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Prolonged QT syndrome

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https://www.readbyqxmd.com/read/29325979/an-unusual-case-of-complete-atrioventricular-block-causing-takotsubo-syndrome
#1
Abhishek Rathore, Bharatraj Banavalikar, Jayaprakash Shenthar, Debashish Acharya, Javed Parvez, Kikkeri Hemanna Setty Srinivasa
Complete atrioventricular (AV) block in association with Takotsubo syndrome (TS) has been well recognized, but the cause and effect relationship has not been elucidated. We describe a 78-year-old female who presented with complete AV block but one week later developed new-onset, diffuse T-wave inversions, QT prolongation, and acceleration of junctional escape rate. Left ventriculogram revealed features typical of TS. One year after permanent pacemaker implantation, complete AV block persisted despite the reversal of wall motion defects implying that conduction abnormality was the trigger of TS rather than its consequence...
January 8, 2018: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29314443/ryanodine-receptor-inhibition-by-dantrolene-effectively-suppresses-ventricular-arrhythmias-in-an-ex-vivo-model-of-long-qt-syndrome
#2
Gerrit Frommeyer, Julius Krawczyk, Christian Ellermann, Nils Bögeholz, Simon Kochhäuser, Dirk G Dechering, Michael Fehr, Lars Eckardt
AIMS: A significant antiarrhythmic potential of ryanodine receptor inhibition was reported in experimental studies. The aim of the present study was to assess potential antiarrhythmic effects of dantrolene in an experimental whole-heart model of drug-induced long-QT syndrome (LQTS). METHODS: In 12 isolated rabbit hearts long-QT-2-syndrome was simulated by infusion of erythromycin (300μM). 12 rabbit hearts were treated with veratridine (0.5μM) to mimic long-QT-3-syndrome...
January 4, 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29306897/critical-roles-of-xirp-proteins-in-cardiac-conduction-and-their-rare-variants-identified-in-sudden-unexplained-nocturnal-death-syndrome-and-brugada-syndrome-in-chinese-han-population
#3
Lei Huang, Kuo-Ho Wu, Liyong Zhang, Qinchuan Wang, Shuangbo Tang, Qiuping Wu, Pei-Hsiu Jiang, Jim Jung-Ching Lin, Jian Guo, Lin Wang, Shih-Hurng Loh, Jianding Cheng
BACKGROUND: Sudden unexplained nocturnal death syndrome (SUNDS) remains an autopsy negative entity with unclear etiology. Arrhythmia has been implicated in SUNDS. Mutations/deficiencies in intercalated disc components have been shown to cause arrhythmias. Human cardiomyopathy-associated 1 (XIRP1) and 3 (XIRP2) are intercalated disc-associated, Xin repeats-containing proteins. Mouse Xirp1 is necessary for the integrity of intercalated disc and for the surface expression of transient outward and delayed rectifier K+ channels, whereas mouse Xirp2 is required for Xirp1 intercalated disc localization...
January 6, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29304463/management-of-qt-prolongation-induced-by-anti-cancer-drugs-target-therapy-and-old-agents-different-algorithms-for-different-drugs
#4
REVIEW
Carmela Coppola, Anna Rienzo, Giovanna Piscopo, Antonio Barbieri, Claudio Arra, Nicola Maurea
The side effects of anticancer drugs still play a critical role in survival and quality of life. Although the recent progresses of cancer therapies have significantly improved the prognosis of oncologic patients, side effects of antineoplastic treatments are still responsible for the increased mortality of cancer survivors. Cardiovascular toxicity is the most dangerous adverse effect induced by anticancer therapies. A survey conducted by the National Health and Nutrition Examination, showed that 1807 cancer survivors followed up for seven years: 51% died of cancer and 33% of heart disease (Vejpongsa and Yeh, 2014)...
