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https://www.readbyqxmd.com/read/28431057/quantitative-analysis-of-pkp2-and-neighbouring-genes-in-a-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-caused-by-heterozygous-pkp2-deletion
#1
Keiko Sonoda, Seiko Ohno, Sou Otuki, Koichi Kato, Nobue Yagihara, Hiroshi Watanabe, Takeru Makiyama, Tohru Minamino, Minoru Horie
Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease mainly caused by desmosome gene mutations. The genetic culprit, however, remains elusive in ∼50% of ARVC patients. One of the reasons for missing genetic abnormalities is the difficulty in detecting large deletions/duplications, which are called as copy number variation (CNV) by the Sanger sequencing method. This study aimed to identify CNVs in PKP2 and a part of other desmosome genes in ARVC patients. Methods and Results: The study cohort consisted of 71 ARVC probands who were diagnosed as definite or borderline cases based on 2010 Task Force Criteria...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28430892/anti-arrhythmic-potential-of-the-late-sodium-current-inhibitor-gs-458967-in-murine-scn5a-1798insd-and-human-scn5a-1795insd-ipsc-derived-cardiomyocytes
#2
Vincent Portero, Simona Casini, Maaike Hoekstra, Arie O Verkerk, Isabella Mengarelli, Luiz Belardinelli, Sridharan Rajamani, Arthur A M Wilde, Connie R Bezzina, Marieke W Veldkamp, Carol Ann Remme
AIM: Selective inhibition of cardiac late sodium current (INaL) is an emerging target in the treatment of ventricular arrhythmias. We investigated the electrophysiological effects of GS-458967 (GS967), a potent, selective inhibitor of INaL, in an overlap syndrome model of both gain and loss of sodium channel function, comprising cardiomyocytes derived from both human SCN5A-1795insD+/- induced pluripotent stem cells (hiPSC-CMs) and mice carrying the homologous mutation Scn5a-1798insD+/-...
April 18, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28428622/inhibition-of-late-sodium-current-suppresses-calcium-related-ventricular-arrhythmias-by-reducing-the-phosphorylation-of-camk-ii-and-sodium-channel-expressions
#3
Xiao-Hong Wei, Shan-Dong Yu, Lu Ren, Si-Hui Huang, Qiao-Mei Yang, Ping Wang, Yan-Peng Chu, Wei Yang, Yan-Sheng Ding, Yong Huo, Lin Wu
Cardiac arrhythmias associated with intracellular calcium inhomeostasis are refractory to antiarrhythmic therapy. We hypothesized that late sodium current (I Na) contributed to the calcium-related arrhythmias. Monophasic action potential duration at 90% completion of repolarization (MAPD90) was significantly increased and ventricular arrhythmias were observed in hearts with increased intracellular calcium concentration ([Ca(2+)]i) by using Bay K 8644, and the increase became greater in hearts treated with a combination of ATX-II and Bay K 8644 compared to Bay K 8644 alone...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28425222/class-iii-antiarrhythmic-drugs-amiodarone-and-dronedarone-impair-kir-2-1-backward-trafficking
#4
Yuan Ji, Hiroki Takanari, Muge Qile, Lukas Nalos, Marien J C Houtman, Fee L Romunde, Raimond Heukers, Paul M P van Bergen En Henegouwen, Marc A Vos, Marcel A G van der Heyden
Drug-induced ion channel trafficking disturbance can cause cardiac arrhythmias. The subcellular level at which drugs interfere in trafficking pathways is largely unknown. KIR 2.1 inward rectifier channels, largely responsible for the cardiac inward rectifier current (IK1 ), are degraded in lysosomes. Amiodarone and dronedarone are class III antiarrhythmics. Chronic use of amiodarone, and to a lesser extent dronedarone, causes serious adverse effects to several organs and tissue types, including the heart. Both drugs have been described to interfere in the late-endosome/lysosome system...
April 19, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28412022/acute-kidney-injury-is-associated-with-microvascular-myocardial-damage-following-myocardial-infarction
#5
Sebastian Johannes Reinstadler, Andreas Kronbichler, Martin Reindl, Hans-Josef Feistritzer, Veronika Innerhofer, Agnes Mayr, Gert Klug, Martin Tiefenthaler, Gert Mayer, Bernhard Metzler
Acute kidney injury (AKI) is a frequent complication in patients with ST-elevation myocardial infarction (STEMI) treated by primary percutaneous coronary intervention. However, the pathophysiology of AKI in this setting is complex and goes beyond the administration of contrast media. Studies assessing the impact of infarct characteristics on AKI are currently lacking. Therefore, we investigated the association of AKI with myocardial as well as microvascular injury in an initial total of 361 consecutive STEMI patients treated by primary percutaneous coronary intervention...
