Sympathetic provocative testing is commonly used to detect the abnormal QT dynamics in long QT syndrome (LQTS) patients, particularly LQTS type 1 and type 2. However, little is known about LQTS type 3 (LQT3). We investigated QT dynamics during exercise testing in LQTS patients, particularly LQT3. This study included 37 subjects, comprising 16 genotyped LQTS patients and 21 unrelated healthy subjects without QT prolongation. LQTS patients were divided into LQT3 and non-LQT3 groups. During exercise tests using a modified Bruce protocol, 12-lead electrocardiogram monitoring was performed using a novel multifunctional electrocardiograph. QT intervals were automatically measured. The QT/heart rate (HR) relationship was visualized by plotting the beat-to-beat confluence of the recorded data. A linear regression analysis was performed to determine the QT/HR slope and intercept. Estimated QT intervals at HR 60 bpm (QT60) were calculated by the regression line formula. QT/HR slopes were steeper for each LQTS group than for the control group (P < 0.001). QT60 values demonstrated a moderate correlation with QT intervals at rest (P < 0.0001) for both groups. The corrected QT intervals (QTc) at 4 min of recovery after exercise were significantly longer in the non-LQT3 group than in the control group but were not different between the LQT3 and the control groups. Abnormal QT dynamics during exercise testing were observed in both LQT3 patients and other LQTS subtypes. This method may be useful for directing genetic testing in subjects with borderline prolonged QT intervals.
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http://dx.doi.org/10.1007/s00246-016-1360-4 | DOI Listing |
Calmodulinopathies are caused by mutations in calmodulin (CaM), and result in debilitating cardiac arrythmias such as long-QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). In addition, many patients exhibit neurological comorbidities, including developmental delay and autism spectrum disorder. Until now, most work into these mutations has focused on cardiac effects, identifying impairment of Ca /CaM-dependent inactivation (CDI) of Ca 1.
View Article and Find Full Text PDFCureus
November 2024
Cardiology, Manchester University NHS Foundation Trust, Manchester, GBR.
In this case report, we present a 24-year-old woman with a previous diagnosis of epilepsy who was admitted to the hospital following loss of consciousness (LOC). It was initially assumed that this was an epileptic seizure based on her previous diagnosis of epilepsy; however, a review of her electrocardiograms (ECGs) revealed a prolonged QT interval. She was admitted to the cardiology ward for continuous ECG monitoring and subsequently developed self-limiting torsades de pointes (TDP).
View Article and Find Full Text PDFHeart Rhythm
December 2024
Department of Experimental Cardiology, Heart Centre, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, the Netherlands; Member of the European Reference Network for rare, low prevalence and complex diseases of the heart: ERN GUARD-Heart. Electronic address:
Background: Congenital long-QT syndrome (LQTS) is characterized by delayed ventricular repolarization, predisposing to potentially lethal ventricular arrhythmias. The variability in disease severity among patients remains largely unexplored, underscoring the limitations of current risk stratification methods.
Objective: We aimed to evaluate the potential utility of exercise stress test (EST) electrocardiographic markers in identifying high-risk LQTS patients.
Int J Mol Sci
November 2024
National Medical Research Center for Therapy and Preventive Medicine, 101990 Moscow, Russia.
Front Cardiovasc Med
October 2024
Department of Cardiology, Rabin Medical Center, Petah-Tikva and the Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Background And Aims: Long QT syndrome (LQTS) and coronary artery disease (CAD) are both associated with increased risk of ventricular tachyarrhythmia However, there are limited data on the incremental risk conferred by CAD in adult patients with congenital LQTS. We aimed to investigate the risk associated with CAD and life threatening events (LTEs) in patients with LQTS after age 40 years.
Methods: The risk of LTEs (comprising aborted cardiac arrest, sudden cardiac death, or appropriate defibrillator shock) from age 40 through 75 years was examined in 1,020 subjects from the Rochester LQTS registry, categorized to CAD ( = 137) or no-CAD ( = 883) subgroups.
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