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https://www.readbyqxmd.com/read/28219978/a-mutation-in-the-g-protein-gene-gnb2-causes-familial-sinus-node-and-atrioventricular-conduction-dysfunction
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28000218/supra-hisian-conduction-block-as-an-unusual-presenting-feature-of-takotsubo-cardiomyopathy
#14
Mukund Aravind Prabhu, Praveen Gopalakrishna Pai, Anjith Vupputuri, Saritha Shekhar, Madhavankutty Santhakumari Harikrishnan, Natarajan Umayammal Kumaraswamy
BACKGROUND: Atrioventricular (AV) block is rare in Takotsubo cardiomyopathy (TC). CASE REPORT: A 66-year-old female presented with fatigue. Her electrocardiogram revealed 3:2 Mobitz Type II AV block, confirmed to be supra-Hisian by electrophysiological study. Echocardiogram and left ventricular angiogram showed moderate left ventricular dysfunction and apical ballooning, whereas coronary angiogram revealed mildly ectatic coronaries. At 2 weeks AV block persisted, needing permanent pacemaker implantation...
December 21, 2016: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/27989053/conventional-and-novel-predictors-of-permanent-pacemaker-after-tavi-in-atrial-fibrillation-patients
#15
Guilherme Portugal, Lino Patrício, Ana Abreu, Duarte Cacela, Mário Oliveira, Rui Ferreira
BACKGROUND: Atrial fibrillation (AF) is common in patients submitted for transcatheter aortic valve implantation (TAVI), and these patients are at higher risk of developing conduction system disturbances requiring pacemaker (PM) implantation. Conventional predictors for PM use in this population have limited applicability. It was hypothesized that a post-procedural heart rate reduction could gauge the degree of atrioventricular (AV) node injury, and that this would be a clinically useful predictor of a need for PM...
May 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/27987072/the-value-of-electrophysiologic-study-in-decision-making-regarding-the-need-for-pacemaker-implantation-after-tavi
#16
Oholi Tovia-Brodie, Yael Ben-Haim, Erel Joffe, Ariel Finkelstein, Aharon Glick, Raphael Rosso, Bernard Belhassen, Yoav Michowitz
PURPOSE: The purpose of this study was to evaluate electrophysiologic study (EPS) in risk stratification of relative indications for pacemaker implantation (PMI) after transcatheter aortic valve implantation (TAVI). METHODS: We reviewed files of all patients who had a left bundle branch block (LBBB) and underwent EPS after TAVI between 3/2009 and 5/2015. The indications for EPS were new-onset LBBB and the presence of an old or a new-onset LBBB associated with either PR prolongation after TAVI (∆PR >20 ms) or with "slow" atrial fibrillation (<100/min)...
December 17, 2016: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/27980762/catheter-ablation-of-ventricular-ectopy-with-para-hisian-origin-importance-of-mapping-both-sides-of-the-interventricular-septum-and-understanding-when-to-stop-ablating
#17
Antonio Madaffari, Anett Große, Santi Raffa, Markus Frommhold, Agnes Fink, J Christoph Geller
Catheter ablation of para-Hisian premature ventricular contractions (PVCs) still represents a challenge and is a compromise between success and inadvertent AV block. We describe a possible strategy to address PVCs from this location with high-amplitude His-bundle potentials at the site of earliest activation.
December 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/27979911/atrioventricular-node-dysfunction-and-ion-channel-transcriptome-in-pulmonary-hypertension
#18
Ian P Temple, Sunil Jit R J Logantha, Mais Absi, Yu Zhang, Eleftheria Pervolaraki, Joseph Yanni, Andrew Atkinson, Maria Petkova, Gillian M Quigley, Simon Castro, Mark Drinkhill, Heiko Schneider, Oliver Monfredi, Elizabeth Cartwright, Min Zi, Tomoko T Yamanushi, Vaikom S Mahadevan, Alison M Gurney, Ed White, Henggui Zhang, George Hart, Mark R Boyett, Halina Dobrzynski
BACKGROUND: Heart block is associated with pulmonary hypertension, and the aim of the study was to test the hypothesis that the heart block is the result of a change in the ion channel transcriptome of the atrioventricular (AV) node. METHODS AND RESULTS: The most commonly used animal model of pulmonary hypertension, the monocrotaline-injected rat, was used. The functional consequences of monocrotaline injection were determined by echocardiography, ECG recording, and electrophysiological experiments on the Langendorff-perfused heart and isolated AV node...
December 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27957348/exercise-stress-echocardiography-reveals-systolic-anterior-motion-of-the-mitral-valve-as-a-cause-of-syncopes-in-a-cardiac-amyloidosis-patient
#19
Tor Skibsted Clemmensen, Henning Mølgaard, Niels Frost Andersen, Steen Baerentzen, Steen Hvitfeldt Poulsen
Patients with cardiac amyloidosis are at increased AV-block and syncope risk. Therefore, a prophylactic pacemaker is often implanted. However, this case illustrates that other mechanisms should be ruled out prior to pacemaker implantation. The patient studied had mitral valve thickening without increased left ventricular outflow track (LVOT) velocity. However, bicycle exercise-stress test with simultaneous echocardiography revealed a stepwise decrease in blood pressure, a substantial increase in the LVOT velocity, and severe systolic anterior motion of the mitral valve...
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27956337/-postsurgical-morbidity-in-pediatric-patients-undergoing-surgery-for-congenital-heart-disease-in-umae-from-yucatan
#20
Addy Castillo-Espínola, Ana Velázquez-Ibarra, Aurea Zetina-Solórzano, Patricia Bolado-García, Gonzalo Gamboa-López
OBJECTIVE: To describe the clinical course of pediatric patients undergoing surgery for congenital heart disease in UMAE from Yucatan. METHODS: Descriptive survey records of pediatric patients congenital heart undergoing surgery for congenital heart disease from November, 1st 2011 to November, 30st 2013. RESULTS: The most frequent heart diseases were the persistence ductus arteriosus (37.6%) and transposition of great vessels. The intensive care stay was 3days (median)...
December 9, 2016: Archivos de Cardiología de México
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