22,304 results match your criteria Cardiomyopathy Dilated


Waitlist Mortality of Amyloid Cardiomyopathy Patients Listed for Heart Transplantation and Implications for Organ Allocation.

J Card Fail 2019 Apr 21. Epub 2019 Apr 21.

Department of Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, United States. Electronic address:

Background: While outcomes of patients with amyloid cardiomyopathy (ACM) undergoing heart transplantation have been reported, there is scant data on the waitlist mortality of these patients. The aim of this study was to investigate whether patients with amyloid cardiomyopathy have higher waitlist mortality compared to those with other types of cardiomyopathies.

Methods: We queried the United Network for Organ Sharing (UNOS) registry for all patients (age ≥ 18 years) listed for heart transplantation between 2008 and 2015. Read More

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http://dx.doi.org/10.1016/j.cardfail.2019.04.011DOI Listing
April 2019
2 Reads

Pacemaker Dysfunction due to a Large Thrombus on Ventricular Lead.

Heart Surg Forum 2019 Mar 11;22(2):E131-E133. Epub 2019 Mar 11.

Department of Cardiology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.

Background: Pacemaker lead-related thrombosis is a rare but severe complication in patients with pacing lead implantation in the right ventricle. We present a case with recurrent syncope after single-chamber implantable cardioverter defibrillator (ICD) implantation. Pacing lead-related thrombosis was observed during open-heart surgery. Read More

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https://journal.hsforum.com/index.php/HSF/article/view/2291
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http://dx.doi.org/10.1532/hsf.2291DOI Listing
March 2019
1 Read

The altered expression of autophagy-related genes participates in heart failure: NRBP2 and CALCOCO2 are associated with left ventricular dysfunction parameters in human dilated cardiomyopathy.

PLoS One 2019 22;14(4):e0215818. Epub 2019 Apr 22.

Cardiocirculatory Unit, Health Research Institute of La Fe University Hospital (IIS La Fe), Valencia, Spain.

This study aimed to analyze changes in the expression of autophagy- and phagocytosis-related genes in patients with dilated cardiomyopathy (DCM), especially in relation to left ventricular (LV) dysfunction. Furthermore, transmission electron microscopy of the diseased tissue was carried out to investigate if the gene expression changes are translated into ultrastructural alterations. LV tissue samples from patients with DCM (n = 13) and from controls (CNT; n = 10) were analyzed by RNA-sequencing, whereupon the altered expression (P < 0. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0215818PLOS

Sugars make the difference - Glycosylation of cardiodepressant antibodies regulates their activity in dilated cardiomyopathy.

Int J Cardiol 2019 Apr 9. Epub 2019 Apr 9.

Department of Internal Medicine B, Cardiology, University Medicine Greifswald, Germany; DZHK (German Centre for Cardiovascular Research), partner site Greifswald, Germany. Electronic address:

Background: Cardiodepressant antibodies contribute to cardiac dysfunction in dilated cardiomyopathy (DCM). Changes in immunoglobulin G (IgG) glycosylation modulate the activity of various autoimmune diseases and influence disease activity as well as severity of various autoimmune diseases. We hypothesized that alterations in IgG glycosylation are involved in the disease course of DCM. Read More

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http://dx.doi.org/10.1016/j.ijcard.2019.04.025DOI Listing

Sudden Death Related Cardiomyopathies - Arrhythmogenic Right Ventricular Cardiomyopathy, Arrhythmogenic Cardiomyopathy, and Exercise-Induced Cardiomyopathy.

Prog Cardiovasc Dis 2019 Apr 17. Epub 2019 Apr 17.

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America; Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America. Electronic address:

Sudden cardiac death (SCD) is a devastating possible outcome of all cardiomyopathies. The risk of SCD is increased in patients with structural heart disease and continues to increase as ventricular dysfunction worsens. There is however a subset of cardiomyopathy, so-called "arrhythmogenic cardiomyopathy" (ACM), that carries an inherent propensity for arrhythmia in all stages of the disease, even preceding ventricular dysfunction. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00330620193007
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http://dx.doi.org/10.1016/j.pcad.2019.04.002DOI Listing
April 2019
1 Read

Effect of IL-6-mediated STAT3 signaling pathway on myocardial apoptosis in mice with dilated cardiomyopathy.