April 12, 2017: Kidney International
https://www.readbyqxmd.com/read/28408648/azithromycin-causes-a-novel-proarrhythmic-syndrome
#6
Zhenjiang Yang, Joseph K Prinsen, Kevin R Bersell, Wangzhen Shen, Liudmila Yermalitskaya, Tatiana Sidorova, Paula B Luis, Lynn Hall, Wei Zhang, Liping Du, Ginger Milne, Patrick Tucker, Alfred L George, Courtney M Campbell, Robert A Pickett, Christian M Shaffer, Nagesh Chopra, Tao Yang, Bjorn C Knollmann, Dan M Roden, Katherine T Murray
BACKGROUND: The widely used macrolide antibiotic azithromycin increases risk of cardiovascular and sudden cardiac death, although the underlying mechanisms are unclear. Case reports, including the one we document here, demonstrate that azithromycin can cause rapid, polymorphic ventricular tachycardia in the absence of QT prolongation, indicating a novel proarrhythmic syndrome. We investigated the electrophysiological effects of azithromycin in vivo and in vitro using mice, cardiomyocytes, and human ion channels heterologously expressed in human embryonic kidney (HEK 293) and Chinese hamster ovary (CHO) cells...
April 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28389016/myotonic-dystrophy-and-brugada-syndrome-a-common-pathophysiologic-pathway
#7
REVIEW
Andrés Ricardo Pérez-Riera, Adrian Baranchuk, Li Zhang, Raimundo Barbosa-Barros, Luiz Carlos de Abreu, Pedro Brugada
Type 1 myotonic dystrophy (DM1) is a hereditary neuromuscular disease affecting multiple organs in human adults. Here we report a 42-year-old man diagnosed with DM1. Having a history of progressive muscular weakness and gradual loss of visual acuity, he was referred to us by his ophthalmologist for risk assessment of undergoing cataract surgery. Cardiology workup revealed type 1 Brugada ECG pattern, positive late potentials and inducible ventricular fibrillation in an electrophysiology study. Literature review revealed that those ECG changes may be observed in DM1, suggesting that DM1 and Brugada syndrome may share a common pathophysiologic pathway...
March 14, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28371837/characterization-of-the-arrhythmogenic-substrate-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-undergoing-ventricular-tachycardia-ablation
#8
Senthil Kirubakaran, Caterina Bisceglia, John Silberbauer, Teresa Oloriz, Giulia Santagostino, Miki Yamase, Giuseppe Maccabelli, Nicola Trevisi, Paolo Della Bella
Aims: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is associated with ventricular arrhythmias, even without RV structural disease. We aimed to characterize the RV substrate using electroanatomical mapping and to define outcomes following ventricular tachycardia (VT) ablation in patients with and without RV structural abnormalities. Methods and results: Twenty-nine patients with definite or suspected ARVC undergoing VT ablation were classified as 'electrical' and 'structural' cardiomyopathy based on the absence or presence of major structural criteria...
March 20, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28369767/multiple-targets-for-flecainide-action-implications-for-cardiac-arrhythmogenesis
#9
REVIEW
Samantha C Salvage, Karthik H Chandrasekharan, Kamalan Jeevaratnam, Angela F Dulhunty, Andrew J Thompson, Antony P Jackson, Christopher L-H Huang
Flecainide suppresses cardiac tachyarrhythmias including paroxysmal atrial fibrillation, supraventricular tachycardia, arrhythmic long QT syndromes (LQTS), as well as the Ca(2+) -mediated, catecholaminergic polymorphic ventricular, tachycardia (CPVT). However, flecainide can also exert pro-arrhythmic effects most notably following myocardial infarction and when used to diagnose Brugada Syndrome (BrS). These divergent actions result from its physiological and pharmacological actions at multiple, interacting, levels of cellular organisation...
