Electromechanical Profiling of the Long-QT Syndrome (LQTS)

NCT04074122 | Unknown DMC

• 1 other identifier: 70856
• Study Type: observational
• Target: 150
• Locations: 0 countries
• Sites: N/A

Brief Summary

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

Conditions

Long QT Syndrome; Sudden Cardiac Death; Ventricular Tachycardia

Keywords

Electromechanical Mapping; Risk Stratification

Outcome Measures

Primary Outcomes (2)

• Differences in regional electromechanical dispersion between LQTS patients and controls

  Electromechanical dispersion in milliseconds

  At day of investigation

• Differences in regional electromechanical dispersion between symptomatic and asymptomatic LQTS patients

  Electromechanical dispersion in milliseconds

  At day of investigation

Secondary Outcomes (4)

• Correlation of electromechanical dispersion between LQTS type 1, 2, and 3.

  At day of investigation

• Relation between global electromechanical window vs regional electromechanical dispersion in LQTS

  At day of investigation

• Correlation between mechanical dispersion using TPM-MRI and cine-MRI

  At day of investigation

• Correlation between mechanical dispersion using TPM-MRI and speckle-tracking echocardiography

  At day of investigation

Study Arms (3)

Symptomatic LQTS patients

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

Diagnostic Test: Adenosine and epinephrine, isoprenaline provocation

Asymptomatic LQTS patients

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

Diagnostic Test: Adenosine and epinephrine, isoprenaline provocation

Healthy controls

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

Diagnostic Test: Adenosine and epinephrine, isoprenaline provocation

Interventions

Adenosine and epinephrine, isoprenaline provocation DIAGNOSTIC_TEST

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

Also known as: ECG-imaging, Tissue-phase mapping with MRI.

Asymptomatic LQTS patients; Healthy controls; Symptomatic LQTS patients

Eligibility Criteria

• Age: 18 Years+
• Gender Eligibility Details: Based on self-representation.
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Potential study subjects will be selected from the Cardiogenetic or Cardiac Outpatient Clinic or during their in-hospital stay.

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• Maastricht University Medical Center: lead
• University of Freiburg: collaborator
• University of Bern: collaborator

Related Publications (6)

• Haugaa KH, Edvardsen T, Leren TP, Gran JM, Smiseth OA, Amlie JP. Left ventricular mechanical dispersion by tissue Doppler imaging: a novel approach for identifying high-risk individuals with long QT syndrome. Eur Heart J. 2009 Feb;30(3):330-7. doi: 10.1093/eurheartj/ehn466. Epub 2008 Oct 21.

  PMID: 18940888 RESULT

• Haugaa KH, Amlie JP, Berge KE, Leren TP, Smiseth OA, Edvardsen T. Transmural differences in myocardial contraction in long-QT syndrome: mechanical consequences of ion channel dysfunction. Circulation. 2010 Oct 5;122(14):1355-63. doi: 10.1161/CIRCULATIONAHA.110.960377. Epub 2010 Sep 20.

  PMID: 20855658 RESULT

• ter Bekke RM, Volders PG. Arrhythmogenic mechano-electric heterogeneity in the long-QT syndrome. Prog Biophys Mol Biol. 2012 Oct-Nov;110(2-3):347-58. doi: 10.1016/j.pbiomolbio.2012.07.007. Epub 2012 Jul 24.

  PMID: 22841828 RESULT

• ter Bekke RM, Haugaa KH, van den Wijngaard A, Bos JM, Ackerman MJ, Edvardsen T, Volders PG. Electromechanical window negativity in genotyped long-QT syndrome patients: relation to arrhythmia risk. Eur Heart J. 2015 Jan 14;36(3):179-86. doi: 10.1093/eurheartj/ehu370. Epub 2014 Sep 8.

  PMID: 25205533 RESULT

• Shimizu W, Antzelevitch C. Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome. J Am Coll Cardiol. 2000 Mar 1;35(3):778-86. doi: 10.1016/s0735-1097(99)00582-3.

  PMID: 10716483 RESULT

• Viskin S, Rosso R, Rogowski O, Belhassen B, Levitas A, Wagshal A, Katz A, Fourey D, Zeltser D, Oliva A, Pollevick GD, Antzelevitch C, Rozovski U. Provocation of sudden heart rate oscillation with adenosine exposes abnormal QT responses in patients with long QT syndrome: a bedside test for diagnosing long QT syndrome. Eur Heart J. 2006 Feb;27(4):469-75. doi: 10.1093/eurheartj/ehi460. Epub 2005 Aug 16.

  PMID: 16105845 RESULT

MeSH Terms

Conditions

Long QT Syndrome; Death, Sudden, Cardiac; Tachycardia, Ventricular

Interventions

Magnetic Resonance Imaging

Condition Hierarchy (Ancestors)

Arrhythmias, Cardiac; Heart Diseases; Cardiovascular Diseases; Cardiac Conduction System Disease; Heart Defects, Congenital; Cardiovascular Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Heart Arrest; Death, Sudden; Death; Tachycardia

Intervention Hierarchy (Ancestors)

Tomography; Diagnostic Imaging; Diagnostic Techniques and Procedures; Diagnosis

Study Officials

• Paul Volders, MD, PhD

  Maastricht UMC+

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Rachel ter Bekke, MD, PhD

CONTACT

+31433877095; rachel.ter.bekke@mumc.nl

Paul Volders, MD, PhD

CONTACT

+31433877093; p.volders@maastrichtuniversity.nl

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 5, 2019
• First Posted: August 29, 2019
• Study Start: January 1, 2020
• Primary Completion: January 1, 2022
• Study Completion: January 1, 2022
• Last Updated: August 29, 2019

Record last verified: 2019-08