Primary diagnosis of atrioventricular pseudo-block in a neonate with definitive diagnosis of long QT syndrome: diagnostic considerations and therapeutic approaches.

Authors: Khorgami, Mohammadrafie¹ (AUTHOR), Naderi, Fatemeh² (AUTHOR) fatemeh_naderi2010@yahoo.com, Kalayinia, Samira³ (AUTHOR)

Source: International Journal of Emergency Medicine. 2/11/2025, Vol. 18 Issue 1, p1-9. 9p.

Subject Terms: *LONG QT syndrome diagnosis, *CONGENITAL heart disease diagnosis, *CONGENITAL heart disease, *LONG QT syndrome, *NEONATAL intensive care units, *AMBULATORY electrocardiography, *TREATMENT effectiveness, *NEONATAL intensive care, *CHEST X rays, *VENTRICULAR tachycardia, *MEXILETINE, *X-rays, *ADRENERGIC beta blockers, *HEART block, *GENETIC testing, *CHILDREN

Management of patient with acute lymphocytic myocarditis and congenital long QT syndrome presenting with electrical storm and incessant Torsade de Pointes: a case report.

Authors: Karwiky, Giky¹ (AUTHOR), Pranata, Raymond¹ (AUTHOR), Undarsa, Alberta Claudia¹ (AUTHOR), Iqbal, Mohamad¹ (AUTHOR), Prameswari, Hawani Sasmaya¹ (AUTHOR), Akbar, Mohammad Rizki¹ (AUTHOR) m.r.akbar@unpad.ac.id

Source: Journal of Medical Case Reports. 12/20/2024, Vol. 18 Issue 1, p1-7. 7p.

Subject Terms: *LONG QT syndrome, *VIDEO-assisted thoracic surgery, *CESAREAN section, *IMPLANTABLE cardioverter-defibrillators, *THUNDERSTORMS

Alleviating the Effects of Short QT Syndrome Type 3 by Allele-Specific Suppression of the KCNJ2 Mutant Allele.

Authors: Wilders, Ronald¹ (AUTHOR) r.wilders@amsterdamumc.nl

Source: International Journal of Molecular Sciences. Dec2024, Vol. 25 Issue 24, p13351. 25p.

Subject Terms: *GAIN-of-function mutations, *LONG QT syndrome, *ACTION potentials, *ION channels, *GENETIC mutation

A novel computational model of swine ventricular myocyte reveals new insights into disease mechanisms and therapeutic approaches in Timothy Syndrome.

Authors: Trancuccio, Alessandro^1,2 (AUTHOR), Tarifa, Carmen³ (AUTHOR), Bongianino, Rossana¹ (AUTHOR), Priori, Silvia G.^1,2,3 (AUTHOR), Santiago, Demetrio J.³ (AUTHOR) demetriojulian.santiago@cnic.es

Source: Scientific Reports. 11/30/2024, Vol. 14 Issue 1, p1-20. 20p.

Subject Terms: *LONG QT syndrome, *ACTION potentials, *HEART diseases, *SWINE, *SENSITIVITY analysis

A Natural History Study of Timothy Syndrome.

Authors: Timothy, Katherine W.¹ (AUTHOR), Bauer, Rosemary^2,3 (AUTHOR), Larkin, Kerry A.^2,4 (AUTHOR), Walsh, Edward P.⁵ (AUTHOR), Abrams, Dominic J.⁵ (AUTHOR), Gonzalez Corcia, Cecilia⁶ (AUTHOR), Valsamakis, Alexandra⁷ (AUTHOR), Pitt, Geoffrey S.⁸ (AUTHOR), Dick, Ivy E.⁹ (AUTHOR) ied@som.umaryland.edu, Golden, Andy² (AUTHOR)

Source: Orphanet Journal of Rare Diseases. 11/23/2024, Vol. 19 Issue 1, p1-21. 21p.

Subject Terms: *LONG QT syndrome, *SYMPTOMS, *GENETIC variation, *RARE diseases, *HYPOGLYCEMIA

The Yield of Genetic Testing and Putative Genetic Factors of Disease Heterogeneity in Long QT Syndrome Patients.

