Long QT Syndrome Type 1 or 2
Conditions
Keywords
Long QT syndrome, stress tests
Brief summary
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.
Detailed description
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.
Interventions
OTHEREpinephrine test
Epinephrine test : 1 hour after exercise test : epinephrine infusion during 20 minutes, and monitoring during 30 minutes after stop infusion with registering of electrocardiograms.
OTHERexercise test
Exercise test : Bruce protocol : exercise during 7 minutes, and rest during 6 minutes with registering of the electrocardiograms.
Sponsors
Nantes University Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
15 Years to No maximum
Healthy volunteers
Yes
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
* QTc interval \>470msec * Treatment interfering with cardiac repolarisation * Under 15 years old * Pregnant women * Contraindication to exercise or epinephrine tests * Patients without social coverage
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| to evaluate the best stress exam (exercise test OR epinephrine test)to unmask long QT syndrome. | day 1 | 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)). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| to evaluate each test characteristics | Day 1 | 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. |
Countries
France
Contacts
Primary ContactVincent PROBST, PU-PH
Backup ContactEric DELANNOY, PH
Outcome results
None listed