December 6, 2017: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/29290967/modelling-the-effects-of-chloroquine-on-kcnj2-linked-short-qt-syndrome
#5
Cunjin Luo, Kuanquan Wang, Henggui Zhang
A gain-of-function KCNJ2 D172N mutation in KCNJ2-encoded Kir2.1 channels underlies one form of short QT syndrome (SQT3), which is associated with increased susceptibility to arrhythmias and sudden death. Anti-malarial drug chloroquine was reported as an effective inhibitor of Kir2.1 channels. Using biophysically-detailed human ventricle computer models, this study assessed the effects of chloroquine on SQT3. The ten Tusscher et al. model of human ventricular cell action potential was modified to recapitulate functional changes in the inward rectifier K+ current (IK1) due to heterozygous and homozygous forms of the D172N mutation...
December 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/29258620/refractory-ventricular-fibrillations-after-surgical-repair-of-atrial-septal-defects-in-a-patient-with-cacna1c-gene-mutation-case-report
#6
Ai Kojima, Fumiaki Shikata, Toru Okamura, Takashi Higaki, Seiko Ohno, Minoru Horie, Shunji Uchita, Yujiro Kawanishi, Kenji Namiguchi, Takumi Yasugi, Hironori Izutani
BACKGROUND: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history...
December 19, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29250293/hypothetical-anatomy-of-brugada-phenomenon-long-qt-sine-long-qt-syndrome-implicating-morphologically-undefined-specific-brugada-s-myocells
#7
REVIEW
Petras Stirbys
The Brugada syndrome (BrS) is associated with increased risk of ventricular arrhythmias and sudden cardiac death. It generates genetically mediated arrhythmias posing a true pathophysiological challenge. In search of the similarities between BrS and long QT syndrome some novel insights are suggested. In patients with BrS the duration of QT interval is usually normal. Some investigators have found prolonged QT interval in the syndrome's natural course or the duration of QT segment have been extended by provocative tests unmasking BrS...
April 2017: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/29241489/hydroquinidine-prevents-life-threatening-arrhythmic-events-in-patients-with-short%C3%A2-qt%C3%A2-syndrome
#8
Andrea Mazzanti, Riccardo Maragna, Gaetano Vacanti, Anna Kostopoulou, Maira Marino, Nicola Monteforte, Raffaella Bloise, Katherine Underwood, Valentina Tibollo, Eleonora Pagan, Carlo Napolitano, Riccardo Bellazzi, Vincenzo Bagnardi, Silvia G Priori
BACKGROUND: Short QT syndrome (SQTS) is a rare and life-threatening arrhythmogenic syndrome characterized by abbreviated repolarization. Hydroquinidine (HQ) prolongs the QT interval in SQTS patients, although whether it reduces cardiac events is currently unknown. OBJECTIVES: This study investigated whether long-term treatment with HQ reduces the occurrence of life-threatening arrhythmic events (LAE) (cardiac arrest or sudden cardiac death) in SQTS patients. METHODS: In this cohort study on consecutive SQTS patients, 2 analyses were performed: 1) a matched-period analysis for the occurrence of LAE in 17 SQTS patients who received long-term HQ; and 2) a comparison of the annual incidence of LAE off- and on-HQ in 16 SQTS patients who survived a cardiac arrest...
December 19, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29234865/electromechanical-heterogeneity-in-the-heart-a%C3%A2-key-to-long-qt-syndrome
#9
REVIEW
F F Dressler, J Brado, K E Odening
In the healthy heart, physiological heterogeneities in structure and in electrical and mechanical activity are crucial for normal, efficient excitation and pumping. Alterations of heterogeneity have been linked to arrhythmogenesis in various cardiac disorders such as long QT syndrome (LQTS). This inherited arrhythmia disorder is caused by mutations in different ion channel genes and is characterized by (heterogeneously) prolonged cardiac repolarization and increased risk for ventricular tachycardia, syncope and sudden cardiac death...
