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arrhythmogenic right

Lilian Maria Lopes, Juliana Torres Pacheco, Regina Schultz, Rossana Pulcineli Vieira Francisco, Marcelo Zugaib
No abstract text is available yet for this article.
February 2018: Arquivos Brasileiros de Cardiologia
Jane E Wilcox, Ray E Hershberger
PURPOSE OF REVIEW: To describe recent advancements in cardiovascular genetics made possible by leveraging next-generation sequencing (NGS), and to provide a framework for practical applications of genetic testing for hypertrophic (HCM), dilated (DCM), and arrhythmogenic right ventricular cardiomyopathies (ARVC). RECENT FINDINGS: The availability of NGS has made possible extensive reference databases. These, combined with recent initiatives to compile previously siloed commercial and research cardiomyopathy data sets, provide a more powerful and precise approach to cardiovascular genetic medicine...
March 19, 2018: Current Opinion in Cardiology
Li Gao, Li-Jie Zhang, Sheng-Hua Li, Li-Li Wei, Bin Luo, Rong-Quan He, Shuang Xia
BACKGROUND: MiR-452-5p has been reported to be down-regulated in prostate cancer, affecting the development of this type of cancer. However, the molecular mechanism of miR-452-5p in prostate cancer remains unclear. Therefore, we investigated the network of target genes of miR-452-5p in prostate cancer using bioinformatics analyses. MATERIALS AND METHODS: We first analyzed the expression profiles and prognostic value of miR-452-5p in prostate cancer tissues from a public database...
March 6, 2018: Pathology, Research and Practice
Jonathan Lichtenberger, Kathryn M Meurs, Etienne Côté
Cardiac arrhythmias often are transient and might not be detected using conventional electrocardiographic (ECG) techniques. The adhesive patch monitor (APM) is a single-lead, lightweight, up to 14-day continuous ambulatory ECG monitor. This study aimed to prospectively assess its usability in four boxer dogs considered either to be healthy or to have arrhythmogenic right ventricular cardiomyopathy. Optimal recording was obtained by placing the APM on the left side of the animal's thorax, at the fifth intercostal space, slightly dorsal to the costochondral junction, and oriented either vertically or parallel to the long axis of the heart...
March 20, 2018: Journal of the American Animal Hospital Association
Chiara Assunta Pilato, Ilaria Stadiotti, Angela Serena Maione, Valentina Saverio, Valentina Catto, Fabrizio Tundo, Antonio Dello Russo, Claudio Tondo, Giulio Pompilio, Michela Casella, Elena Sommariva
A normal adult heart is composed of several different cell types, among which cardiac mesenchymal stromal cells represent an abundant population. The isolation of these cells offers the possibility of studying their involvement in cardiac diseases, and, in addition, provides a useful primary cell model to investigate biological mechanisms. Here, the method for the isolation of C-MSC from arrhythmogenic cardiomyopathy patients' bioptic samples is described. The endomyocardial biopsy sampling is guided in the right ventricular areas adjacent to the scar visualized by electro-anatomical mapping...
February 28, 2018: Journal of Visualized Experiments: JoVE
Thomas P Mast, Karim Taha, Maarten J Cramer, Joost Lumens, Jeroen F van der Heijden, Berto J Bouma, Maarten P van den Berg, Folkert W Asselbergs, Pieter A Doevendans, Arco J Teske
OBJECTIVES: The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols. BACKGROUND: ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC...
March 9, 2018: JACC. Cardiovascular Imaging
Maciej Kubala, Rajeev K Pathak, Shuanglun Xie, Ruben Casado Arroyo, Cory M Tschabrunn, Tatsuya Hayashi, Fermin C Garcia, Gregory E Supple, Pasquale Santangeli, David S Frankel, Erica S Zado, David J Callans, Francis E Marchlinski
BACKGROUND: Repolarization abnormalities in arrhythmogenic right ventricular (RV) cardiomyopathy and their relationship to ventricular tachycardia substrate are incompletely understood. METHODS AND RESULTS: In 40 patients (29 men, mean age 38 years) with arrhythmogenic RV cardiomyopathy, we compared the extent and location of abnormal T (NegT) waves ≥1 mm in depth (n=32) and downsloping elevated ST segment (n=13), in ≥2 adjacent leads, to area and location of endocardial bipolar (<1...