Eur Rev Med Pharmacol Sci 2019 Apr;23(7):3042-3050

Department of Geriatrics, Quanzhou First Hospital of Fujian Medical University, Quanzhou, China.

Objective: To investigate the effect of interleukin-6 (IL-6) gene knockout on apoptosis of myocardial cells in mice with Coxsackievirus B3 (CVB3)-induced dilated cardiomyopathy (DCM) and its potential mechanism, so as to provide certain references for the clinical prevention and treatment of DCM.

Materials And Methods: A total of 40 male C57 mice were randomly divided into Sham group (n=20) and DCM group (n=20) using a random number table. Another 20 mice with IL-6 gene knockout were enrolled into DCM+IL-6 KO group (n=20). Read More

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http://www.europeanreview.org/article/17586
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http://dx.doi.org/10.26355/eurrev_201904_17586DOI Listing
April 2019
2 Reads

Use of Novel Electrogram "Lumipoint" Algorithm to Detect Critical Isthmus and Abnormal Potentials for Ablation in Ventricular Tachycardia.

JACC Clin Electrophysiol 2019 Apr 27;5(4):470-479. Epub 2019 Mar 27.

Electrophysiology and Ablation Unit, Bordeaux University Hospital (Centre Hospitalier Universitaire [CHU]), Pessac, France; Institut Hospitalo-Universitaire (IHU), LIRYC, Electrophysiology and Heart Modeling Institute, Fondation Bordeaux Université, Pessac- Bordeaux, France; Université Bordeaux, Institut National de la Santé et de la Recherche Médicale U1045, Bordeaux, France.

Objectives: This study reports the use of a novel "Lumipoint" algorithm in ventricular tachycardia (VT) ablation.

Background: Automatic mapping systems aid rapid acquisition of activation maps. However, they may annotate farfield rather than nearfield signal in low voltage areas, making maps difficult to interpret. Read More

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http://dx.doi.org/10.1016/j.jacep.2019.01.016DOI Listing

The assembly and evaluation of antisense oligonucleotides applied in exon skipping for titin-based mutations in dilated cardiomyopathy.

J Mol Cell Cardiol 2019 Apr 15;131:12-19. Epub 2019 Apr 15.

Department of Cardiology and Cardiovascular Diseases, Eberhard Karls University, Tübingen, Germany; Department of Invasive Electrophysiology, RWTH Aachen, Pauwelsstr. 30, Aachen, Germany. Electronic address:

The leading cause of genetic dilated cardiomyopathy (DCM) is due to mutations in the TTN gene, impacting approximately 15-20% of familial and 18% of sporadic DCM cases. Currently, there is potential for a personalized RNA-based therapeutic approach in titin-based DCM, utilizing antisense oligonucleotide (AON) mediated exon-skipping, which attempts to reframe mutated titin transcripts, resulting in shortened, functional protein. However, the TTN gene is massive with 363 exons; each newly identified TTN exon mutation provides a challenge to address when considering the potential application of AON mediated exon skipping. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00222828193003
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http://dx.doi.org/10.1016/j.yjmcc.2019.04.014DOI Listing
April 2019
2 Reads

MYH7 mutation identified by next-generation sequencing in three infant siblings with bi-ventricular noncompaction presenting with restrictive hemodynamics: A report of three siblings with a severe phenotype and poor prognosis.

J Cardiol Cases 2019 Apr 11;19(4):140-143. Epub 2019 Feb 11.

Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine, Hirosaki, Japan.

Noncompaction of the ventricular myocardium (NVM) is a genetically heterogeneous cardiomyopathy. Various mutations associated with NVM have been identified in several genes. NVM patients usually present with complications of dilated cardiomyopathy. Read More

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http://dx.doi.org/10.1016/j.jccase.2018.12.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451088PMC
April 2019
2 Reads

A successful case of percutaneous fistula closure in a patient with high output heart failure and extracardiac arteriovenous fistula.

J Cardiol Cases 2019 Apr 29;19(4):125-128. Epub 2018 Dec 29.

Department of Cardio-Renal Medicine and Hypertension, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Intra-cardiac shunt diseases may cause chronic ventricular volume overload, but extra-cardiac fistula could also cause high-output heart failure (HF). A patient presented with high-output HF and significant extra-cardiac shunt flow. Although the size and shape of the patient's left ventricle suggested dilated cardiomyopathy, considerable origins were not identified except for a high-flow fistula between the right subclavian artery and right internal jugular vein. Read More

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http://dx.doi.org/10.1016/j.jccase.2018.12.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451089PMC
April 2019
1 Read

Patients with Isolated Focal Right Ventricular Dyskinetic Segments: Toward a Better Understanding of This Cohort.