December 12, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29201009/in-vitro-and-in-silico-risk-assessment-in-acquired-long-qt-syndrome-the-devil-is-in-the-details
#10
REVIEW
William Lee, Monique J Windley, Jamie I Vandenberg, Adam P Hill
Acquired long QT syndrome, mostly as a result of drug block of the Kv11. 1 potassium channel in the heart, is characterized by delayed cardiac myocyte repolarization, prolongation of the T interval on the ECG, syncope and sudden cardiac death due to the polymorphic ventricular arrhythmia Torsade de Pointes (TdP). In recent years, efforts are underway through the Comprehensive in vitro proarrhythmic assay (CiPA) initiative, to develop better tests for this drug induced arrhythmia based in part on in silico simulations of pharmacological disruption of repolarization...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29194862/a-case-report-is-mexiletine-usage-effective-in-the-shortening-of-qtc-interval-and-improving-the-t-wave-alternans-in-timothy-syndrome
#11
Gulhan Tunca Sahin, Yakup Ergul
Timothy syndrome (TS) is a multisystemic disease that occurs because of a mutation in CACN1C gene and is characterized by prolonged QT interval. Mexiletine is a Class 1B antiarrhythmic drug that causes the disappearance of T-wave alternans by shortening QTc and peak-to-end of the T wave. It may block the development of torsades de pointes in a prolonged QT. This study presented the case of a patient diagnosed with TS and had a cardiac arrest history, prolonged QT, and T-wave alternans. After mexiletine treatment, the QTc interval shortened and T-wave alternans disappeared...
November 30, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29182204/-long-qt-syndrome-and-polymorphic-ventricular-tachycardia-due-to-hypopituitarism-report-of-one-case
#12
José Miguel García-Castro, Antonia García-Martín, Emilio Guirao-Arrabal, Pedro Luis Carrillo-Alascio
Symptoms of hypopituitarism are usually chronic and nonspecific, but rarely the disease can have acute and life threatening manifestations. We report a 53 years old female with a pituitary adenoma that was admitted to our hospital because of syncope. The electrocardiogram showed sinus bradycardia with a prolonged QT interval. Frequent runs of non-sustained polymorphic ventricular tachycardia were noted on telemetry. The patient had a history of severe acute headaches in the previous days and laboratory tests revealed severe secondary hypothyroidism, adrenal insufficiency and a decrease in pituitary hormones...
July 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/29174490/risk-of-cardiac-events-associated-with-antidepressant-therapy-in-patients-with-long-qt-syndrome
#13
Meng Wang, Barbara Szepietowska, Bronislava Polonsky, Scott McNitt, Arthur J Moss, Wojciech Zareba, David S Auerbach
Patients with long QT syndrome (LQTS) are at a high risk of cardiac events. Many patients with LQTS are treated with antidepressant drugs (ADs). We investigated the LQTS genotype-specific risk of recurrent cardiac arrhythmic events (CAEs) associated with AD therapy. The study included 59 LQT1 and 72 LQT2 patients from the Rochester-based LQTS Registry with corrected QT (QTc) prolongation and a history of AD therapy. Using multivariate Anderson-Gill models, we estimated the LQTS genotype-specific risk of recurrent CAEs (ventricular tachyarrhythmias, aborted cardiac arrest, or sudden cardiac death) associated with time-dependent ADs...
November 13, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29173600/a-practical-approach-to-avoiding-cardiovascular-adverse-effects-of-psychoactive-medications
#14
REVIEW
Judith Brouillette, Stanley Nattel
Drugs that act on mental state, generally termed "psychoactive agents," are among the most widely used medications in medicine. Psychoactive agents can affect the cardiovascular system and must be used carefully to avoid negative cardiovascular consequences. In the present article we review the potential adverse cardiovascular consequences of psychoactive medications and provide suggestions for practical approaches to avoiding them. We consider adverse reactions in terms of: (1) arrhythmias (particularly acquired long QT syndrome); (2) blood pressure; (3) ventricular function; (4) effect on risk factors; (5) teratogenicity; and (6) drug interactions...
