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https://www.readbyqxmd.com/read/29352276/arrhythmogenic-drugs-can-amplify-spatial-heterogeneities-in-the-electrical-restitution-in-perfused-guinea-pig-heart-an-evidence-from-assessments-of-monophasic-action-potential-durations-and-jt-intervals
#1
Oleg E Osadchii
Non-uniform shortening of the action potential duration (APD90) in different myocardial regions upon heart rate acceleration can set abnormal repolarization gradients and promote arrhythmia. This study examined whether spatial heterogeneities in APD90 restitution can be amplified by drugs with clinically proved proarrhythmic potential (dofetilide, quinidine, procainamide, and flecainide) and, if so, whether these effects can translate to the appropriate changes of the ECG metrics of ventricular repolarization, such as JT intervals...
2018: PloS One
https://www.readbyqxmd.com/read/29319343/obesity-and-sudden-cardiac-death-in-the-young-clinical-and-pathological-insights-from-a-large-national-registry
#2
Gherardo Finocchiaro, Michael Papadakis, Harshil Dhutia, Della Cole, Elijah R Behr, Maite Tome, Sanjay Sharma, Mary N Sheppard
Aims Obesity is an increasing public health problem and a risk factor for cardiovascular diseases. The aim of the study was to determine the main features and aetiologies in a large cohort of sudden cardiac deaths that occurred in obese subjects. Methods Between 1994 and 2014, 3684 consecutive cases of unexpected sudden cardiac death were referred to our cardiac pathology centre. This study was confined to young individuals (age ≤ 35 years) for whom information about body mass index was available and consisted of 1033 cases...
January 1, 2018: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/29288195/sequencing-of-linkage-region-on-chromosome-12p11-identifies-pkp2-as-a-candidate-gene-for-left-ventricular-mass-in-dominican-families
#3
Nicole D Dueker, Shengru Guo, Ashley Beecham, Liyong Wang, Susan H Blanton, Marco R Di Tullio, Tatjana Rundek, Ralph L Sacco
Increased left ventricular mass (LVM) is an intermediate phenotype for cardiovascular disease (CVD) and a predictor of stroke. Using families from the Dominican Republic, we have previously shown LVM to be heritable and found evidence for linkage to chromosome 12p11. Our current study aimed to further characterize the QTL by sequencing the 1 LOD unit down region in ten families from the Dominican Republic with evidence for linkage to LVM. Within this region, we tested 5,477 common variants (CVs; minor allele frequency [MAF] ≥5%) using the QTDT test...
December 29, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/29284304/altered-long-non-coding-rna-transcriptomic-profiles-in-ischemic-stroke
#4
Wenzhen He, Duncan Wei, De Cai, Siqia Chen, Shunxian Li, Wenjie Chen
BACKGROUND: Our previous study described the important regulatory roles of microRNAs (miRNAs) in ischemic stroke. However, the functional significance of long non coding RNA (lncRNAs) in ischemic stroke was largely unknown. This study was aimed to identify the lncRNA profiling and elucidate the regulatory mechanisms in pathophysiology of stroke. MATERIALS AND METHODS: Herein, we used RNA sequencing to sequence the RNA from blood of three ischemic stroke patients and three normal controls...
December 28, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/29282897/uniaxial-strain-of-cultured-mouse-and-rat-cardiomyocyte-strands-slows-conduction-more-when-its-axis-is-parallel-to-impulse-propagation-than-when-it-is-perpendicular
#5
Andrea Buccarello, Michela Azzarito, Frédéric Michoud, Stéphanie P Lacour, Jan P Kucera
AIM: Cardiac tissue deformation can modify tissue resistance, membrane capacitance and ion currents, and hence cause arrhythmogenic slow conduction. Our aim was to investigate whether uniaxial strain causes different changes in conduction velocity (θ) when the principal strain axis is parallel vs. perpendicular to impulse propagation. METHODS: Cardiomyocyte strands were cultured on stretchable custom microelectrode arrays and θ was determined during steady-state pacing...
December 28, 2017: Acta Physiologica
https://www.readbyqxmd.com/read/29250293/hypothetical-anatomy-of-brugada-phenomenon-long-qt-sine-long-qt-syndrome-implicating-morphologically-undefined-specific-brugada-s-myocells
#6
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/29245138/cardiac-sarcoidosis-presenting-as-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-with-ventricular-aneurysms-a-case-report
#7
Marija Petrovic, L Maximilian Buja, Biswajit Kar, Jure Colnaric, Sarita Damaraju, Bihong Zhao, Bindu Akkanti, Milan Radovanovic, Rajko Radovancevic, Pranav Loyalka, Igor D Gregoric
A 70-year old Caucasian man with recurrent ventricular tachycardia and progressive biventricular failure attributed to arrhythmogenic right ventricular cardiomyopathy/dysplasia was evaluated for heart transplantation. Cardiac ventriculography revealed an abnormal left ventricle with five saccular aneurysms. Heart transplantation was performed. Pathology of the explanted heart showed multifocal sarcoid granulomas. Replacement fibrosis was widespread in both ventricles and associated with saccular aneurysms. No genetic basis was identified...