J Cardiovasc Imaging 2019 Apr;27(2):93-101

Division of Cardiovascular Medicine, Clemenceau Medical Center, Beirut, Lebanon.

Background: The 2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) provided guidance for the classification of patients as definitive, borderline or possible ARVC. However, many patients with clinical suspicion for ARVC have isolated RV dyskinetic segments only and partly meet cardiac magnetic resonance (CMR) imaging criteria. This subgroup of patients and the implication of this imaging finding remain not well defined. Read More

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http://dx.doi.org/10.4250/jcvi.2019.27.e16DOI Listing
April 2019
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Whole-Exome Sequencing Reveals Novel Genetic Variation for Dilated Cardiomyopathy in Pediatric Chinese Patients.

Pediatr Cardiol 2019 Apr 16. Epub 2019 Apr 16.

Department of Cardiology, Children's Hospital of Nanjing Medical University, 72 Guangzhou Road, Nanjing, 210008, China.

Dilated cardiomyopathy (DCM) is characterized by left or bilateral ventricular dilation and systolic dysfunction without rational conditions, which can lead to progressive heart failure and sudden cardiac death. Most of the pathogenic genes have been reported in adult population by locus mapping in familial cases and animal model studies. However, it still remains challenging to decipher the role of genetics in the etiology of pediatric DCM. Read More

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http://dx.doi.org/10.1007/s00246-019-02096-1DOI Listing
April 2019
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Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Eur J Heart Fail 2019 Apr 16. Epub 2019 Apr 16.

Berlin-Brandenburg Center for Regenerative Therapies, Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK) Berlin, Department of Cardiology, Campus Virchow Klinikum, Charite - Universitaetsmedizin Berlin, Berlin, Germany.

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. Read More

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http://dx.doi.org/10.1002/ejhf.1461DOI Listing
April 2019
2 Reads
6.526 Impact Factor

ECG in dilated cardiomyopathy: specific findings and long-term prognostic significance.

J Cardiovasc Med (Hagerstown) 2019 Apr 9. Epub 2019 Apr 9.

Cardiovascular Department, 'Ospedali Riuniti' and University of Trieste.

Objective: The objective was to provide an exhaustive characterization of ECG features in a large cohort of dilated cardiomyopathies (DCMs) and then investigate their possible prognostic role in the long term.

Background: ECG is an accessible, reproducible, low-cost diagnostic and prognostic tool. However, an extensive description of ECG features and their long-term prognostic role in a large cohort of DCM is lacking. Read More

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http://dx.doi.org/10.2459/JCM.0000000000000804DOI Listing
April 2019
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[Idiopathic dilated cardiomyopathy in children: Prognostic indicators].

Turk Kardiyol Dern Ars 2019 Apr;47(3):207-215

Dr. Sami Ulus Obstetrics and Gynecology, Children's Health and Diseases Training and Research Hospital, Ankara, Turkey.

Objective: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients. Read More

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http://dx.doi.org/10.5543/tkda.2018.72809DOI Listing
April 2019
2 Reads

Nexilin is a New Component of Junctional Membrane Complexes Required for Cardiac T-Tubule Formation.

Circulation 2019 Apr 15. Epub 2019 Apr 15.

Department of Medicine, University of California San Diego, La Jolla, CA.

Background: Membrane contact sites (MCS) are fundamental for transmission and translation of signals in multicellular organisms. A prime example is junctional membrane complexes (JMC) in the cardiac dyads, where T-tubules are juxtaposed to the sarcoplasmic reticulum (SR). T-tubule uncoupling and remodeling are well-known features of cardiac disease and heart failure. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.119.039751DOI Listing
April 2019
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Analyses for Prevalence and Outcome of Tricuspid Regurgitation in China: An Echocardiography Study of 134,874 Patients.

Cardiology 2019 Apr 12;142(1):40-46. Epub 2019 Apr 12.

Department of Cardiology, Shanghai Institute of Cardiovascular Disease, Zhongshan Hospital, Fudan University, Shanghai, China.

Background: The aim of this study is to investigate the prevalence and outcome of tricuspid regurgitation (TR) in the Chinese population.

