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https://www.readbyqxmd.com/read/28917552/crispr-correction-of-the-prkag2-gene-mutation-in-the-patient-s-ipsc-derived-cardiomyocytes-eliminates-the-electrophysiological-and-structural-abnormalities
#1
Ronen Ben Jehuda, Binyamin Eisen, Yuval Shemer, Lucy N Mekies, Agnes Szantai, Irina Reiter, Huanhuan Cui, Kaomei Guan, Shiraz Haron-Khun, Dov Freimark, Silke R Sperling, Mihaela Gherghiceanu, Michael Arad, Ofer Binah
BACKGROUND: Mutations in the PRKAG2 gene encoding the γ-subunit of adenosine monophosphate-kinase (AMPK) cause hypertrophic cardiomyopathy (HCM) and familial-Wolff-Parkinson-White syndrome (WPW). Patients carrying the R302Q mutation in PRKAG2 present sinus bradycardia, escape rhythms, ventricular pre-excitation, supraventricular tachycardia and atrioventricular block. This mutation affects AMPK activity and increases glycogen storage in cardiomyocytes. The link between glycogen storage, WPW, HCM and arrhythmias remains unknown...
September 13, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28916226/-accelerated-atrioventricular-junctional-rhythm
#2
Juan Ruiz-García, Luis Borrego-Bernabé, Irene Canal-Fontcuberta, Eduardo Alegría-Barrero
No abstract text is available yet for this article.
September 12, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28912205/epicardial-breakthrough-waves-during-sinus-rhythm-depiction-of-the-arrhythmogenic-substrate
#3
Elisabeth M J P Mouws, Eva A H Lanters, Christophe P Teuwen, Lisette J M E van der Does, Charles Kik, Paul Knops, Jos A Bekkers, Ad J J C Bogers, Natasja M S de Groot
BACKGROUND: Epicardial breakthrough waves (EBW) during atrial fibrillation are important elements of the arrhythmogenic substrate and result from endo-epicardial asynchrony, which also occurs to some degree during sinus rhythm (SR). We examined the incidence and characteristics of EBW during SR and its possible value in the detection of the arrhythmogenic substrate associated with atrial fibrillation. METHODS AND RESULTS: Intraoperative epicardial mapping (interelectrode distances 2 mm) of the right atrium, Bachmann's bundle, the left atrioventricular groove, and the pulmonary vein area was performed during SR in 381 patients (289 male, 67±10 years) with ischemic or valvular heart disease...
September 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28906536/endomyocardial-fibrosis-in-sudan-clinical-and-echocardiographic-features
#4
Siddiq Ibrahim Khalil, Suha Khalil, Salma El Tigani, Hanan A Saad
OBJECTIVE: Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan. METHODS: The study started in January 2007 and is on-going. All the patients attending our echocardiography clinics in four different hospitals in Khartoum, Sudan, were included...
July 2017: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/28904525/knowing-about-device-algorithms-to-understand-the-rhythm-and-role-of-managed-ventricular-pacing
#5
Antoine Kossaify, Hiba Hamdar
A 66-year-old patient with a DR pacemaker for intermittent atrioventricular block presented with an electrocardiogram (ECG) showing some P waves non followed by QRS complexes, raising suspicion of device dysfunction. The device was equipped with a special algorithm (Managed Ventricular Pacing; Medtronic), and the observed ECG tracing was a normal consequence of the function of such algorithms. Being aware of the function of specific algorithms is essential to adequately analyze rhythms.
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/28904256/current-surgical-outcomes-of-congenital-heart-surgery-for-patients-with-down-syndrome-in-japan
#6
Takaya Hoashi, Norimichi Hirahara, Arata Murakami, Yasutaka Hirata, Hajime Ichikawa, Junjiro Kobayashi, Shinichi Takamoto
BACKGROUND: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28901234/risk-factors-for-unfavorable-outcomes-after-bidirectional-cavopulmonary-anastomosis
#7
Nataliya R Nichay, Yuriy N Gorbatykh, Igor A Kornilov, Ilya A Soynov, Yuriy Y Kulyabin, Artem V Gorbatykh, Sergey M Ivantsov, Alexander V Bogachev-Prokophiev, Alexander M Karaskov
BACKGROUND: Bidirectional cavopulmonary anastomosis (BCPA) is an important preliminary step toward the Fontan procedure; thus, understanding of risk factors for morbidity and mortality after BCPA may ultimately promote improved rates of success with Fontan completion and general survival. This study evaluated survival and predictors of unfavorable outcomes in patients after BCPA. METHODS: Clinical data of 157 patients who underwent BCPA from 2003 to 2015 at a single center were retrospectively analyzed...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28899955/natural-history-and-clinical-predictors-of-atrial-tachycardia-in-adults-with-congenital-heart-disease
#8
Pablo Ávila, José María Oliver, Pastora Gallego, Ana González-García, María José Rodríguez-Puras, Esther Cambronero, José Ruiz-Cantador, Ana Campos, Rafael Peinado, Raquel Prieto, Fernando Sarnago, Raquel Yotti, Francisco Fernández-Avilés
BACKGROUND: Atrial tachycardias (ATs) are a significant source of morbidity in adults with congenital heart disease (CHD). This study evaluates the incidence and clinical predictors of AT in a cohort of patients with CHD. METHODS AND RESULTS: We included 3311 adults (median age at entry 22.6 years, 50.6% males) with CHD (49% simple, 39% moderate, and 12% complex) prospectively followed up in a tertiary center for 37 607 person-years. Predictors of AT were identified by multivariable Cox regression analysis accounting for left truncation...