Authors: Bukaeva, Anna¹ (AUTHOR) alersh@mail.ru, Ershova, Alexandra¹ (AUTHOR) kharlapmaria@yahoo.com, Kharlap, Maria¹ (AUTHOR) sanyutabe@gmail.com, Kiseleva, Anna¹ (AUTHOR) vlakutsenko@ya.ru, Kutsenko, Vladimir¹ (AUTHOR) divashuk@gmail.com, Sotnikova, Evgeniia¹ (AUTHOR) mpokrovskaia@list.ru, Divashuk, Mikhail^1,2 (AUTHOR) vostryakova.elizaveta@gmail.com, Pokrovskaya, Maria¹ (AUTHOR) blokhina0310@gmail.com, Garbuzova, Elizaveta¹ (AUTHOR) anna.petukhova.96@gmail.com, Blokhina, Anastasia¹ (AUTHOR) azharikova89@gmail.com, Kopylova, Oksana¹ (AUTHOR) ramensky@gmail.com, Zotova, Evgenia¹ (AUTHOR) marija.zaicenoka@gmail.com, Petukhova, Anna¹ (AUTHOR) vyatkin@gmail.com, Zharikova, Anastasia^1,3 (AUTHOR) meshkov@lipidclinic.ru, Ramensky, Vasily^1,3,4 (AUTHOR) drapkina@bk.ru, Zaicenoka, Marija^1,5 (AUTHOR), Vyatkin, Yuri^1,4 (AUTHOR), Meshkov, Alexey¹ (AUTHOR), Drapkina, Oxana¹ (AUTHOR)

Source: International Journal of Molecular Sciences. Nov2024, Vol. 25 Issue 22, p11976. 16p.

Subject Terms: *LONG QT syndrome, *GENETIC testing, *GENETIC disorder diagnosis, *MOLECULAR diagnosis, *DISEASE vectors

Quercetin is a foe in the heart by targeting the hERG potassium channel.

Authors: Zihao Lu¹, Shuwen Li¹, Rui Wei², Wenwen Li¹, Yuqian Huang¹, Tingting Yang¹, Meng Yan¹ yanmeng0727@xzhmu.edu.cn

Source: Iranian Journal of Basic Medical Sciences. Nov2024, Vol. 27 Issue 11, p1397-1404. 8p.

Subject Terms: *TRANSCRIPTION factor Sp1, *LONG QT syndrome, *ACTION potentials, *AMINO acid residues, *CARDIAC arrest, *QUERCETIN

Transmural APD heterogeneity determines ventricular arrhythmogenesis in LQT8 syndrome: Insights from Bidomain computational modeling.

Authors: Scacchi, Simone¹ (AUTHOR), Pavarino, Luca F.² (AUTHOR) luca.pavarino@unipv.it, Mazzanti, Andrea^3,4,5 (AUTHOR), Trancuccio, Alessandro^3,4,5 (AUTHOR), Priori, Silvia G.^3,4,5 (AUTHOR), Colli Franzone, Piero² (AUTHOR)

Source: PLoS ONE. 7/5/2024, Vol. 19 Issue 7, p1-21. 21p.

Subject Terms: *VENTRICULAR arrhythmia, *LONG QT syndrome, *ARRHYTHMIA, *ACTION potentials, *VENTRICULAR tachycardia, *HETEROGENEITY, *HETEROZYGOSITY

When Bad Luck Strikes Twice: Beckwith Wiedemann Syndrome Associated with Familial Long QT Syndrome Type I.

Authors: Petchesi, Codruta Diana^1,2, Kozma, Kinga^1,3 kozmaki@gmail.com, Iuhas, Alin Remus^3,4, Hodisan, Ramona¹, Jurca, Alexandru Daniel¹

Source: Archives of Pharmacy Practice. Jul-Sep2024, Vol. 15 Issue 3, p49-53. 5p.

Subject Terms: *LONG QT syndrome, *GENOMIC imprinting, *GENETIC disorders, *GENETIC variation, *CHROMOSOMES

Tpeak‐Tend ECG Marker in Obesity and Cardiovascular Diseases: A Comprehensive Review.

Authors: Dykiert, Irena¹ (AUTHOR), Florek, Kamila² (AUTHOR), Kraik, Krzysztof² (AUTHOR), Gać, Paweł³ (AUTHOR) pawel.gac@umw.edu.pl, Poręba, Rafał⁴ (AUTHOR), Poręba, Małgorzata⁵ (AUTHOR), Melchio, Remo (AUTHOR)

Source: Scientifica. 6/25/2024, Vol. 2024, p1-15. 15p.

Subject Terms: *DISEASE risk factors, *LONG QT syndrome, *CARDIOVASCULAR diseases, *MYOCARDIAL infarction, *HEART failure, *ARRHYTHMIA

Joint effects of CD8A and ICOS in Long QT Syndrome (LQTS) and Beckwith-Wiedemann Syndrome (BWS).

