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https://www.readbyqxmd.com/read/28302382/congenital-heart-defect-causing-mutation-in-nkx2-5-displays-in-vivo-functional-deficit
#1
Abeer F Zakariyah, Rashida F Rajgara, John P Veinot, Ilona S Skerjanc, Patrick G Burgon
The Nkx2.5 gene encodes a transcription factor that plays a critical role in heart development. In humans, heterozygous mutations in NKX2.5 result in congenital heart defects (CHDs). However, the molecular mechanisms by which these mutations cause the disease remain unknown. NKX2.5-R142C is a mutation that was reported to be associated with atrial septal defect (ASD) and atrioventricular (AV) block in 13-patients from one family. The R142C mutation is located within both the DNA-binding domain and the nuclear localization sequence of NKX2...
March 14, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28299825/fingolimod-gilenya-%C3%A2-in-multiple-sclerosis-bradycardia-atrioventricular-blocks-and-mild-effect-on-the-qtc-interval-something-to-do-with-the-l-type-calcium-channel
#2
Sylvie Pilote, Chantale Simard, Benoit Drolet
Cardiac arrhythmias and ECG abnormalities including bradycardia, prolongation of the QT interval and atrioventricular (AV) conduction blocks have been extensively observed with fingolimod, the first marketed oral drug for treating the relapsing-remitting form of multiple sclerosis. The present study was aiming to further elucidate the effects of fingolimod on cardiac electrophysiology at three different levels; 1) in vitro, 2) ex vivo and 3) in vivo. 1) Patch-clamp experiments in whole cell configuration were performed on Cav 1...
March 16, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28294370/is-there-a-prognostic-relevance-of-electrophysiological-studies-in-bundle-branch-block-patients
#3
Harilaos Bogossian, Gerrit Frommeyer, Kornelius Göbbert, Fuad Hasan, Quy Suu Nguyen, Ilias Ninios, Dejan Mijic, Dirk Bandorski, Reinhard Hoeltgen, Melchior Seyfarth, Bernd Lemke, Lars Eckardt, Markus Zarse
BACKGROUND: The present European guidelines suggest a diagnostic electrophysiological (EP) study to determine indication for cardiac pacing in patients with bundle branch block and unexplained syncope. We evaluated the prognostic relevance of an EP study for mortality and the development of permanent complete atrioventricular (AV) block in patients with symptomatic bifascicular block and first-degree AV block. HYPOTHESIS: The HV interval is a poor prognostic marker to predict the development of permanent AV block in patients with symptomatic bifascicular block (BFB) and AV block I°...
March 15, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28280512/a-case-of-congenitally-corrected-transposition-of-great-arteries-an-infrequent-happenstance
#4
Prakash Ajmera, Vikas Medep
Congenitally corrected transposition of the great arteries (CCTGA) is rare form of congenital heart diseases. It may be present with or without associated anomalies. Patients with CCTGA are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. We report a case of a 42-year-old man who presented at neurosurgery department with dizziness, seizures, and loss of consciousness, in whom isolated CCTGA was discovered incidentally...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28262258/safer-and-escape-junctional-rhythm-a-singular-trigger-for-pacemaker-mediated-tachycardia
#5
Pasquale Crea, Teresa Crea, Giuseppe Picciolo, Francesco Luzza
A 70-year-old man, who previously received a dual chamber pacemaker for paroxysmal AV block (Sorin Symphony DR 2550), was noted on telemetry to have multiple episodes of rapid ventricular pacing at approximately 120bpm. Evaluation of the telemetry strips revealed that all of the rapid ventricular pacing episodes were initiated by brief runs of escape junctional rhythm. Programmed bradycardia parameters were AAI SafeR with lower rate limit of 50bpm.
February 20, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28259696/comparison-of-strategies-for-catheter-ablation-of-focal-atrial-tachycardia-originating-near-the-his-bundle-region
#6
Evgeny Lyan, Mauro Toniolo, Alexey Tsyganov, Luca Rebellato, Alessandro Proclemer, Massimiliano Manfrin, Anatoly Nechepurenko, Alexandr Morozov, Ayan Abdrahmanov, Grigory Gromyko, Vitaly Lyashenko, Marina Yakovleva
BACKGROUND: Recent studies have suggested that para-Hisian atrial tachycardia (AT) can be successfully ablated from the right atrium (RA), left atrium (LA) or non-coronary cusp (NCC) in the aorta; however, the best approach remains unclear. OBJECTIVE: This study aimed to compare different approaches to radiofrequency catheter ablation (RFCA) of para-Hisian AT. METHODS: We retrospectively analyzed 68 consecutive patients (72% females, aged 61±13 years) treated with RFCA for this type of AT...
