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| Name | Class |
|---|---|
| University of Freiburg | OTHER |
| University of Bern | OTHER |
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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Symptomatic LQTS patients | Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI). |
| |
| Asymptomatic LQTS patients | Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI). |
| |
| Healthy controls | Pharmacological (adenosine, epinephrine, isoprenaline) provocation, ECG-imaging and tissue-phase mapping using magnetic resonance imaging (TPM-MRI). |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Adenosine and epinephrine, isoprenaline provocation | Diagnostic Test | High-resolution electromechanical mapping at baseline and after provocative measures. |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation of electromechanical dispersion between LQTS type 1, 2, and 3. | Electromechanical dispersion in milliseconds | At day of investigation |
| Relation between global electromechanical window vs regional electromechanical dispersion in LQTS |
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Inclusion Criteria:
LQTS group (Group 1):
Control group (Group 2):
> Control subjects with structurally normal hearts.
Exclusion Criteria:
Based on self-representation.
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Potential study subjects will be selected from the Cardiogenetic or Cardiac Outpatient Clinic or during their in-hospital stay.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Rachel ter Bekke, MD, PhD | Contact | +31433877095 | rachel.ter.bekke@mumc.nl | |
| Paul Volders, MD, PhD | Contact | +31433877093 | p.volders@maastrichtuniversity.nl |
| Name | Affiliation | Role |
|---|---|---|
| Paul Volders, MD, PhD | Maastricht UMC | Principal Investigator |
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 18940888 | Result | 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. | |
| 20855658 | Result |
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| ID | Term |
|---|---|
| D008133 | Long QT Syndrome |
| D016757 | Death, Sudden, Cardiac |
| D017180 | Tachycardia, Ventricular |
| ID | Term |
|---|---|
| D001145 | Arrhythmias, Cardiac |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D000075224 | Cardiac Conduction System Disease |
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| ID | Term |
|---|---|
| D008279 | Magnetic Resonance Imaging |
| ID | Term |
|---|---|
| D014054 | Tomography |
| D003952 | Diagnostic Imaging |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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|
Electromechanical dispersion in milliseconds
| At day of investigation |
| Correlation between mechanical dispersion using TPM-MRI and cine-MRI | Time-to-diastolic peak in milliseconds | At day of investigation |
| Correlation between mechanical dispersion using TPM-MRI and speckle-tracking echocardiography | Time-to-peak in milliseconds | At day of investigation |
| 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. |
| 22841828 | 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. |
| 25205533 | 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. |
| 10716483 | 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. |
| 16105845 | 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. |
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D006323 | Heart Arrest |
| D003645 | Death, Sudden |
| D003643 | Death |
| D013610 | Tachycardia |