September 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28899884/supraventricular-tachycardia-diagnosed-by-smartphone-ecg
#9
Ariana Tabing, Travis Edward Harrell, Steven Romero, Gregory Francisco
Diagnosis of paroxysmal supraventricular tachycardia (PSVT) may be difficult due to its episodic nature, which can be brief and self-limited, limiting the ability for clinicians to diagnose the specific rhythm disorder in a timely manner. We present a case of PSVT, which was unable to be diagnosed through typical evaluation with an event monitor despite several years of symptoms. The patient was ultimately diagnosed using the AliveCor Mobile ECG, a smartphone-based ECG device and application, which he purchased himself and captured a typical atrioventricular node re-entrant tachycardia...
September 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28899346/one-symptom-two-arrhythmias-the-rare-and-the-even-rarer
#10
Florian Zauner, Jan-Thorben Sieweke, Stephan Hohmann, David Duncker, Christian Riehle, L Christian Napp, Ulrike Flierl, Thorben König, Christian Veltmann
BACKGROUND: Wolff-Parkinson-White (WPW) syndrome and idiopathic left ventricular tachycardia (ILVT) are rare and up to now the coexistence of both entities has rarely been reported. In patients with ventricular preexcitation the underlying mechanism of paroxysmal tachycardia most likely is atrioventricular reentrant tachycardia (AVRT). However, without ECG documentation of the tachycardia diagnosis of the underlying mechanism cannot be made due to similar clinical presentation of AVRT and ILVT...
September 12, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28895705/-complete-heart-block-and-asystole-in-a-child-with-ataxia-telangiectasia
#11
Daniel Palanca Arias, Ariadna Ayerza Casas, Mercedes Domínguez Cajal, Marta López Ramón, Lorenzo Jiménez Montañés
Ataxia-telangiectasia is a disorder characterized by cerebellar ataxia, telangiectasia, immunodeficiency, and increased predisposition to cancer susceptibility. Mutations in the ataxia telangiectasia mutated gene seem to play an important role in normal cell function and in cardiovascular remodeling. We report a case of a 14-year-old boy with ataxia-telangiectasia and high-grade B-non-Hodgkin lymphoma who remained in continuous complete remission after chemotherapy and who was admitted into our Emergency Room presenting with episodes of presyncope...
October 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28894151/the-effects-of-ageing-and-adrenergic-challenge-on-electrocardiographic-phenotypes-in-a-murine-model-of-long-qt-syndrome-type-3
#12
Karan R Chadda, Shiraz Ahmad, Haseeb Valli, Ingrid den Uijl, Ali Bak Al-Hadithi, Samantha C Salvage, Andrew A Grace, Christopher L-H Huang, Kamalan Jeevaratnam
Long QT Syndrome 3 (LQTS3) arises from gain-of-function Nav1.5 mutations, prolonging action potential repolarisation and electrocardiographic (ECG) QT interval, associated with increased age-dependent risk for major arrhythmic events, and paradoxical responses to β-adrenergic agents. We investigated for independent and interacting effects of age and Scn5a+/ΔKPQ genotype in anaesthetised mice modelling LQTS3 on ECG phenotypes before and following β-agonist challenge, and upon fibrotic change. Prolonged ventricular recovery was independently associated with Scn5a+/ΔKPQ and age...
September 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28893961/conduction-disturbances-after-transcatheter-aortic-valve-replacement-current-status-and-future-perspectives
#13
REVIEW
Vincent Auffret, Rishi Puri, Marina Urena, Chekrallah Chamandi, Tania Rodriguez-Gabella, François Philippon, Josep Rodés-Cabau
Transcatheter aortic valve replacement (TAVR) has become a well-accepted option for treating patients with aortic stenosis at intermediate to high or prohibitive surgical risk. TAVR-related conduction disturbances, mainly new-onset left bundle-branch block and advanced atrioventricular block requiring permanent pacemaker implantation, remain the most common complication of this procedure. Furthermore, improvements in TAVR technology, akin to the increasing experience of operators/centers, have translated to a major reduction in periprocedural complications, yet the incidence of conduction disturbances has remained relatively high, with perhaps an increasing trend over time...
