Evaluate the effect of the menstrual cycle on QTc among females with LQT1 or LQT2 and to put our previous findings in a broader perspective by including controls groups and older females.
ID
Source
Brief title
Condition
- Cardiac arrhythmias
- Cardiac and vascular disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Concomitant estradiol and pregnanediol fluctuations will be obtained together
with the QTc at three time points during the menstrual cycle or approximately
corresponding with the phases of the menstrual cycle in LQTS-females with and
without HCT, respectively.
Secondary outcome
Daily QTc fluctuations during 1 month in men and women both with and without
HCT.
Background summary
Congenital long QT syndrome (LQTS) is a potentially lethal inherited disorder
defined by prolongation of the QT-interval corrected for heart rate (QTc) on
the electrocardiogram (ECG). If untreated, polymorphic ventricular tachycardia
(Torsades de Pointes, TdP) may occur, leading to syncope or sudden death.
Children and adolescents constitute an especially important risk group. The QTc
varies with age and sex in patients with LQTS, depending on the different
genotypes. These changes are associated with differences in the clinical course
of the disease. It has been proposed that sex hormones may play an important
role in the complex relationship between genotype, age, sex and QTc. Hence,
studies have been performed to examine the influences of pregnancy and
menopause in women with LQTS. Recently, we have assessed the effect of the
menstrual cycle on the QTc in twenty LQTS type 1 (LQT1) and type 2 (LQT2)
females between the age of 12-21 years. We found that the QTc was longer in
LQT2-females during the luteal-phase and that pregnanediol levels were
associated with significant changes in heart rate and QTc during a resting
supine position. Furthermore, we found a decreased QT-interval/QTc adaptation
after standing during the ovulation-phase but not the luteal-phase, especially
in LQT2-girls. To put these findings in a broader perspective, we want to
expand our protocol as suggested earlier (METC 2014_159 and METC2015_186).
Study objective
Evaluate the effect of the menstrual cycle on QTc among females with LQT1 or
LQT2 and to put our previous findings in a broader perspective by including
controls groups and older females.
Study design
This study will be a prospective observational study.
Study burden and risks
As only extra ECG recordings and urinary samples of hormone levels are being
conducted, the risk of this study is expected to be low. The burden for LQTS
females will consist of 3 visits with ECG recordings and collection of urinary
samples with a total duration of ~30 minutes. LQTS-females without HCT will be
asked to identify ovulation using urinary ovulation predictors. Furthermore,
LQTS males and females will be requested to record an self-recorded
ECGs three times a day for the period of approximately 4 weeks. The recording
of these ECGs and the sending of them to the investigator takes up 1-2 minutes
per day. This study does not have an advantage for the participating patients.
However, all ECG recordings will be analysed by the investigator and shared
with the treating physician for use to optimize individual risk stratification
in regular care, which might have an advantage for the participants.
Furthermore, acquiring more insight in the role of the menstrual cycle may
improve risk stratification for life-threatening arrhythmias for all
LQTS-patients.
Meibergdreef 9
Amsterdam 1105AZ
NL
Meibergdreef 9
Amsterdam 1105AZ
NL
Listed location countries
Age
Inclusion criteria
Genetically confirmed LQT1 or LQT2
Age between 12 and 30 years
Exclusion criteria
Pregnancy and post-partum period until the end of breastfeeding
The use of hormonal contraceptive therapy other than combined
oestrogen-progestin oral contraceptives (i.e. intrauterine contraception,
contraceptive vaginal ring, etonogestrel contraceptive implant, injectable
contraceptives or progestin pills)
History of anorexia nervosa or polycystic ovary syndromeHistory of anorexia
nervosa or PCOS
BMI for age greater or less than the 99th percentile
Double mutation carrier or compound heterozygote
Mutations of unknown pathogenicity
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL75145.018.20 |