Authors: Meng, Ling-bing¹ (AUTHOR), Li, Yongchao² (AUTHOR), Lv, Tingting¹ (AUTHOR), Lv, Changhua¹ (AUTHOR), Liu, Lianfeng¹ (AUTHOR), Zhang, Ping¹ (AUTHOR) zhpdoc@126.com

Source: Journal of Cardiothoracic Surgery. 6/6/2024, Vol. 19 Issue 1, p1-15. 15p.

Subject Terms: *LONG QT syndrome, *T cell receptors, *GENE regulatory networks, *IMMUNOLOGIC memory, *SYNDROMES

Early Screening for Long QT Syndrome and Cardiac Anomalies in Infants: A Comprehensive Study.

Authors: Nosetti, Luana¹ (AUTHOR) luana.nosetti@uninsubria.it, Zaffanello, Marco² (AUTHOR) marco.zaffanello@univr.it, Lombardi, Carolina^3,4 (AUTHOR), Gerosa, Alessandra¹ (AUTHOR), Piacentini, Giorgio² (AUTHOR), Abramo, Michele¹ (AUTHOR), Agosti, Massimo⁵ (AUTHOR) massimo.agosti@uninsubria.it

Source: Clinics & Practice. Jun2024, Vol. 14 Issue 3, p1038-1053. 16p.

Subject Terms: *LONG QT syndrome, *MEDICAL screening, *SUDDEN infant death syndrome, *CONGENITAL heart disease, *BUNDLE-branch block, *BRUGADA syndrome, *ARRHYTHMIA

Comparison of QTc interval changes in drug-resistant tuberculosis patients on delamanid-containing regimens versus shorter treatment regimens.

Authors: Jefman Efendi Marzuki, H.Y.^1,2, Nafrialdi, Nafrialdi³, Sawitri, Neni⁴, Sugiri, Yani Jane⁵, Gusti Agung Ayu Putu Sri Darmayani, I^1,6, Ascobat, Purwantyastuti³ purwanty2703@yahoo.com

Source: International Journal of Risk & Safety in Medicine. 2024, Vol. 35 Issue 2, p181-190. 10p.

Subject Terms: *LONG QT syndrome, *RETROSPECTIVE studies, *HOSPITALS, *RELATIVE medical risk, *ELECTROCARDIOGRAPHY, *ANTITUBERCULAR agents, *LONGITUDINAL method, *VENTRICULAR tachycardia, *COMPARATIVE studies, *CONFIDENCE intervals

Geographic Terms: INDONESIA

Neonatal death of siblings with Uhl's disease and KCNH2 mutation - A rare association.

Authors: Ventura, Francesco^1,2 (AUTHOR) francesco.ventura@unige.it, Barranco, Rosario¹ (AUTHOR), Buffelli, Francesca³ (AUTHOR), Fulcheri, Ezio³ (AUTHOR), Coviello, Domenico⁴ (AUTHOR), Palmieri, Antonella⁵ (AUTHOR)

Source: Annals of Pediatric Cardiology. Jul/Aug2024, Vol. 17 Issue 4, p292-294. 3p.

Subject Terms: *CONGENITAL heart disease, *LONG QT syndrome, *CARDIOMYOPATHIES, *AUTOPSY, *RARE diseases, *PERINATAL death, *AGE factors in disease, *GENETIC mutation, *CARDIOPULMONARY resuscitation, *GENETIC testing

Transcriptome and open chromatin analysis reveals the process of myocardial cell development and key pathogenic target proteins in Long QT syndrome type 7.

Authors: Chen, Peipei^1,2 (AUTHOR), Long, Junyu³ (AUTHOR), Hua, Tianrui² (AUTHOR), Zheng, Zhifa⁴ (AUTHOR), Xiao, Ying² (AUTHOR), Chen, Lianfeng² (AUTHOR), Yu, Kang¹ (AUTHOR) yuk1997@sina.com, Wu, Wei^2,5 (AUTHOR) camsww@163.com, Zhang, Shuyang^2,5 (AUTHOR) shuyangzhang103@nrdrs.org

Source: Journal of Translational Medicine. 3/25/2024, Vol. 22 Issue 1, p1-21. 21p.

