View clinical trials related to Long QT Syndrome.
Filter by:In patients expressing the SCN5A-E1784K mutation (Glu1784Lys), cardiovascular risk is difficult to define as the stratification of these patients is challenging. From our experience, major cardiovascular events (MCE) tend to occur more frequently in patients expressing overlap syndrome phenotype (Brugada syndrome and Long QT syndrome type 3)than in patients expressing a single phenotype (whether Brugada syndrome or Long QT syndrome type 3). This trials is led on the impact on Risk Stratification of Overlap Syndrome Phenotype in Patients With E1784K Mutation in SCN5A ( RISKOVER )
Congenital long QT syndrome (LQTS) is a rare genetic disorder characterized by prolongation of the corrected QT interval (QTc) on the electrocardiogram. LQTS is associated with a risk of syncope or sudden death from ventricular arrhythmia. The increase in the duration of the corrected QT interval and / or changes in the morphology of the T wave on the electrocardiogram are markers of an increased risk of sudden death and syncope. Recently, a personal portable 6-lead device (DI, DII, DIII, aVF, aVL and aVR) connected to the patient's smartphone has entered the market (KARDIA MOBILE 6L, AliveCor, Mountain View, CALIFORNIA, USA). The APPLE WATCH Series 6 (Apple, Cupertino, CA, USA) can also record an ECG. If the device is designed to record a single lead (DI), several works have shown that it is possible to record 9 leads (DI, DII, DIII, V1, V2, V3, V4, V5 and V6) by moving simply the device (Spaccarotella CAS et al. JAMA Cardiology 2020). These devices were originally designed to screen for atrial fibrillation, but they produce accurate 6- and 9-lead ECGs and could potentially allow ambulatory ECG monitoring of patients with LQTS.
The objectives of the study are to investigate if KCNQ1 mutation in Romano-Ward long QT patients can be associated with changes in insulin regulation and with psychological features of compulsivity, impulsivity and behavioural rigidity.
The QUALIMYORYTHM trial is a multicentre controlled study, aiming to assess health-related quality of life (HRQoL) of 107 children aged 6 to 18 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathies (hypertrophic, dilated, or restrictive cardiomyopathy), and to compare the results to those of 107 age and gender-matched healthy subjects. The secondary objective is to assess, in this population, the HRQoL according to disease characteristics, level of physical activity, exercise capacity, and socio-demographic data. Participants will wear a fitness tracker for 2 weeks.
In studies, the effects of drugs used for anesthesia and analgesia on QT distance were evaluated in isolation. However, drugs are administered in combination with each other during anesthesia induction. Therefore, drugs interact in terms of positive and undesirable effects. In addition, most of the studies examining anesthesia and QT distance have been conducted in non-cardiac surgery. The target group in this study is the adult patient group who will undergo cardiac surgery. The primary aim of our study is to investigate the effect of two different types of anesthesia induction techniques on QT distance in patients undergoing open-heart surgery. QT evaluation will be performed after endotracheal intubation after anesthesia induction.
Azithromycin (AZ) is used in the pediatric group in COVID-19 diseas. It has been reported to cause prolongation in the QT interval in adult age group. The aim of this study is to evaluate the effect of AZ on ventricular repolarization in COVID-19 positive pediatric patients. METHOD The study was conducted prospectively in July-August 2020. COVID-19 pediatric patients who received AZ treatment were included in the study. ECG was obtained before treatment and on the 1st, 3rd and 5th days after the treatment. Measurements were made with Image J program®. QTmax, QTmin, Tp-emax, Tp-emin intervals were measured. QTcmax, QTcmin, Tp-ecmax, Tp-ecmin, QTcd, Tp-ecd, and QTc / Tp-ec ratios were calculated with Bazett formula.