March 1, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28248410/low-permanent-pacemaker-rates-following-lotus-device-implantation-for-transcatheter-aortic-valve-replacement-due-to-modified-implantation-protocol
#7
Florian Krackhardt, Behrouz Kherad, Maximilian Krisper, Burkert Pieske, Michael Laule, Carsten Tschöpe
BACKGROUND: Conduction disturbances requiring permanent pacemaker implantation following transcatheter aortic valve replacement (TAVR) are a common problem. Pacemaker implantation rates after TAVR appear to be higher compared to conventional aortic valve replacement. The aim of this study was to analyze whether a high annulus implantation conveys the benefit of a decreased rate of permanent pacemaker implantation while being safe and successful according to VAR2-criteria. METHODS: A total of 23 patients with symptomatic severe aortic valve stenosis, an aortic annulus of 19-27mm and at high risk for surgery were treated with the Lotus valve...
March 1, 2017: Cardiology Journal
https://www.readbyqxmd.com/read/28219978/a-mutation-in-the-g-protein-gene-gnb2-causes-familial-sinus-node-and-atrioventricular-conduction-dysfunction
#8
Birgit Stallmeyer, Johanna Kuß, Stefan Kotthoff, Sven Zumhagen, Kirsty S Vowinkel, Susanne Rinne, Lina A Matschke, Corinna Friedrich, Ellen Schulze-Bahr, Stephan Rust, Guiscard Seebohm, Niels Decher, Eric Schulze-Bahr
Rationale: Familial sinus node and atrioventricular (AV) conduction dysfunction is a rare disorder that leads to paroxysmal dizziness, fatigue and syncope due to a temporarily or permanent reduced heart rate. To date, only a few genes for familial sinus and/or AV conduction dysfunction are known and the majority of cases remain etiologically unresolved. Objective: We aim to identify the disease gene in a large three-generation family (n=25) with autosomal dominant sinus node dysfunction (SND) and AV block (AVB) and to characterise the mutation-related pathomechanisms in familial SND+AVB...
February 20, 2017: Circulation Research
https://www.readbyqxmd.com/read/28211989/timothy-syndrome-1-genotype-without-syndactyly-and-major-extracardiac-manifestations
#9
Róbert Sepp, Lidia Hategan, Attila Bácsi, Judit Cseklye, László Környei, János Borbás, Márta Széll, Tamás Forster, István Nagy, Zoltán Hegedűs
Timothy syndrome 1 (TS1) is a rare genetic disorder characterized by multisystem abnormalities including QT prolongation, congenital heart defects, facial dysmorphism, episodic hypoglycemia, and neurological symptoms. A morphological hallmark of TS1 is syndactyly, present in all cases. TS1 is caused by the canonical p.Gly406Arg mutation in the alternatively spliced exon 8A in the CACNA1C gene, encoding for the main cardiac L-type calcium channel. A variant case of TS1 is reported. The proband had intermittent fetal bradycardia with heart rate of 72 bpm...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28197280/predictors-of-permanent-pacemaker-implantation-after-coronary-artery-bypass-grafting-and-valve-surgery-in-adult-patients-in-current-surgical-era
#10
Bandar Al-Ghamdi, Yaseen Mallawi, Azam Shafquat, Alexandra Ledesma, Nadiah AlRuwaili, Mohamed Shoukri, Shahid Khan, Aly Al Sanei
BACKGROUND: Permanent pacemaker (PPM) implantation after cardiac surgery is required in 0.4-6% of patients depending on cardiac surgery type. PPM implantation in the early postoperative period may reduce morbidity and postoperative hospital stay. We performed a retrospective review of electronic medical records of adult patients with coronary artery bypass grafting (CABG), valve surgery, or both, over a 3-year period. Our aim was to identify predictors of PPM requirements and PPM dependency on follow-up in the current surgical era...
