped long-QT syndrome patients and healthy controls at baseline and during smart provocation.

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.

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.