March 2018: Circulation. Arrhythmia and Electrophysiology
Adalena Tsatsopoulou
No abstract text is available yet for this article.
May 1, 2018: International Journal of Cardiology
E Gandjbakhch, E Varlet, G Duthoit, V Fressart, P Charron, C Himbert, C Maupain, C Bordet, F Hidden-Lucet, J Nizard
INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected...
May 1, 2018: International Journal of Cardiology
Jennifer Karmouch, Alexandros Protonotarios, Petros Syrris
PURPOSE OF REVIEW: To date 16 genes have been associated with arrhythmogenic cardiomyopathy (ACM). Mutations in these genes can lead to a broad spectrum of phenotypic expression ranging from disease affecting predominantly the right or left ventricle, to biventricular subtypes. Understanding the genetic causes of ACM is important in diagnosis and management of the disorder. This review summarizes recent advances in molecular genetics and discusses the application of next-generation sequencing technology in genetic testing in ACM...
March 14, 2018: Current Opinion in Cardiology
Patrícia B S Celestino-Soper, Ty C Lynnes, Lili Zhang, Karen Ouyang, Samuel Wann, Victoria L Clyde, Matteo Vatta
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder that may lead to sudden death and can affect humans and other primates. In 2012, the alpha male bonobo of the Milwaukee County Zoo died suddenly and histologic evaluation found features of ARVC. This study sought to discover a possible genetic cause for ARVC in this individual. We sequenced our subject's DNA to search for deleterious variants in genes involved in cardiovascular disorders. Variants found were annotated according to the human genome, following currently available classification used for human diseases...
March 12, 2018: Scientific Reports
Mangyuan Wang, Kai Chen, Xiao Chen, Liang Chen, Jiangping Song, ShengShou Hu
Arrhythmogenic right ventricular cardiomyopathy (ARVC) and dilated cardiomyopathy (DCM), despite being two dramatically different entities, have overlapping phenotypes. As it is easy to misdiagnose between ARVC and DCM, there is a need to establish a new differential diagnostic parameter to differentiate the two. We investigated the utility of endomyocardial biopsy (EMB) for the differential diagnosis, and our study had three aims. The first was to verify the EMB high diagnostic efficacy. The second was to investigate the EMB perforation risk at the right ventricle (RV) free wall of end-stage ARVC...
February 14, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Prabhpreet Singh, Amit Noheria
Invasive electrophysiology (EP) mapping and catheter ablation has increasingly become the standard of care for many cardiac arrhythmias like supraventricular tachycardias, atrial fibrillation, premature ventricular complexes (PVC), and monomorphic ventricular tachycardia. In this review, we discuss the recent progress made in the mapping and ablation of ventricular fibrillation (VF). Ventricular activation during VF is apparently disorganized, making mapping and interpretation difficult. Prolonged mapping during VF would require mechanical circulatory support as VF causes complete hemodynamic collapse...
March 6, 2018: Current Treatment Options in Cardiovascular Medicine
Adalena Tsatsopoulou, Eduardo Bossone
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the most common phenotype described within the spectrum of arrhythmogenic cardiomyopathies. It usually presents in early adolescence with severe ventricular arrhythmias along with cardiac structural and functional alterations mainly of the right ventricular myocardium. Though the estimated prevalence of ARVC in the general population is only 1:5000, it represents one of the most common causes of juvenile sudden death. However, detection of early RV dysfunction in ARVC may be challenging requiring high clinical suspicion and an algorithmic approach...
April 15, 2018: International Journal of Cardiology
Giuseppe Cannavale, Marco Francone, Nicola Galea, Francesco Vullo, Antonio Molisso, Iacopo Carbone, Carlo Catalano
Ectopic cardiac fatty images are not rarely detected incidentally by computed tomography and cardiac magnetic resonance, or by exams focused on the heart as in general thoracic imaging evaluations. A correct interpretation of these findings is essential in order to recognize their normal or pathological meaning, focusing on the eventually associated clinical implications. The development of techniques such as computed tomography and cardiac magnetic resonance allowed a detailed detection and evaluation of adipose tissue within the heart...
