12,283 results match your criteria: "Long QT Syndrome"

Congenital long QT syndrome (LQTS) is a rare hereditary cardiac disorder characterized by prolongation of the QT interval on electrocardiogram (ECG), predisposing affected individuals to life-threatening arrhythmias. We present a case of a newborn with congenital LQTS and 2:1 atrioventricular (AV) block who presented with bradycardia and QT prolongation. Continuous intravenous lidocaine infusion was initiated, because of hypoglycemia with beta-blockers, resulting in stabilization of AV conduction and prevention of malignant arrhythmias.

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Background: This case highlights the management of concomitant acute myocarditis and congenital long QT syndrome with electrical storm and incessant Torsade de Pointes.

Case Presentation: An 18 years-old Southeast Asian para 1 abortus 0 (P1A0) postpartum patient with cesarean section owing to severe preeclampsia, acute lymphocytic myocarditis, and prolonged QT interval owing to long QT syndrome. She has incessant Torsade de Pointes treated with beta-blocker, lidocaine, overdrive pacing with a temporary transvenous pacemaker, left cardiac sympathetic denervation per video-assisted thoracoscopic surgery, and implantable cardioverter-defibrillator implantation.

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Objective: Hydroxychloroquine paired with Azithromycin, Vitamin C, Vitamin D, and Zinc (HAZDPac), was used as a multidrug therapy method to treat COVID-19 illness and superimposed secondary bacterial pneumonia. Concerns have been raised though about such combinations regarding cardiac QTc interval prolongation and risks of arrhythmias, which we set out to address in this study.

Design: We evaluated cardiac safety in a Phase II Double-Blind Randomized Placebo-Controlled Trial of Combination Therapy to Treat COVID-19 Infections study, conducted by ProgenaBiome.

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Unexpected pheochromocytoma leading to cardiac arrest during the perioperative period: a case report and literature review.

BMC Anesthesiol

December 2024

Department of Anaesthesiology, The Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, Zhejiang, 322000, China.

Background: Pheochromocytomas (PCCs) are rare neuroendocrine catecholamine (CA)-secreting tumours that originate from chromaffin tissue and can produce and store CAs. Unexpected PCCs pose a serious threat to the perioperative safety of patients and a considerable challenge to anaesthesiologists because of the risks of fatal hypertensive crises and other stresses.

Case Presentation: A 37-year-old woman who was scheduled for tonsillectomy and palatopharyngoplasty under general anaesthesia experienced a malignant cardiovascular event after induction, which was characterized mainly by a sharp increase in heart rate and blood pressure, ultimately leading to cardiac arrest and the occurrence of secondary long QT syndrome.

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The mechanism of LQTS related CaM mutation E141G interfering with Ca1.2 channels function through its C-lobe.

J Physiol Biochem

December 2024

Department of Pharmaceutical Toxicology, School of Pharmacy, China Medical University, Shenyang, 110122, China.

Mutations in the CALM1-3 genes, which encode calmodulin (CaM), have been reported in clinical cases of long QT syndrome (LQTS). Specifically, the CaM mutant E141G (CaM) in the variant CALM1 gene has been identified as a causative factor in LQTS. This mutation disrupts the normal Ca-dependent inactivation (CDI) function of Ca1.

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Background: Droperidol is a first-generation antipsychotic medication that has been used for various indications in the emergency department (ED); however, its use has been controversial due to reports of QT prolongation and the risk of torsades de pointes (TdP). The aim of the study is to evaluate the safety of droperidol administration in the ED.

Methods: This was a retrospective study, conducted at an academic level I trauma center.

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Background: First-time documentation of rare diseases is normally in the form of case reports. These are typically based on unexpected observations by vigilant clinicians and lead to further research on prevalence and aetiology. One of the best-known Norwegian examples is Jervell and Lange-Nielsen syndrome.

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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.

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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).

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Novel risk predictor of arrhythmias for patients with potassium channel related congenital Long-QT Syndrome.

Heart 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.