November 10, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/29225258/isolated-cardiac-sarcoidosis-mimicking-arrhythmogenic-right-ventricular-cardiomyopathy
#8
Hirotaka Waki, Kazuo Eguchi, Shinichi Toriumi, Tomokazu Ikemoto, Tsukasa Suzuki, Noriyoshi Fukushima, Kazuomi Kario
The diagnosis of cardiac sarcoidosis (CS) has become easier due to advances in imaging modalities, but we sometimes encounter difficult-to-diagnose patients. We herein report the case of a 60-year-old Japanese woman who was diagnosed with isolated CS, although she also met the diagnostic criteria of arrhythmogenic right ventricular cardiomyopathy (ARVC). A histological examination by an endomyocardial biopsy of the right ventricle revealed the typical findings of granulomatous change for CS. Although she did not show any characteristics of systemic sarcoidosis, oral prednisolone treatment was introduced, and she achieved a good response...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29212896/the-novel-desmin-mutation-p-glu401asp-impairs-filament-formation-disrupts-cell-membrane-integrity-and-causes-severe-arrhythmogenic-left-ventricular-cardiomyopathy-dysplasia
#9
Francisco José Bermúdez-Jiménez, Víctor Carriel, Andreas Brodehl, Miguel Alaminos, Antonio Campos, Ilona Schirmer, Hendrik Milting, Beatriz Álvarez Abril, Miguel Álvarez, Silvia López-Fernández, Diego García-Giustiniani, Lorenzo Monserrat, Luis Tercedor, Juan Jiménez-Jáimez
Background -Desmin (DES) mutations cause severe skeletal and cardiac muscle disease with heterogeneous phenotypes. Recently, DES mutations were described in patients with inherited arrhythmogenic right ventricular cardiomyopathy/dysplasia (iARVC/D), although their cellular and molecular pathomechanisms are not precisely known. Our aim is to describe clinically and functionally the novel DES-p.Glu401Asp mutation as a cause of inherited left ventricular arrhythmogenic cardiomyopathy/dysplasia (iLVAC/D). Methods -We identified the novel DES mutation p...
December 6, 2017: Circulation
https://www.readbyqxmd.com/read/29207943/auxiliary-diagnostic-potential-of-ventricle-geometry-and-late-gadolinium-enhancement-in-left-ventricular-non-compaction-non-randomized-case-control-study
#10
Marko Boban, Vladimir Pesa, Ivo Darko Gabric, Sime Manola, Viktor Persic, Helena Antic-Kauzlaric, Marinko Zulj, Aleksandar Vcev
BACKGROUND: There are still ambiguities existing in regard to left ventricular non-compaction (LVNC) diagnostic imaging. The aim of our study was to analyze diagnostic potential of late gadolinium enhancement (LGE) and ventricle geometry in patients with LVNC and controls. METHODS: Data on cardiac magnetic resonance imaging (CMR) studies for LVNC were reassessed from the hospital's database (3.75 years; n=1975 exams). Matching sample of controls included cases with no structural heart disease, hypertrophic or dilative cardiomyopathy, arrhythmogenic right ventricular dysplasia or subacute myocarditis...
December 6, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29194452/hyperthyroidism-but-not-hypertension-impairs-pitx2-expression-leading-to-wnt-microrna-ion-channel-remodeling
#11
Estefanía Lozano-Velasco, Rosemary Wangensteen, Andrés Quesada, Carlos Garcia-Padilla, Julia A Osorio, María Dolores Ruiz-Torres, Amelia Aranega, Diego Franco
PITX2 is a homeobox transcription factor involved in embryonic left/right signaling and more recently has been associated to cardiac arrhythmias. Genome wide association studies have pinpointed PITX2 as a major player underlying atrial fibrillation (AF). We have previously described that PITX2 expression is impaired in AF patients. Furthermore, distinct studies demonstrate that Pitx2 insufficiency leads to complex gene regulatory network remodeling, i.e. Wnt>microRNAs, leading to ion channel impairment and thus to arrhythmogenic events in mice...
2017: PloS One
https://www.readbyqxmd.com/read/29191297/verification-of-heart-disease-implications-for-a-new-heart-transplantation-allocation-system
#12
Pejman Raeisi-Giglou, E Rene Rodriguez, Eugene H Blackstone, Carmela D Tan, Eileen M Hsich
OBJECTIVES: This study sought to determine the accuracy of the pre-transplantation clinical diagnosis of heart disease in the United Network for Organ Sharing (UNOS) database. BACKGROUND: Because survival on the heart transplantation waitlist depends on underlying heart disease, a new allocation system will include the type of heart disease. Accuracy of the pre-transplantation clinical diagnosis and the effect of misclassification are unknown. METHODS: We included all adults who received transplants at our center between January 2009 to December 2015...