Subject Terms: *LONG QT syndrome, *ARRHYTHMIA, *INDUCED pluripotent stem cells, *ACTION potentials, *POTASSIUM channels, *POTASSIUM ions

A case of short QT‐interval postventricular arrhythmia arrest from Torsade De Pointes, a new phenotype, or the result of tachycardia‐mediated imbalance.

Authors: Ifedili, Ikechukwu¹ iifedili@uthsc.edu, Maturana, Miguel¹, Kayali, Sharif¹, Levine, Yehoshua¹, Kabra, Rajesh^1,2, Jha, Sunil K.¹

Source: Journal of Cardiovascular Electrophysiology. Mar2024, Vol. 35 Issue 3, p501-504. 4p.

Subject Terms: *LONG QT syndrome, *SHORT QT syndrome, *SYNCOPE, *VENTRICULAR fibrillation, *VENTRICULAR tachycardia, *ARRHYTHMIA, *ELECTROCARDIOGRAPHY, *VENTRICULAR arrhythmia, *CARDIAC arrest, *DISEASE relapse, *DISEASE complications, *ADOLESCENCE

Proceed with caution: Standard protocol exercise stress tests fail to replicate the diagnostic utility of supine‐stand tests for long QT syndrome.

Authors: Pinsky, Alexa M.¹, Kulkarni, Veda K.¹, Bos, J. Martijn¹, Neves, Raquel¹, Allison, Thomas G.¹, Ackerman, Michael J.^1,2 ackerman.michael@mayo.edu

Source: Pacing & Clinical Electrophysiology. Mar2024, Vol. 47 Issue 3, p455-461. 7p.

Subject Terms: *LONG QT syndrome diagnosis, *MEDICAL protocols, *RECEIVER operating characteristic curves, *DATA analysis, *RESEARCH funding, *STANDING position, *STATISTICAL sampling, *RETROSPECTIVE studies, *DESCRIPTIVE statistics, *ELECTROCARDIOGRAPHY, *CARDIOPULMONARY system, *SUPINE position, *HEART beat, *MEDICAL records, *ACQUISITION of data, *CASE-control method, *ONE-way analysis of variance, *STATISTICS, *EXERCISE tests, *DATA analysis software, *CONFIDENCE intervals, *GENOTYPES, *SENSITIVITY & specificity (Statistics)

Geographic Terms: MINNESOTA

Kardiologisches Management in der Schwangerschaft bei Long-QT 2 Syndrom.

Alternate Title: Pregnancy Management in a Patient with Long QT Syndrome Type 2 (LQT2) carrier of the variant KCNH2: Trp100X.

Authors: Hametner, Greta¹ greta_hametner@gmx.net, Osranek, Martin¹, Brunckhorst, Corinna², Domingo, Argelia Medeiros³

Source: Praxis (16618157). feb2024, Vol. 113 Issue 2, p50-54. 5p.

Subject Terms: *CARDIAC arrest, *LONG QT syndrome, *ION channels, *VENTRICULAR arrhythmia, *PREGNANCY, *BRUGADA syndrome, *ELECTROCARDIOGRAPHY

Identification and characterization of two novel KCNH2 mutations contributing to long QT syndrome.

Authors: Owusu-Mensah, Anthony¹ (AUTHOR) aowus003@odu.edu, Treat, Jacqueline² (AUTHOR), Bernardi, Joyce² (AUTHOR), Pfeiffer, Ryan² (AUTHOR), Goodrow, Robert² (AUTHOR), Tsevi, Bright³ (AUTHOR), Lam, Victoria¹ (AUTHOR), Audette, Michel⁴ (AUTHOR), Cordeiro, Jonathan M.^2,5 (AUTHOR), Deo, Makarand³ (AUTHOR)

Source: PLoS ONE. 1/5/2024, Vol. 19 Issue 1, p1-17. 17p.

Subject Terms: *LONG QT syndrome, *GENETIC testing, *ACTION potentials, *PURKINJE cells, *GENETIC mutation, *ARRHYTHMIA

Comparison of QT Correction Methods in the Pediatric Population of a Community Hospital: A Retrospective Study.

Authors: Kwag, Koren Hyogene¹ khk7006@nyp.org, Kak, Ibrahim¹, Ying Li², Khass, Walid¹, McKechnie, Alec¹, Nulman, Oksana¹, Brown, Brande¹, Chhabra, Manoj¹

Source: Congenital Heart Disease. 2024, Vol. 19 Issue 1, p107-121. 15p.

Subject Terms: *CHILD patients, *LONG QT syndrome, *WILCOXON signed-rank test, *ACTION potentials, *ALGORITHMS