August 2016: Cardiology Research
https://www.readbyqxmd.com/read/28185059/severe-proarrhythmic-potential-of-the-antiemetic-agents-ondansetron-and-domperidone
#11
Gerrit Frommeyer, Christina Fischer, Christian Ellermann, Philipp S Lange, Dirk G Dechering, Simon Kochhäuser, Michael Fehr, Lars Eckardt
The potential of ondansetron and domperidone, both clinically established antiemetic agents, to increase the QT-interval has been described in several case reports. Therefore, the aim of the present study was to investigate whether these drugs may provoke polymorphic ventricular tachycardia in a sensitive experimental model of drug-induced proarrhythmia. In 10 female rabbits, ondansetron (1, 5 and 10 µM, n = 10) or domperidone (0.5, 1 and 2 µM, n = 8) was infused after obtaining baseline data. Eight endo- and epicardial monophasic action potentials and a simultaneously recorded 12-lead ECG reproduced the clinically observed QT-prolongation (ondansetron: 1 µM:+17 ms, 5 µM:+41 ms, 10 µM:+78 ms, p < 0...
February 9, 2017: Cardiovascular Toxicology
https://www.readbyqxmd.com/read/28155224/symptomatic-inspiration-induced-atrio-ventricular-block-treated-with-pacemaker-implant
#12
Diego A Hoffmann, Andrés Nicolás Atamañuk, Graciela Aurora Ruiz, Raul Chirife, Maria Cristina Tentori, Simón Salzberg
BACKGROUND: The respiratory sinus arrhythmia (RSA) is the most common cardiac arrhythmia. It consists of phasic variations of sinus cycle length produced by an autonomic reflex, and is characterized by increased heart rate during inspiration and decline in expiration. This phenomenon is due to a reduction of the parasympathetic tone during inspiration, causing a rise in the heart rate, while during expiration the opposite phenomenon occurs. This arrhythmia is more frequent in young adults, although it can also be seen in any age group...
February 3, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28125405/av-nodal-reentrant-tachycardia-with-a-2-1-right-bundle-branch-block-missed-as-bidirectional-ventricular-tachycardia-in-the-first-superficial-evaluation
#13
Mohammad Ali Akbarzadeh, Isa Khaheshi, Mehdi Memaryan, Mohammad Parsa Mahjoob, Mohammadreza Naderian
A 95-year old woman was admitted to our emergency unit because of acute abdominal pain. After urgent surgery according to the acute abdomen, she was referred to intensive care unit (ICU) of the emergency unit as she was intubated. It was developed a run of new arrhythmia which was diagnosed by cardiology resident as bidirectional ventricular tachycardia due to beat to beat changing the axis of the QRS. However, a second and more precise evaluation of the abnormal ECG suggested a narrow supraventricular tachycardia, most probably AV nodal reentrant tachycardia with a 2:1 right bundle branch block...
January 25, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28117225/influence-of-2nd-degree-av-blocks-ecg-recording-length-and-recording-time-on-heart-rate-variability-analyses-in-horses
#14
B H Eggensperger, C C Schwarzwald
OBJECTIVES: To assess the influence of 2nd-degree AV blocks (AVB) on RR interval-based heart rate variability (HRV) variables; to investigate the effect of using PP interval time series and of artifact filtering on HRV analyses; to investigate the influence of electrocardiogram (ECG) recording length and time of recording; and to calculate day-to-day variability and reference intervals of HRV variables. ANIMALS: Thirty healthy adult horses. METHODS: RR and PP interval time series were extracted from 10-h Holter ECGs and an automated filter was applied to the RR time series (RRf)...
January 20, 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/28114077/an-energy-efficient-ecg-signal-processor-detecting-cardiovascular-diseases-on-smartphone
#15
Sanjeev Kumar Jain, Basabi Bhaumik
A novel disease diagnostic algorithm for ECG signal processing based on forward search is implemented in Application Specific Integrated Circuit (ASIC) for cardiovascular disease diagnosis on smartphone. An ASIC is fabricated using 130-nm CMOS low leakage process technology. The area of our PQRST ASIC is 1.21 mm (2). The energy dissipation of PQRST ASIC is 96 pJ with a supply voltage of 0.9 V. The outputs from the ASIC are fed to an Android application that generates diagnostic report and can be sent to a cardiologist via email...
