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The aim of the study is to evaluate the best stress exam to unmask long QT in patient with KCNQ1 or KCNH2 mutation without long QT interval in rest electrocardiogram.
65 patients with KCNQ1 or KCNH2 mutation presenting QTc interval <470msec and 65 patients without KCNQ1 or KCNH2 mutation presenting QTc interval <470msec will be included.
All patients will have the 2 tests: exercise test and epinephrine test, in one half day.
At the end of the inclusions, two experts will examine examens results without knowing in which arm (mutated or not) the patient belongs.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients with KCNQ1 or KCNH2 mutation | Experimental |
| |
| patients WITHOUT KCNQ1 or KCNH2 mutation (control group) | Other |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| exercise test | Other | Exercise test : Bruce protocol : exercise during 7 minutes, and rest during 6 minutes with registering of the electrocardiograms. |
|
| Measure | Description | Time Frame |
|---|---|---|
| to evaluate the best stress exam (exercise test OR epinephrine test)to unmask long QT syndrome. | Exercise test and epinephrine test will be done at inclusion (day 1). The primary outcome of this study is to evaluate the best stress exam (exercise test versus epinephrine test), defined by a "positive" test to unmask long QT syndrome. Test is positive when QT interval lengthens 30 ms At the end of study inclusions, two reviewers will analyse, independently, electrocardiograms resulting from these tests to determine for each patient if a test was positive and which one. These reviewers will do this job without being informed of the patient status (mutated patient or not mutated patient (control)). | day 1 |
| Measure | Description | Time Frame |
|---|---|---|
| to evaluate each test characteristics | Exercise test and epinephrine test will be done at inclusion (day 1). The secondary outcome is to evaluate each test (exercise test and epinephrine test) characteristics. At the end of study inclusions, two reviewers will independently analyse electrocardiograms resulting from these tests to evaluate their characteristics. | Day 1 |
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Inclusion Criteria:
Patients with QTc<470 msec, and with molecular analysis (KCNQ1 or KCNH2 genes) performed in order to include 65 patients with KCNQ1 or KCNH2 mutation and 65 patients without KCNQ1 or KCNH2 mutation (controls).
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Vincent PROBST, PU-PH | Contact | +33 2 40 16 57 00 | vincent.probst@chu-nantes.fr | |
| Eric DELANNOY, PH | Contact |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Bordeaux Universitary Hospital | Recruiting | Bordeaux | 33604 | France |
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| ID | Term |
|---|---|
| D029597 | Romano-Ward Syndrome |
| D008133 | Long QT Syndrome |
| ID | Term |
|---|---|
| D001145 | Arrhythmias, Cardiac |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D000075224 | Cardiac Conduction System Disease |
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| ID | Term |
|---|---|
| D005080 | Exercise Test |
| ID | Term |
|---|---|
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| Epinephrine test | Other | Epinephrine test : 1 hour after exercise test : epinephrine infusion during 20 minutes, and monitoring during 30 minutes after stop infusion with registering of electrocardiograms. |
|
| Marseille Universitary Hospital | Recruiting | Marseille | 13385 | France |
|
| CHU Montpellier | Recruiting | Montpellier | 34295 | France |
|
| Nantes Universitary Hospital | Recruiting | Nantes | 44093 | France |
|
| Rennes Universitary Hospital | Recruiting | Rennes | 35033 | France |
|
| CHU Rouen | Recruiting | Rouen | 76031 | France |
|
| CHU Toulouse | Recruiting | Toulouse | 31059 | France |
|
| CHU Tours | Recruiting | Tours | 37170 | France |
|
| 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 |
| D012129 | Respiratory Function Tests |
| D003948 | Diagnostic Techniques, Respiratory System |
| D016552 | Ergometry |
| D008919 | Investigative Techniques |