Electromechanical Profiling of the Long-QT Syndrome (LQTS)

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

150 participants

Study Classification

OBSERVATIONAL

Study Start Date

2020-01-01

Study Completion Date

2022-01-01

Brief Summary

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

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Detailed Description

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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.

Conditions

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Long QT Syndrome Sudden Cardiac Death Ventricular Tachycardia

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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Symptomatic LQTS patients

Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI).

Adenosine and epinephrine, isoprenaline provocation

Intervention Type DIAGNOSTIC_TEST

High-resolution electromechanical mapping at baseline and after provocative measures.

Asymptomatic LQTS patients

Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI).

Adenosine and epinephrine, isoprenaline provocation

Intervention Type DIAGNOSTIC_TEST

High-resolution electromechanical mapping at baseline and after provocative measures.

Healthy controls

Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI).

Adenosine and epinephrine, isoprenaline provocation

Intervention Type DIAGNOSTIC_TEST

High-resolution electromechanical mapping at baseline and after provocative measures.

Interventions

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Adenosine and epinephrine, isoprenaline provocation

High-resolution electromechanical mapping at baseline and after provocative measures.

Intervention Type DIAGNOSTIC_TEST

Other Intervention Names

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ECG-imaging Tissue-phase mapping with MRI.

Eligibility Criteria

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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.

Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes