Evaluate the effect of menarche and menstrual cycle on the QTc interval among girls with genetically-confirmed mutations causing LQTS type 1 (LQT1) and type 2 (LQT2).
ID
Source
Brief title
Condition
- Cardiac and vascular disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The QTc interval will be measured pre- and post-menarche and during 3 phases of
the menstrual cycle. In boys QTc interval will be measured at approximately
the same time intervals as the girls during their menstrual cycle.
Secondary outcome
Not applicable
Background summary
The congenital long QT syndrome (LQTS) is an inherited disorder defined by the
prolongation of the QT interval on electrocardiogram (ECG). If untreated,
polymorphic ventricular tachycardia (torsade des pointes) may occur, leading to
syncope or even sudden death. Normal cardiac repolarization adapts to heart
rate, so a standardized heart rate correction formula is used to correct the QT
interval for heart rate (QTc) allowing the comparison of the QT intervals at
different heart rates. The QTc interval varies with age and gender in patients
with LQTS, depending on the differ genotypes. These changes are associated with
differences in the clinical course of the disease. It hadsbeen proposed that
sex hormones may play a role in the complex relationship between genotype, age,
gender and QTc interval.
Hence, studies have been performed to examine the influences of pregnancy and
menopause in women with LQTS. Currently, however, there is no data regarding
the influence of menarche and menstruation cycle in these patients.
Study objective
Evaluate the effect of menarche and menstrual cycle on the QTc interval among
girls with genetically-confirmed mutations causing LQTS type 1 (LQT1) and type
2 (LQT2).
Study design
The study consists of both a retrospective- and a prospective observational
design.
Study burden and risks
In all patients three ECGs will be obtained. Girls not using oral contraceptive
therapy will be asked to use a urinary ovulation predictor and to measure daily
basal body temperatureto identify ovulation. They will document this daily in a
study dairy This study does not provide an advantage for the participating
patient. However, acquiring more information about hormonal influences on QTc
interval will give more insight into the clinical course of LQTS and may be
helpful in risk-stratification and the further management of these same
patients in the future.
Meibergdreef 9
Amsterdam 1100 DD
NL
Meibergdreef 9
Amsterdam 1100 DD
NL
Listed location countries
Age
Inclusion criteria
All girls and boys born between January 1st 1985 and December 31st 2001, who are diagnosed based on their genotype with LQT1 or LQT2 and seen in the Academic Medical Centre in Amsterdam between January 1st 2003 and December 31st 2013, will be included in the study population.
Exclusion criteria
- Premenarcheal
- Pregnancy
- Use of hormonal contraceptive therapy other than combined estrogen-progestin oral contraceptives (i.e. intrauterine contraception, contraceptive vaginal ring, etonogestrel contraceptive implant, injectable contraceptives or progestin pills)
- Double mutation carriers or compound heterozygote
- Body mass index (BMI) for age greater than the 95th percentile
- History of polycystic ovary syndrome (PCOS)
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 | NL49238.018.14 |