Methods: The echocardiography database, including 134,874 patients at our heart center from 2010 to 2012, was retrospectively analyzed.

Results: The rates of mild, moderate, and severe TR were 2. Read More

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http://dx.doi.org/10.1159/000496601DOI Listing
April 2019
1 Read
2.044 Impact Factor

ERK1/2 mediates the lipopolysaccharide-induced upregulation of FGF-2, uPA, MMP-2, MMP-9 and cellular migration in cardiac fibroblasts.

Chem Biol Interact 2019 Apr 11;306:62-69. Epub 2019 Apr 11.

Department of Biotechnology, Asia University, Taichung, Taiwan; College of Medicine, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Tzu Chi University, Hualien, Taiwan; Graduate Institute of Basic Medical Science, China Medical University, Taichung, Taiwan. Electronic address:

Myocardial fibrosis is a critical event during septic shock. Upregulation in the fibrosis signaling cascade proteins such as fibroblast growth factor (FGF), urokinase plasminogen activator (uPA), tissue plasminogen activator (tPA) and activation of matrix metalloproteinases (MMPs) are widely associated with the development of myocardial infarction, dilated cardiomyopathy, cardiac fibrosis and heart failure. However, evidences suggest that the common upstream mediators of fibrosis cascade play little role in cardiac fibrosis induced by LPS; further, it is unknown if LPS directly triggers the expressions and/or activity of FGF-2, uPA, tPA, MMP-2 and MMP-9 in cardiac fibroblasts. Read More

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http://dx.doi.org/10.1016/j.cbi.2019.04.010DOI Listing
April 2019
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A Matched-Filter-Based Algorithm for Subcellular Classification of T-System in Cardiac Tissues.

Biophys J 2019 Apr 22;116(8):1386-1393. Epub 2019 Mar 22.

Department of Chemistry, University of Kentucky, Lexington, Kentucky; Department of Chemical and Materials Engineering, University of Kentucky, Lexington, Kentucky.

In mammalian ventricular cardiomyocytes, invaginations of the surface membrane form the transverse tubular system (T-system), which consists of transverse tubules (TTs) that align with sarcomeres and Z-lines as well as longitudinal tubules (LTs) that are present between Z-lines in some species. In many cardiac disease etiologies, the T-system is perturbed, which is believed to promote spatially heterogeneous, dyssynchronous Ca release and inefficient contraction. In general, T-system characterization approaches have been directed primarily at isolated cells and do not detect subcellular T-system heterogeneity. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00063495193019
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http://dx.doi.org/10.1016/j.bpj.2019.03.010DOI Listing
April 2019
3 Reads

Human Umbilical Cord Mesenchymal Stem Cells Alleviate Myocardial Endothelial-Mesenchymal Transition in a Rat Dilated Cardiomyopathy Model.

Transplant Proc 2019 Apr 31;51(3):936-941. Epub 2019 Jan 31.

Department of Pediatrics, Second Affiliated Hospital of Shantou University Medical College, Shantou, Guangdong, China; Department of Paediatrics, Children's Hospital of Shenzhen, Guangdong, China; Department of Pediatrics, Maternal and Child Health Care Hospital of Pingshan District, Shenzhen, Guangdong, China. Electronic address:

Background: Human umbilical cord-derived mesenchymal stem cells (HuMSCs) have been shown to suppress cardiac fibrosis; however, the underlying mechanisms are not fully understood. Recent studies have shown that endothelial-mesenchymal transition (EndMT) plays a crucial part in myocardial fibrosis. In the present study, we investigated the suppressive role of HuMSCs in cardiac fibrosis and related mechanisms in a rat dilated cardiomyopathy (DCM) model. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183083
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http://dx.doi.org/10.1016/j.transproceed.2019.01.080DOI Listing
April 2019
3 Reads

Advances in the Genetic Basis and Pathogenesis of Sarcomere Cardiomyopathies.

Annu Rev Genomics Hum Genet 2019 Apr 12. Epub 2019 Apr 12.

Department of Genetics, Harvard Medical School, Boston, Massachusetts 02115, USA; email: ,

Hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) are common heart muscle disorders that are caused by pathogenic variants in sarcomere protein genes. HCM is characterized by unexplained cardiac hypertrophy (increased chamber wall thickness) that is accompanied by enhanced cardiac contractility and impaired relaxation. DCM is defined as increased ventricular chamber volume with contractile impairment. Read More

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https://www.annualreviews.org/doi/10.1146/annurev-genom-0831
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http://dx.doi.org/10.1146/annurev-genom-083118-015306DOI Listing
April 2019
2 Reads

Plasma N-terminal pro-B-type natriuretic peptide (BNP) in mesalazine-induced myopericarditis.

