Electromechanical profiling of arrhythmogenic substrates and triggers in the long-QT syndrome

Recruiting

Conditions: long-QT syndrome
Romano-Ward syndrome
10007521
10007510

Registration Number: NL-OMON54669

Lead Sponsor: Medisch Universitair Ziekenhuis Maastricht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 85

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 (LVEF < 50%).
* 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.
* Moderate to severe valvular disease.
* Inability to undergo MRI scan (control population).

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Cardiac event risk prediction in LQTS patients using regional electromechanical<br /><br>dispersion.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Secondary study points consist of:<br /><br>• difference in electromechanical dispersion (in ms) between symptomatic and<br /><br>asymptomatic genotyped LQTS patients and controls.<br /><br>• comparison of electromechanical dispersion (in ms) between LQTS type 1, 2,<br /><br>and 3.<br /><br>• relation between global electromechanical window vs regional<br /><br>electromechanical dispersion in LQTS.<br /><br>• Comparison of mechanical dispersion using TPM-MRI and cine-MRI.<br /><br>• Comparison of mechanical dispersion using TPM-MRI and speckle-tracking<br /><br>echocardiography.</p><br>