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Functional investigation of genetic variants found in long QT syndrome can provide evidence that is needed to confirm the genetic diagnosis and establish the cause of the condition. We performed functional assessment to determine the -score, using a clinically calibrated automated patch clamp assay, for 2 missense variants found in 2 families that have been diagnosed with long QT syndrome. These variants are currently classified as variant of uncertain significance in ClinVar.

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Can wearables outscore general practitioners? Congenital long QT syndrome diagnosis initiated by a smartwatch.

HeartRhythm Case Rep

October 2024

I. Department of Internal Medicine, Cardiology, Angiology, Haemostaseology, and Medical Intensive Care, Medical Centre Mannheim, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.

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During the COVID-19 pandemic, there has been heightened interest in the QT interval, a crucial indicator of ventricular electrical activity. Mendelian randomization (MR) is used here to investigate the genetic causation between QT interval alterations and COVID-19. Genetic proxies representing three COVID-19 phenotypes-severe, hospitalized, and COVID-19-were identified in over 1,000,000 individuals of European ancestry.

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Bupropion is used as smoking cessation drug and antidepressant. Drug overdose has multi-system involvement including neurotoxicity and cardiovascular collapse. Use of haemoperfusion in bupropion toxicity is generally not useful, however in life-threatening condition it could be a life saver.

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Article Synopsis
  • * Bilateral CSD (BCSD) shows an approximate 60% reduction in implantable cardioverter defibrillator shocks and over 50% one-year survival without shock or transplant, while the 2017 guidelines endorse the left CSD for specific RVT types.
  • * Despite the promising results, many studies have small sample sizes leading to potential underreporting of complications; more research is needed to fully understand the long
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Computerized QT and QTc Measurements From Bedside ICU Monitors Are Similar to Those Derived From a Standard 12-Lead ECG.

Ann Noninvasive Electrocardiol

January 2025

ECG Monitoring Research Lab, Department of Physiological Nursing, School of Nursing, University of California San Francisco, San Francisco, California, USA.

Article Synopsis
  • QT/QTc prolongation can lead to torsade de pointes, and while 12-lead ECGs are commonly used for assessment, they disrupt patient care; bedside monitors with QT/QTc software have been implemented in the ICU.
  • A study assessed the accuracy of QT/QTc measurements from these bedside monitors using four leads compared to standard 12-lead ECGs, involving measurements taken within 30 minutes of each other.
  • The results indicated good agreement between the two methods, suggesting that bedside monitor QT/QTc readings could effectively identify at-risk patients without needing as many 12-lead ECGs, though further research is needed with larger and non-ICU patient samples.
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QTc interval prolongation and its associated factors among HIV infected patients on ART in Mettu Town.

Sci Rep

December 2024

Department of Biomedical Sciences, College of Medicine and Health Science, Wolkite University, Wolkite, Ethiopia.

QTc interval prolongation, which is linked to cardiac arrhythmia and sudden cardiac death, is common in HIV-infected patients. This prolongation can be attributed to the side effects of antiretroviral medications, the direct impact of HIV on the heart, and chronic inflammatory responses that disrupt cardiac electrical activity. This study aimed at assessing QTc interval prolongation and associated factors among HIV-infected adults on ART.

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The sodium/glucose cotransporter 2 inhibitor Empagliflozin inhibits long QT 3 late sodium currents in a mutation specific manner.

J Mol Cell Cardiol

December 2024

Department of Pharmacology, Alberta Diabetes Institute, Faculty of Medicine and Dentistry, University of Alberta, 7-55 Medical Sciences Building, Edmonton T6G 2H7, Alberta, Canada. Electronic address:

Article Synopsis
  • Sodium/glucose cotransporter 2 inhibitors (SGLT2is), such as empagliflozin, show potential heart protection benefits in individuals with or without diabetes and can inhibit a key cardiac sodium current linked to congenital long QT syndrome type 3 (LQT3).
  • Researchers used the whole-cell patch-clamp technique to study how empagliflozin affects late sodium current (late I) in various LQT3 mutations of the Nav1.5 channel.
  • Empagliflozin specifically inhibited late I in certain mutations without altering channel kinetics, suggesting it could be an effective targeted treatment for patients with LQT3 mutations in the inactivation gate area.
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