April 3, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28364267/utility-of-12-lead-and-signal-averaged-holter-electrocardiograms-after-pilsicainide-provocation-for-risk-stratification-in-brugada-syndrome
#10
Jun Kakihara, Masahiko Takagi, Yusuke Hayashi, Hiroaki Tatsumi, Atsushi Doi, Minoru Yoshiyama
Non-invasive risk stratification for ventricular fibrillation (VF) in Brugada syndrome (BrS) has not been fully evaluated. The aim of this study was to assess the utility of signal-averaged Holter electrocardiogram (Holter SAECG) and 12-lead Holter electrocardiogram (Holter ECG) after a pilsicainide provocation test for non-invasive risk stratification in BrS. We enrolled 30 consecutive patients with BrS [divided into 2 groups: the VF group, those with a previous history of VF (n = 10); and the non-VF group, those without a history of VF (n = 20)] and 10 control subjects without type 1 ECG...
March 31, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28352365/heart-failure-modulates-electropharmacological-characteristics-of-sinoatrial-nodes
#11
Shih-Lin Chang, Hui-Lun Chuang, Yao-Chang Chen, Yu-Hsun Kao, Yung-Kuo Lin, Yung-Hsin Yeh, Shih-Ann Chen, Yi-Jen Chen
The impact of heart failure (HF) on sinoatrial node (SAN) channel regulation and electropharmacological responses has remained elusive. The present study aimed to investigate the effects of HF on the electrical activity of SANs with and without pharmacological interventions. Action potentials (APs) were recorded in isolated SANs from normal rabbits (control) and those with HF (rapid ventricular pacing for 4 weeks) prior to and after administration of a funny current blocker (ivabradine; 0.1, 0.3, 3 or 10 µM), a calmodulin kinase II inhibitor (KN-93; 0...
February 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28329265/the-hemi-mustard-bidirectional-glenn-and-rastelli-procedures-for-anatomical-repair-of-congenitally-corrected-transposition-of-the-great-arteries-left-ventricular-outflow-tract-obstruction-with-positional-heart-anomalies%C3%A2
#12
Sen Zhang, Kai Ma, Shoujun Li, Zhongdong Hua, Hao Zhang, Jun Yan, Keming Yang, Kunjing Pang, Xu Wang, Lei Qi, Qiuming Chen
OBJECTIVES: The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies...
February 27, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28314848/effects-of-late-sodium-current-blockade-on-ventricular-refibrillation-in-a-rabbit-model
#13
Mohammed Ali Azam, Nima Zamiri, Stéphane Massé, Marjan Kusha, Patrick F H Lai, Govind K Nair, Nigel S Tan, Christopher Labos, Kumaraswamy Nanthakumar
BACKGROUND: After defibrillation of initial ventricular fibrillation (VF), it is crucial to prevent refibrillation to ensure successful resuscitation outcomes. Inability of the late Na(+) current to inactivate leads to intracellular Ca(2+) dysregulation and arrhythmias. Our aim was to determine the effects of ranolazine and GS-967, inhibitors of the late Na(+) current, on ventricular refibrillation. METHODS AND RESULTS: Long-duration VF was induced electrically in Langendorff-perfused rabbit hearts (n=22) and terminated with a defibrillator after 6 minutes...
March 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28299606/three-dimensional-speckle-tracking-longitudinal-strain-is-related-to-myocardial-fibrosis-determined-by-late-gadolinium-enhancement
#14
Marco Spartera, Anna Damascelli, Ferenc Mozes, Francesco De Cobelli, Giovanni La Canna
Three-dimensional (3D) speckle tracking echocardiography (STE) is a reliable clinical tool for accurate measurements of left ventricular (LV) volumes and ejection fraction (EF). In this prospective study, we sought to identify an association between 3DSTE longitudinal strain abnormalities and areas of late gadolinium enhancement (LGE). In 50 patients (52 ± 18.5 years old) referred to our hospital for clinically indicated CMR, 3D full-volume trans-thoracic acquisitions on apical views were performed within 1 h of CMR, in order to obtain LV volumes and ejection fraction as well as global and segmental peak systolic longitudinal strain...
March 15, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28267553/left-and-right-atrial-feature-tracking-in-acute-myocarditis-a-feasibility-study
#15
Anastasia Dick, Björn Schmidt, Guido Michels, Alexander C Bunck, David Maintz, Bettina Baeßler
PURPOSE: The present study aims at evaluating the feasibility and reproducibility of cardiac magnetic resonance (CMR) feature tracking (FT) derived strain and strain rate (SR) parameters of the left and right atrium (LA, RA) in patients with acute myocarditis as well as their potential to detect diastolic dysfunction. In addition, the diagnostic value of LA and RA strain parameters in the setting of acute myocarditis is investigated. METHODS: CMR cine data of 30 patients with CMR-positive acute myocarditis were retrospectively analyzed...