This research will determine if: 1) Oral progesterone attenuates drug-induced QT interval, J-Tpeak and Tpeak-Tend lengthening in postmenopausal women 50 years of age or older, and 2) Transdermal testosterone attenuates drug-induced QT interval, J-Tpeak and Tpeak-Tend lengthening in men 65 years of age or older. This investigation will consist of two concurrent prospective, randomized, double-blind, placebo-controlled crossover-design studies in a) Postmenopausal women, and b) Men 65 years of age or older. Study 1: Each postmenopausal woman will take progesterone or placebo capsules for 1 week. After a 14-day "washout" (no progesterone or placebo) each subject will then take the alternative therapy (progesterone or placebo) for 1 week. After 7 days of each treatment, subjects will present to the clinical research center to receive a small dose of the QT interval-lengthening drug ibutilide, and the effect on the QT, J-Tpeak and Tpeak-Tend intervals during the progesterone and placebo phases will be compared. Study 2: Each man 65 years of age or older will apply transdermal testosterone or transdermal placebo gel for 3 days. After a 7-day "washout" (no testosterone or placebo) each subject will then apply the alternative therapy (testosterone or placebo gel) for 1 week. After 3 days of each treatment, subjects will present to the clinical research center to receive a small dose of the QT interval-lengthening drug ibutilide, and the effect on the QT, J-Tpeak and Tpeak-Tend intervals during the testosterone and placebo phases will be compared.
Use lay language. Current guidelines regarding physical activity in patients with inherited arrhythmia and cardiomyopathy are mostly dedicated to adult patients, with a special focus on sports competition. Their application to the pediatric population has been scarcely evaluated. Physical activity is well known for its health benefits but may be dangerous in this population, which leads to confusion within the medical community and among patients. Actual physical activity of children with such inherited cardiac disorders is unknown. This study aimed to assess the level of physical activity in children with inherited arrhythmia and cardiomyopathy, and the adherence to the current European guidelines on the subject. Secondary objectives aimed to assess through a qualitative analysis the impact of the disease on physical activity and daily life in this population. The level of physical activity and adherence to current guidelines will be determined from interviews between the patient and the principal investigator. Each patient will be questioned in order to explore the experiences, motivations and feelings of participants regarding physical activity. The standardized questionnaire was created by the principal investigator and members of the clinical research team. The investigators believe that many children practice physical activity outside the current guidelines and hope to identify the main determinants of physical activity in this population.
Novel therapy for the Long QT Syndrome based on the mechanism of action of the disease-causing mutations Long QT syndrome type 2 (LQT2) accounts for ~ 35% of all LQTS cases and is difficult to manage, as beta-blockers frequently fail to provide full protection. Most LQT2 patients (pts) have a Class 2 mutation, which implies defective "trafficking". Lumacaftor (LUM) is a drug developed and currently indicated for the treatment of cystic fibrosis (CF) in patients homozygous for the F508del mutation in the CFTR gene. LUM corrects protein folding and trafficking defects of mutant and misfolded CFTR channels, restoring their cell surface expression. The investigators recently demonstrated that LUM can rescue in vitro the LQTS phenotype observed in human induced pluripotent stem cell- derived cardiomyocytes (hiPSC-CMs) from pts with LQT2 Class 2 mutations (PMID: 29020304) and in these same two patients Orkambi administrated for 7 days at the same dosage approved for cystic fibrosis showed to reduce their QTc (PMID: 30753398). With the present phase II clinical trial (MAST2) the investigators will enroll 20 LQT2 patients (see inclusion and exclusion criteria) and they will test in vivo the efficacy of Orkambi in shortening their QTc. Patients will be admitted to hospital for a maximum of 7 days (minimum in-hospital stay based on evidence of QTc shortening). Orkambi will be administered at the dose approved for cystic fibrosis and during the entire period continuous ECG monitoring through both telemetry and 12-lead 24-hr Holter monitoring will be performed and QTc length and morphology will be analyzed.
The purpose of this study is to further develop and evaluate a diagnostic procedure suitable for use in an inexpensive diagnostic instrument suitable for screening for Long QT Syndrome (LQTS) in the primary care environment.