JACC Case Rep. 2025 Dec 24:106521. doi: 10.1016/j.jaccas.2025.106521. Online ahead of print.ABSTRACTBACKGROUND: Prolonged QT interval on electrocardiogram along with sensorineural deafness is consistent with Jervell and Lange-Nielsen syndrome (JLNS). We report a case where genetic testing revealed d
Medicine (Baltimore). 2026 Jan 16;105(3):e46991. doi: 10.1097/MD.0000000000046991.ABSTRACTRATIONALE: Long QT syndrome (LQTS) constitutes an inherited cardiac. Studies indicate that untreated LQTS carries a high mortality rate, and up to 20% of sudden infant death syndrome cases are associated with t
Eur J Clin Pharmacol. 2026 Jan 19;82(2):57. doi: 10.1007/s00228-025-03990-9.ABSTRACTPURPOSE: Drug-induced long QT interval syndrome (LQTS) is a risk for Torsade de Pointes (TdP) arrhythmia. The purpose was to investigate patients diagnosed with TdP in relation to dispensed risk drugs, the presence o
Clin Pharmacol Drug Dev. 2026 Jan;15(1):e70015. doi: 10.1002/cpdd.70015.ABSTRACTMilvexian is an oral factor XIa inhibitor in development for prevention of major thromboembolic conditions. This randomized, double-blind, placebo- and positive-controlled, multiple-dose, four-period crossover study asse
Sepiapterin and its major metabolite 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (BH(4)) bind to distinct variants of phenylalanine hydroxylase (PAH), which converts excess phenylalanine to tyrosine, thereby stabilizing, enhancing, and prolonging PAH activity. Sepiapterin was recently approved in Europ
JACC Case Rep. 2026 Jan 12:106667. doi: 10.1016/j.jaccas.2025.106667. Online ahead of print.ABSTRACTBACKGROUND: Congenital long QT syndrome (LQTS) predisposes to torsades de pointes and sudden cardiac death. Although it usually manifests in childhood or early adulthood, external triggers may unmask
Heart Rhythm. 2026 Jan 10:S1547-5271(26)00028-7. doi: 10.1016/j.hrthm.2026.01.013. Online ahead of print.ABSTRACTBACKGROUND: Arrhythmia-risk assessment in congenital long-QT syndrome (LQTS) and drug-induced QT prolongation (diQTP) is primarily based on clinical, genetic, and electrical parameters. E
Int J Mol Sci. 2026 Jan 4;27(1):519. doi: 10.3390/ijms27010519.ABSTRACTThe REPRIEVE Trial recently reported high rates of sudden cardiac death (SCD) middle-aged people living with HIV-1 infection (PWH) using the WHO/NIH-recommended two nucleoside reverse transcriptase inhibitors (NRTIs)/one integras
JCI Insight. 2026 Jan 8:e201297. doi: 10.1172/jci.insight.201297. Online ahead of print.ABSTRACTCongenital long QT syndrome (LQTS) promotes risk for life-threatening cardiac arrhythmia and sudden death in children and young adults. Pathogenic variants in the voltage-gated potassium channel KCNQ1 are
Biochem Pharmacol. 2026 Jan 5;245:117681. doi: 10.1016/j.bcp.2026.117681. Online ahead of print.ABSTRACTDonepezil (DPZ), a first-line therapeutic agent for Alzheimer's disease, causes acquired long QT syndrome (LQTS) by inhibiting the hERG potassium channel. However, there are no specific countermea
J Pharmacokinet Pharmacodyn. 2026 Jan 5;53(1):6. doi: 10.1007/s10928-025-10012-9.ABSTRACTPhase I single and multiple ascending dose studies are more and more often used to evaluate QT liability of new drugs. However, these studies are not primarily tailored to concentration-QT analysis and to contro
Front Med (Lausanne). 2025 Dec 16;12:1730755. doi: 10.3389/fmed.2025.1730755. eCollection 2025.ABSTRACTOBJECTIVE: This study aims to compare the efficacy of different types of beta-blockers in preventing arrhythmias in patients with long QT syndrome (LQTS), and simultaneously analyze the relationshi