2018: BioMed Research International
Shuanglun Xie, Benoit Desjardins, Maciej Kubala, Jackson Liang, Jiandu Yang, Rob J van der Geest, Robert Schaller, Michael Riley, David Callans, Erica Zado Pac, Francis Marchlinski, Saman Nazarian
BACKGROUND: Criteria for identification of anatomic ventricular tachycardia (VT) substrates in arrhythmogenic right ventricular cardiomyopathy (ARVC) on late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) are unclear. OBJECTIVE: We sought to define a) the association of regional RV epicardial voltage amplitude with the distribution of LGE, and b) appropriate image signal intensity (SI) thresholds for VT substrate identification, in ARVC. METHODS: Pre-procedural LGE-CMR and epicardial electrogram mapping were performed in 10 ARVC patients...
March 1, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
C M Mak, S Pl Chen, N S Mok, W K Siu, H Hc Lee, C K Ching, P T Tsui, N C Fong, Y P Yuen, W T Poon, C Y Law, Y K Chong, Y W Chan, T C Yung, K Yy Fan, C W Lam
INTRODUCTION: Hereditary channelopathies and cardiomyopathies are potentially lethal and are clinically and genetically heterogeneous, involving at least 90 genes. Genetic testing can provide an accurate diagnosis, guide treatment, and enable cascade screening. The genetic basis among the Hong Kong Chinese population is largely unknown. We aimed to report on 28 unrelated patients with positive genetic findings detected from January 2006 to December 2015. METHODS: Sanger sequencing was performed for 28 unrelated patients with a clinical diagnosis of channelopathies or cardiomyopathies, testing for the following genes: KCNQ1, KCNH2, KCNE1, KCNE2, and SCN5A, for long QT syndrome; SCN5A for Brugada syndrome; RYR2 for catecholaminergic polymorphic ventricular tachycardia; MYH7 and MYBPC3 for hypertrophic cardiomyopathy; LMNA for dilated cardiomyopathy; and PKP2 and DSP for arrhythmogenic right ventricular dysplasia/cardiomyopathy...
March 2, 2018: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
Kathleen T Hickey, Amir Elzomor
The discovery of the human genome has ushered in a new era of molecular testing, advancing our knowledge and ability to identify cardiac channelopathies. Genetic variations can affect the opening and closing of the potassium, sodium, and calcium channels, resulting in arrhythmias and sudden death. Cardiac arrhythmias caused by disorders of ion channels are known as cardiac channelopathies. Nurses are important members of many interdisciplinary teams and must have a general understanding of the pathophysiology of the most commonly encountered cardiac channelopathies, electrocardiogram characteristics, approaches to treatment, and care for patients and their families...
2018: AACN Advanced Critical Care
Mateusz K Hołda, Agata Krawczyk-Ożóg, Mateusz Koziej, Danuta Sorysz, Jakub Hołda, Dariusz Dudek, Wiesława Klimek-Piotrowska
BACKGROUND: Recent studies suggest that the left-sided septal pouch (SP) may increase the risk of cryptogenic stroke and act as an arrhythmogenic substrate. The aim of this study was to compare two transesophageal echocardiography (TEE) projections of the interventricular septum: mid-esophageal bicaval and short-axis views toward evaluating their ability to detect SPs. MATERIALS AND METHODS: A total of 146 patients with both bicaval and short-axis TEE views were included in this study...
February 28, 2018: Echocardiography
Lu Yang, Guodong Ma, Tianyu Yu, Huikuan Gao, Yongliang Wang, Yongquan Wu
RATIONALE: Vasospastic angina is caused by sudden occlusive vasoconstriction of a segment of an epicardial artery, with transient ST-segment elevation on electrocardiography. Brugada Syndrome is an inherited arrhythmogenic cardiac disorder with a diagnostic electrocardiography characterized by coved-type ST-segment elevation in right precordial leads (V1-V3). Those two diseases usually have no correlation. In this report, we discuss an interesting case of a patient who was diagnosed as vasospastic angina according to his coronary angiography, but his electrocardiography showed a Brugada-like ST-segment elevation...
March 2018: Medicine (Baltimore)
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