December 2017: JACC. Heart Failure
https://www.readbyqxmd.com/read/29178656/unique-genetic-background-and-outcome-of-non-caucasian-japanese-probands-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#13
Yuko Wada, Seiko Ohno, Takeshi Aiba, Minoru Horie
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy mainly caused by desmosomal gene mutation. More than half of Caucasian probands have desmosomal mutations, which lead to earlier onset of ventricular arrhythmias. Among non-Caucasians, the genetic background of ARVD/C probands and its prognostic impact remain unclear. METHODS AND RESULTS: We genotyped 99 unrelated Japanese ARVD/C probands for plakophilin 2 (PKP2), desmoglein 2 (DSG2), desmoplakin (DSP), and desmocollin 2 (DSC2) between 2005 and 2014...
November 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29177016/-caterpillar-right-ventricle-unusual-manifestation-of-a-rare-disease
#14
Suneesh Kalliath, Gopalan Nair Rajesh
A 66-year-old man presented with abdominal distension and pedal oedema for the past 10 years. He had history of right heart failure on several occasions in the past, and one of these admissions prompted referral to a cardiac specialist. On examination, he had markedly elevated jugular venous pressure with prominent 'y-descent', a tricuspid regurgitation murmur, gross ascites and pedal oedema. A full blood count, routine biochemical screen and serum bicarbonate levels were normal. Right ventricular angiogram revealed a 'caterpillar'-like aneurysm of the right ventricle (RV) apex (figure 1 arrow) with a dilated right ventricular outflow tract and significant tricuspid regurgitation with a dilated right atrium (see online supplementary video 1)...
2017: Heart Asia
https://www.readbyqxmd.com/read/29173404/sudden-cardiac-death-in-genetic-cardiomyopathies
#15
REVIEW
Gourg Atteya, Rachel Lampert
Sudden cardiac death (SCD) caused by ventricular arrhythmias is common in patients with genetic cardiomyopathies (CMs) including dilated CM, hypertrophic CM, and arrhythmogenic right ventricular CM (ARVC). Phenotypic features can identify individuals at high enough risk to warrant placement of an implantable cardioverter-defibrillator, although risk stratification schemes remain imperfect. Genetic testing is valuable for family cascade screening but with few exceptions (eg, LMNA mutations) do not identify higher risk for SCD...
December 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/29158215/risk-stratification-in-arrhythmogenic-right-ventricular-cardiomyopathy
#16
REVIEW
Hugh Calkins, Domenico Corrado, Frank Marcus
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease...
November 21, 2017: Circulation
https://www.readbyqxmd.com/read/29154691/optimal-screening-methods-to-detect-cardiac-disorders-in-athletes-an-evidence-based-review
#17
Zachary K Winkelmann, Ashley K Crossway
Reference/Citation:  Harmon KG, Zigman M, Drezner JA. The effectiveness of screening history, physical exam, and ECG to detect potentially lethal cardiac disorders in athletes: a systematic review/meta-analysis. J Electrocardiol. 2015;48(3):329-338. CLINICAL QUESTION:   Which screening method should be considered best practice to detect potentially lethal cardiac disorders during the preparticipation physical examination (PE) of athletes? DATA SOURCES:   The authors completed a comprehensive literature search of MEDLINE, CINAHL, Cochrane Library, Embase, Physiotherapy Evidence Database (PEDro), and SPORTDiscus from January 1996 to November 2014...
November 20, 2017: Journal of Athletic Training
https://www.readbyqxmd.com/read/29141175/sudden-cardiac-arrest-during-participation-in-competitive-sports
#18
Cameron H Landry, Katherine S Allan, Kim A Connelly, Kris Cunningham, Laurie J Morrison, Paul Dorian
BACKGROUND: The incidence of sudden cardiac arrest during participation in sports activities remains unknown. Preparticipation screening programs aimed at preventing sudden cardiac arrest during sports activities are thought to be able to identify at-risk athletes; however, the efficacy of these programs remains controversial. We sought to identify all sudden cardiac arrests that occurred during participation in sports activities within a specific region of Canada and to determine their causes...
November 16, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29107359/heart-transplantation-in-arrhythmogenic-right-ventricular-cardiomyopathy-experience-from-the-nordic-arvc-registry
#19
Thomas Gilljam, Kristina H Haugaa, Henrik K Jensen, Anneli Svensson, Henning Bundgaard, Jim Hansen, Göran Dellgren, Finn Gustafsson, Hans Eiskjær, Arne K Andreassen, Johan Sjögren, Thor Edvardsen, Anders G Holst, Jesper Hastrup Svendsen, Pyotr G Platonov
OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. METHODS: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry...
October 21, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29105955/right-ventricular-systolic-function-and-mechanical-dispersion-identify-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#20
Meriam Åström Aneq, Eva Maret, Lars Brudin, Anneli Svensson, Jan Engvall
PURPOSE: To assess right ventricular (RV) regional and global systolic function using feature tracking (FT) in patients with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) and to investigate if changes in strain amplitude and mechanical dispersion indicate a propensity for arrhythmia. MATERIALS AND METHODS: Twenty-seven patients fulfilling Task Force Criteria for ARVC and 24 healthy volunteers underwent MR at 1·5 Tesla. Steady-state free precession cine of long-axis slices and a short-axis stack of the RV was acquired...
November 6, 2017: Clinical Physiology and Functional Imaging
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