December 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/29160150/electrocardiogram-screening-in-children-with-congenital-sensorineural-hearing-loss-prevalence-and-follow-up-of-abnormalities
#15
Zainab Farzal, Jonathan Walsh, Faisal I Ahmad, Jason Roberts, Sunita J Ferns, And Carlton J Zdanski
Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29133340/effects-of-hawthorn-crataegus-pentagyna-leaf-extract-on-electrophysiologic-properties-of-cardiomyocytes-derived-from-human-cardiac-arrhythmia-specific-induced-pluripotent-stem-cells
#16
Sara Pahlavan, Marziyeh Shalchi Tousi, Mahdi Ayyari, Abolfazl Alirezalu, Hassan Ansari, Tomo Saric, Hossein Baharvand
Cardiac arrhythmias are major life-threatening conditions. The landmark discovery of induced pluripotent stem cells has provided a promising in vitro system for modeling hereditary cardiac arrhythmias as well as drug development and toxicity testing. Nowadays, nutraceuticals are frequently used as supplements for cardiovascular therapy. Here we studied the cardiac effects of hawthorn (Crataegus pentagyna) leaf extract using cardiomyocytes (CMs) differentiated from healthy human embryonic stem cells, long QT syndrome type 2 (LQTS2), and catecholaminergic polymorphic ventricular tachycardia type 1 (CPVT1) patient-specific induced pluripotent stem cells...
November 13, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29133066/-qtc-interval-in-the-neonatal-period-in-a-mexican-population-a-pilot-study
#17
Rocio Alejandra Peña-Juárez, Adrián Garcia-Canales, Luis Martin Garrido-García, Cesar Augusto Valerio-Carballo
INTRODUCTION: QT interval prolongation is associated with ventricular arrhythmias and sudden death syndrome. OBJECTIVE: To determine the value of QTc interval in healthy newborns in a general hospital in Jalisco, Mexico, and their outcome during their first months of life. METHODS: The study included healthy newborns from March to November 2016, in the Hospital General of Occidente in Jalisco, Mexico. A 12-lead electrocardiogram was performed at a speed of 25mm/s during the first 48h of life...
November 10, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/29121487/risk-of-cardiac-events-in-long-qt-syndrome-patients-when-taking-antiseizure-medications
#18
David S Auerbach, Yitschak Biton, Bronislava Polonsky, Scott McNitt, Robert A Gross, Robert T Dirksen, Arthur J Moss
Many antiseizure medications (ASMs) affect ion channel function. We investigated whether ASMs alter the risk of cardiac events in patients with corrected QT (QTc) prolongation. The study included people from the Rochester-based Long QT syndrome (LQTS) Registry with baseline QTc prolongation and history of ASM therapy (n = 296). Using multivariate Anderson-Gill models, we assessed the risk of recurrent cardiac events associated with ASM therapy. We stratified by LQTS genotype and predominant mechanism of ASM action (Na(+) channel blocker and gamma-aminobutyric acid modifier...
October 20, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/29118886/polymorphic-ventricular-tachycardia-secondary-to-subarachnoid-haemorrhage-a-rare-occurrence-in-the-setting-of-normal-qtc
#19
Siddharth Paresh Shah, Priyanka Pitroda, Kinner Patel, Rahul Chandak, Timothy Ford
Subarachnoid hemorrhage (SAH) is a neurologic emergency associated with high mortality rate. Polymorphic ventricular tachycardia (VT) is a rare arrhythmia. It can occur in any setting of a long QT interval and bradycardia. This may result from a cardiomyopathy (both ischemic and non-ischemic), acute coronary ischemia, congenital long QT syndrome, electrolyte disturbances and cerebrovascular diseases. We report a rare case of polymorphic VT of unclear etiology with a normal corrected QT, likely secondary to SAH...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29101013/the-congenital-long-qt-syndrome-type-3-an-update
#20
REVIEW
Andrés Ricardo Pérez-Riera, Raimundo Barbosa-Barros, Rodrigo Daminello Raimundo, Marianne Penachini da Costa de Rezende Barbosa, Isabel Cristina Esposito Sorpreso, Luiz Carlos de Abreu
Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is consequence of mutation of gene SCN5A which codes for the Nav1.5 Na+ channel α-subunit and electrocardiographically characterized by a tendency to bradycardia related to age, prolonged QT/QTc interval (mean QTc value 478 ± 52 ms), accentuated QT dispersion consequence of prolonged ST segment, late onset of T wave and frequent prominent U wave because of longer repolarization of the M cell across left ventricular wall...
October 31, 2017: Indian Pacing and Electrophysiology Journal
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