September 26, 2016: IEEE Transactions on Biomedical Circuits and Systems
https://www.readbyqxmd.com/read/28106024/-a-rare-cause-of-2-1-atrioventricular-block-and-congestive-heart-failure-in-preterm-infants-hypocalcemia
#16
Emine Azak, Hatice Tatar Aksoy, Handan Ünsal, İbrahim İlker Çetin
Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2:1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.
January 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28100182/electrocardiographic-assessments-and-cardiac-events-after-fingolimod-first-dose-a-comprehensive-monitoring-study
#17
MULTICENTER STUDY
Volker Limmroth, Tjalf Ziemssen, Michael Lang, Stephan Richter, Bert Wagner, Judith Haas, Stephan Schmidt, Kathrin Gerbershagen, Christoph Lassek, Luisa Klotz, Olaf Hoffmann, Christian Albert, Katrin Schuh, Monika Baier-Ebert, Guillaume Wendt, Heinke Schieb, Susanne Hoyer, Ralf Dechend, Wilhelm Haverkamp
BACKGROUND: First dose observation for cardiac effects is required for fingolimod, but recommendations on the extent vary. This study aims to assess cardiac safety of fingolimod first dose. Individual bradyarrhythmic episodes were evaluated to assess the relevance of continuous electrocardiogram (ECG) monitoring. METHODS: START is an ongoing open-label, multi-center study. At the time of analysis 3951 patients were enrolled. The primary endpoints are the incidence of bradycardia (heart rate < 45 bpm) and second-/third-degree AV blocks during treatment initiation...
January 18, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28097671/junctional-ectopic-tachycardia-localization-and-procedural-approach-using-cryoablation
#18
Alyson R Pierick, Ian H Law, Jennifer R Muldonado, Nicholas H VON Bergen
BACKGROUND: Idiopathic junctional ectopic tachycardia (JET) may still be difficult to control with antiarrhythmic therapy. Transcatheter ablation can be challenging and may be associated with a high risk of unintended AV block. The objective of this manuscript is to report the procedural technique, the location of the successful ablation and the procedural characteristics while utilizing 3D mapping for cryoablation of JET. METHODS: A retrospective analysis was performed on all patients who had undergone cryothermal ablation for the treatment of JET at a single center...
January 18, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28089344/heparan-sulfate-storage-in-the-cardiac-conduction-system-triggers-atrioventricular-block
#19
Rie Kato, Hiroaki Miyahara, Tatsuya Kawano, Atsuko Matsuzuka, Kimiko Noda, Tatsuro Izumi
OBJECTIVE: To elucidate the novel biological functions of heparan sulfate (HS) by clinic-pathologically studying a patient with paroxysmal atrioventricular (AV) block. PATIENT: A long-surviving male patient with Sanfilippo syndrome type A presented with paroxysmal AV block at age 33years. He then survived another 2.5years after the onset of paroxysmal AV block and pacemaker implantation. METHODS AND RESULTS: His cardiac histopathological examination at autopsy showed HS storage in the cardiac conduction system (CCS), especially in the atrioventricular node (AVN)-His bundle branches...
January 12, 2017: Brain & Development
https://www.readbyqxmd.com/read/28018021/exome-sequencing-identifies-compound-heterozygous-mutations-in-scn5a-associated-with-congenital-complete-heart-block-in-the-thai-population
#20
Chuphong Thongnak, Pornprot Limprasert, Duangkamol Tangviriyapaiboon, Suchaya Silvilairat, Apichaya Puangpetch, Ekawat Pasomsub, Chonlaphat Sukasem, Wasun Chantratita
Background. Congenital heart block is characterized by blockage of electrical impulses from the atrioventricular node (AV node) to the ventricles. This blockage can be caused by ion channel impairment that is the result of genetic variation. This study aimed to investigate the possible causative variants in a Thai family with complete heart block by using whole exome sequencing. Methods. Genomic DNA was collected from a family consisting of five family members in three generations in which one of three children in generation III had complete heart block...
2016: Disease Markers
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