BMJ Case Rep 2019 Apr 11;12(4). Epub 2019 Apr 11.

Cardiology and Medicine, Frimley Health NHS Foundation Trust, Slough, Berkshire, UK.

Mesalazine (5-aminosalicylic acid)-based products are a widely used treatment for inflammatory bowel disease in children and adults. Associated myopericarditis is an uncommon but recorded phenomenon related to drug hypersensitivity. Unless recognised, this important complication may culminate in the development of dilated cardiomyopathy and severe heart failure. Read More

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http://dx.doi.org/10.1136/bcr-2018-229142DOI Listing
April 2019
1 Read

Autosomal recessive ADCY5-Related dystonia and myoclonus: Expanding the genetic spectrum of ADCY5-Related movement disorders.

Parkinsonism Relat Disord 2019 Feb 28. Epub 2019 Feb 28.

Department of Genetics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia; Department of Biomedical Research, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

Introduction: ADCY5-related hyperkinesia encompasses a heterogeneous group of phenotypes, including paroxysmal chorea, myoclonus, and dystonia. The disease is attributed to mutations of ADCY5, which encodes an adenylate cyclase enzyme. The disease can occur in a sporadic or familial pattern. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2019.02.039DOI Listing
February 2019
1 Read

Effects of pulmonary artery banding in doxorubicin-induced left ventricular cardiomyopathy.

J Thorac Cardiovasc Surg 2019 Mar 2. Epub 2019 Mar 2.

Pediatric Heart Center Giessen, Justus-Liebig-University, Giessen, Germany.

Objective: Central pulmonary banding has been proposed as a novel alternative for the treatment of left ventricular dilated cardiomyopathy in children. We sought to investigate the effects of central pulmonary banding in an experimental model of doxorubicin-induced left ventricular dilated cardiomyopathy.

Methods: Four-month-old sheep (n = 28) were treated with intermittent intracoronary injections of doxorubicin (0. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00225223193053
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http://dx.doi.org/10.1016/j.jtcvs.2019.01.138DOI Listing
March 2019
3 Reads

Epigenetics in dilated cardiomyopathy.

Curr Opin Cardiol 2019 May;34(3):260-269

Departments of Anesthesiology, Physiology and Medicine, David Geffen School of Medicine, University of California at Los Angeles, California, USA.

Purpose Of Review: Characterized by enlarged ventricle and loss of systolic function, dilated cardiomyopathy (DCM) has the highest morbidity among all the cardiomyopathies. Although it is well established that DCM is typically caused by mutations in a large number of genes, there is an emerging appreciation for the contribution of epigenetic alteration in the development of DCM.

Recent Findings: We present some of the recent progress in the field of epigenetics in DCM by focusing on the four major epigenetic modifications, that is, DNA methylation, histone modification, chromatin remodeling as well as the noncoding RNAs. Read More

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http://dx.doi.org/10.1097/HCO.0000000000000616DOI Listing
May 2019
2 Reads

Significance of preoperative right ventricular function on mid-term outcomes after surgical ventricular restoration for ischemic cardiomyopathy.

Gen Thorac Cardiovasc Surg 2019 Apr 9. Epub 2019 Apr 9.

Department of Cardiovascular Surgery, Faculty of Medicine, University of Miyazaki, 5200 Kiyotakecho Kihara, Miyazaki-city, Miyazaki, 889-1692, Japan.

Objectives: To analyze our surgical experiences with surgical ventricular restoration (SVR) for dilated ischemic cardiomyopathy (ICM) and to determine the significance of preoperative right ventricular (RV) function on outcomes.

Methods And Results: This study retrospectively analyzed 19 patients who underwent SVR between April 2010 and May 2016. Their mean age and New York Heart Association functional class were 62 ± 11 years and 2. Read More

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http://link.springer.com/10.1007/s11748-019-01123-5
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http://dx.doi.org/10.1007/s11748-019-01123-5DOI Listing
April 2019
2 Reads

Myocardin ablation in a cardiac-renal rat model.