Genes (Basel). 2025 Dec 3;16(12):1448. doi: 10.3390/genes16121448.ABSTRACTInherited arrhythmias and cardiomyopathies are a group of potentially lethal genetic cardiac disorders which are often passed down through generations and pose risks to several family members. While individually rare, these co
Sci Rep. 2025 Dec 26. doi: 10.1038/s41598-025-33025-3. Online ahead of print.ABSTRACTActivating transcription factor 3 (ATF3) is a crucial regulator of gene expression in response to physiological stress across various tissues. Abnormal ATF3 expression is associated with cardiac dysfunction; however
CONCLUSION: We have uncovered a new genetic basis for LQTS. Our findings demonstrate that the MOG1^(L18F) variant impairs AV conduction, increases I(NaL), opposes repolarizing currents, and prolongs both action potential duration and the QT interval, ultimately leading to ventricular arrhythmias, pa
medRxiv [Preprint]. 2025 Dec 16:2025.12.15.25341924. doi: 10.64898/2025.12.15.25341924.ABSTRACTLoss-of-function variants in KCNQ1 are the primary cause of congenital Long QT Syndrome (LQTS), characterized by QT prolongation and increased risk of fatal arrhythmias. The surge in genetic testing contin
Orphanet J Rare Dis. 2025 Dec 22;20(1):634. doi: 10.1186/s13023-025-04172-7.ABSTRACTBACKGROUND: Long QT syndrome (LQTS) is a malignant cardiac channelopathy for patients with traditionally normal heart structures. Sporadic cases reported left ventricular hypertrabeculation (LVHT) was a deadly double
No abstractCirculation. 2025 Dec 23;152(25):1806-1808. doi: 10.1161/CIRCULATIONAHA.125.074358. Epub 2025 Dec 22.NO ABSTRACTPMID:41428794 | PMC:PMC12721645 | DOI:10.1161/CIRCULATIONAHA.125.074358
CONCLUSIONS: Hypernatremia during Na(v)GOF prevents early ventricular repolarization due to I(to) activation (mouse) and prolongs repolarization in the absence of I(to) (guinea pig). Future studies in animal models electrophysiologically similar to humans are needed to determine if hypernatremia and
Open Heart. 2025 Dec 18;12(2):e003119. doi: 10.1136/openhrt-2024-003119.ABSTRACTINTRODUCTION AND OBJECTIVES: Brugada syndrome (BS) is a channelopathy associated with an increased risk of sudden cardiac death (SCD). Intense physical activity is a recognised trigger of life-threatening arrhythmias in
The class III antiarrhythmic drug amiodarone (AMIO) inhibits the rapidly activating delayed rectifier K^(+) current that is conducted by the human ether-a-go-go-related gene (hERG) encoded channel. Like other class III antiarrhythmic drugs, AMIO can cause long QT syndrome. In the present study, we i
PNAS Nexus. 2025 Dec 8;4(12):pgaf379. doi: 10.1093/pnasnexus/pgaf379. eCollection 2025 Dec.ABSTRACTLong QT syndrome type 3 (LQTS3), caused by gain-of-function mutations in the SCN5A gene, encompasses a spectrum of clinical presentations, ranging from asymptomatic carriers to severe arrhythmic phenot
J Diabetes Res. 2025 Dec 1;2025:8664620. doi: 10.1155/jdr/8664620. eCollection 2025.ABSTRACTINTRODUCTION: There is a notable link between diabetes mellitus (DM) and cardiovascular diseases (CVDs), particularly abnormal ventricular repolarization marked by a prolonged QT interval, which significantly
Case Rep Pediatr. 2025 Nov 30;2025:5621484. doi: 10.1155/crpe/5621484. eCollection 2025.ABSTRACTLong QT syndrome (LQTS) is a hereditary arrhythmic disorder associated with sudden cardiac death. We report a neonatal case of congenital LQTS that went undiagnosed in utero, leading to severe postnatal c