April 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28254126/economic-value-and-cost-effectiveness-of%C3%A2-cardiac-resynchronization-therapy-among-patients-with-mild-heart-failure-projections-from-the-reverse-long-term-follow-up
#16
Michael R Gold, Amie Padhiar, Stuart Mealing, Manpreet K Sidhu, Stelios I Tsintzos, William T Abraham
OBJECTIVES: This study investigated the cost effectiveness of early cardiac resynchronization therapy (CRT) implantation among patients with mild heart failure (HF). The differential cost effectiveness between CRT using a defibrillator (CRT-Ds) and CRT using a pacemaker (CRT-P) was also assessed. BACKGROUND: Cardiac resynchronization has been shown to be cost effective in New York Heart Association (NYHA) functional classes III/IV but is less studied in class II HF...
March 2017: JACC. Heart Failure
https://www.readbyqxmd.com/read/28242607/invasive-assessment-of-the-coronary-microcirculation-in-reperfused-st-segment-elevation-myocardial-infarction-patients-where-do-we-stand
#17
Heerajnarain Bulluck, Nicolas Foin, Jack W Tan, Adrian F Low, Murat Sezer, Derek J Hausenloy
For patients presenting with an acute ST-segment-elevation myocardial infarction, the most effective therapy for reducing myocardial infarct size and preserving left ventricular systolic function is primary percutaneous coronary intervention (PPCI). However, mortality and morbidity remain significant. This is partly attributed to the development of microvascular obstruction, which occurs in around 50% of ST-segment-elevation myocardial infarction patients post-PPCI, and it is associated with adverse left ventricular remodeling and worse clinical outcomes...
March 2017: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28242588/effects-of-successful-percutaneous-coronary-intervention-of-chronic-total-occlusions-on-myocardial-perfusion-and-left-ventricular-function
#18
Wijnand J Stuijfzand, P Stefan Biesbroek, Pieter G Raijmakers, Roel S Driessen, Stefan P Schumacher, Pepijn van Diemen, Jeffrey van den Berg, Robin Nijveldt, Adriaan A Lammertsma, Simon J Walsh, Colm G Hanratty, James C Spratt, Albert C van Rossum, Alexander Nap, Niels van Royen, Paul Knaapen
AIMS: The aim of the present study was to investigate the effects of successful PCI CTO on absolute myocardial blood flow (MBF) and functional recovery. METHODS AND RESULTS: Patients with a documented CTO were prospectively examined for ischemia and viability with H215O positron emission tomography (PET) and late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR). Sixty-nine consecutive patients, in whom PCI was successful, underwent follow-up PET and CMR after approximately 12 weeks to evaluate potential improvement of MBF as well as systolic function...
February 28, 2017: EuroIntervention
https://www.readbyqxmd.com/read/28238244/nt-probnp-as-a-potential-marker-of-left-atrial-dysfunction-in-rheumatic-mitral-stenosis-correlation-with-left-atrial-function-after-pbmv
#19
Rashi Khare, Sudhanshu Dwivedi
BACKGROUND: Rheumatic heart disease is a common reason for cardiovascular morbidity and mortality. Plasma levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are known to rise in patients with MS, but the role of this compound as a non-invasive marker of left atrial (LA) dysfunction is yet to be established. The study aim was to correlate levels of NT-proBNP in patients with rheumatic mitral stenosis (MS), with echocardiographic parameters of LA function including tissue Doppler-derived strain/strain rate (S/Sr) indices, hemodynamic parameters of LA function, before and after percutaneous balloon mitral valvotomy (PBMV) for a follow up of one year...
September 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28231026/late-administration-of-a-palladium-lipoic-acid-complex-poly-mva-modifies-cardiac-mitochondria-but-not-functional-or-structural-manifestations-of-radiation-induced-heart-disease-in-a-rat-model
#20
Vijayalakshmi Sridharan, John W Seawright, Francis J Antonawich, Merrill Garnett, Maohua Cao, Preeti Singh, Marjan Boerma
Exposure of the heart to ionizing radiation can cause adverse myocardial remodeling. In small animal models, local heart irradiation causes persistent alterations in cardiac mitochondrial function and swelling. POLY-MVA is a dietary supplement that contains a palladium lipoic acid complex that targets mitochondrial complex I and has been demonstrated to have greater redox potential than lipoic acid alone. POLY-MVA improves mitochondrial function and anti-oxidant enzyme activity in the aged rat heart. In this study, we tested whether POLY-MVA can mitigate cardiac effects of ionizing radiation...
March 2017: Radiation Research
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