September 12, 2017: Circulation
https://www.readbyqxmd.com/read/28893549/continuous-optimization-of-cardiac-resynchronization-therapy-reduces-atrial-fibrillation-in-heart-failure-patients-results-of-the-adaptive-cardiac-resynchronization-therapy-trial
#14
David Birnie, Harrison Hudnall, Bernd Lemke, Kazutaka Aonuma, Kathy Lai-Fun Lee, Maurizio Gasparini, John Gorcsan, Jeffrey Cerkvenik, David O Martin
BACKGROUND: Data from randomized trials have suggested a modest or no effect of conventional cardiac resynchronization therapy (convCRT) on the incidence of atrial fibrillation (AF). AdaptivCRT (aCRT, Medtronic, Mounds View, MN) is a recently described algorithm for synchronized left ventricular (LV) pacing and continuous optimization of cardiac resynchronization therapy (CRT). OBJECTIVE: We compared the long-term effects of aCRT with convCRT pacing on the incidence of AF...
September 8, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28893257/preoperative-single-ventricle-function-determines-early-outcome-after-second-stage-palliation-of-single-ventricle-heart
#15
Jacek Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Bogusław Mazurek, Lidia Tomkiewicz-Pająk
BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart...
September 11, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28891553/hyperthyroidism-and-sick-sinus-syndrome-a-rare-but-challenging-association-a-study-of-three-cases
#16
M Tudoran, C Tudoran
Hyperthyroidism is usually associated with sinus tachycardia or supraventricular tachyarrhythmias, but rarely with dysfunction of the sinus node or other conduction disturbances. Evidence of bradyarrhythmia in patients with hyperthyroidism is clinically relevant, but the fact that several drugs with negative chronotropic effects (beta-blockers and calcium channel antagonists) are frequently used in the management of these patients must be taken into account. In the presence of sick sinus syndrome (SSS) or other conductance disturbances, therapy with agents that delay the activity of sinus node or atrioventricular conduction can lead to extreme bradycardia with syncope...
August 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28886988/perinatal-ciclosporin-a-exposure-elicits-sex-related-cardiac-dysfunction-and-inflammation-in-the-rat-progeny
#17
Hany M El-Bassossy, Zuheir Awan, Mahmoud M El-Mas
Ciclosporin A (CSA) has been identified with harmful cardiotoxicity but little, if any, is known about the influence of perinatal exposure upon the cardiac function of the progeny. The present work examines the premise that perinatal contact with CSA undermines the cardiac function of sexually mature rats. Administration of CSA (15mg/kg/day sc) to pregnant rats from day 6 of conception till weaning led to a decrease in gradient of the end-systolic pressure-volume relationship by a factor of ten for male progeny and a factor of two for female progeny...
September 5, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28878402/genetic-mechanisms-contribute-to-the-development-of-heart-failure-in-patients-with-atrioventricular-block-and-right-ventricular-apical-pacing
#18
Nana Liu, Min Zheng, Shijie Li, Hui Bai, Zhouying Liu, Cui Hong Hou, Shu Zhang, Jielin Pu
Right ventricular apical (RVA) pacing can lead to progressive left ventricular dysfunction and heart failure (HF), even in patients with normal cardiac structure and function. Our study conducted candidate gene screening and lentivirus transfected neonatal rat cardiomyocytes (NRCMs) to explore the genetic and pathogenic mechanisms of RVA pacing induced cardiomyopathy in third degree atrioventricular block (III AVB) patients. We followed 887 III AVB patients with baseline normal cardiac function and RVA pacing...
September 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28876373/minimally-invasive-epicardial-pacemaker-implantation-in-neonates-with-congenital-heart-block
#19
Roberto Costa, Katia Regina da Silva, Martino Martinelli Filho, Roger Carrillo
BACKGROUND: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). OBJECTIVE: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. METHODS: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB...
September 4, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28875729/role-of-cardiac-pacing-in-congenital-complete-heart-block
#20
Stephanie F Chandler, Francis Fynn-Thompson, Douglas Y Mah
Congenital complete heart block affects 1/15,000 live-born infants, predominantly due to atrioventricular nodal injury from maternal antibodies of mothers with systemic lupus erythermatosus or Sjogren's syndrome. The majority of these children will need a pacemaker implanted prior to becoming young adults. This article will review the various patient and technical factors that influence the type of pacemaker implanted, and the current literature on optimal pacing practices. Areas covered: A literature search was performed using PubMed, Embase and Web of Science...
September 6, 2017: Expert Review of Cardiovascular Therapy
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