Sci Rep 2019 Apr 10;9(1):5872. Epub 2019 Apr 10.

Department of Experimental Medicine and Biotechnology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Cardiorenal syndrome is defined by primary heart failure conditions influencing or leading to renal injury or dysfunction. Dilated cardiomyopathy (DCM) is a major co-existing form of heart failure (HF) with renal diseases. Myocardin (MYOCD), a cardiac-specific co-activator of serum response factor (SRF), is increased in DCM porcine and patient cardiac tissues and plays a crucial role in the pathophysiology of DCM. Read More

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http://dx.doi.org/10.1038/s41598-019-42009-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458122PMC
April 2019
4 Reads
5.078 Impact Factor

mA-mRNA methylation regulates cardiac gene expression and cellular growth.

Life Sci Alliance 2019 Apr 9;2(2). Epub 2019 Apr 9.

Department of Cardiology, Angiology, and Pneumology, University Hospital Heidelberg, University of Heidelberg, Heidelberg, Germany

Conceptually similar to modifications of DNA, mRNAs undergo chemical modifications, which can affect their activity, localization, and stability. The most prevalent internal modification in mRNA is the methylation of adenosine at the N-position (mA). This returns mRNA to a role as a central hub of information within the cell, serving as an information carrier, modifier, and attenuator for many biological processes. Read More

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http://dx.doi.org/10.26508/lsa.201800233DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458851PMC
April 2019
1 Read

Biallelic variants in SMAD6 are associated with a complex cardiovascular phenotype.

Hum Genet 2019 Apr 8. Epub 2019 Apr 8.

Institute of Human Genetics, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.

Rare heterozygous variants in SMAD6 have been identified as a significant genetic contributor to bicuspid aortic valve-associated thoracic aortic aneurysm on one hand and non-syndromic midline craniosynostosis on the other. In this study, we report two individuals with biallelic missense variants in SMAD6 and a complex cardiac phenotype. Trio exome sequencing in Proband 1, a male who had aortic isthmus stenosis, revealed the homozygous SMAD6 variant p. Read More

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http://dx.doi.org/10.1007/s00439-019-02011-xDOI Listing
April 2019
1 Read

Titin in muscular dystrophy and cardiomyopathy: Urinary titin as a novel marker.

Clin Chim Acta 2019 Apr 5;495:123-128. Epub 2019 Apr 5.

Department of Cardiovascular Medicine, Fukushima Medical University, Fukushima, Japan.

Titin, encoded by the gene TTN, is the largest human protein, and plays central roles in sarcomeric structures and functions in skeletal and cardiac muscles. Mutations of TTN are causally related to specific types of muscular dystrophies and cardiomyopathies. A developed methodology of next generation sequencing has recently led to the identification of novel TTN mutations in such diseases. Read More

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http://dx.doi.org/10.1016/j.cca.2019.04.005DOI Listing
April 2019
2 Reads

MURC/CAVIN-4 facilitates store-operated calcium entry in neonatal cardiomyocytes.

Biochim Biophys Acta Mol Cell Res 2019 Apr 2;1866(8):1249-1259. Epub 2019 Apr 2.

Département de Pharmacologie et Physiologie, Faculté de Médecine et des Sciences de la Santé, Université de Sherbrooke, QC J1H 5N4, Canada; Institut de Pharmacologie de Sherbrooke, Faculté de Médecine et des Sciences de la Santé, Université de Sherbrooke, Sherbrooke, QC J1H 5N4, Canada. Electronic address:

Intact store-operated calcium entry (SOCE) mechanisms ensure the maintenance of Ca homeostasis in cardiomyocytes while their dysregulation promotes the development of cardiomyopathies. To better understand this calcium handling process in cardiomyocytes, we sought to identify unknown protein partners of stromal interaction molecule 1 (STIM1), a main regulatory protein of SOCE. We identified the muscle-related coiled-coil protein (MURC), also known as Cavin-4, as a candidate and showed that MURC interacts with STIM1 in cardiomyocytes. Read More

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http://dx.doi.org/10.1016/j.bbamcr.2019.03.017DOI Listing
April 2019
1 Read
5.019 Impact Factor

Myocarditis evolving in cardiomyopathy: when genetics and offending causes work together.

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B90-B95. Epub 2019 Mar 29.

Cardiovascular Department, Azienda Sanitaria Universitaria Integrata di Trieste e Università degli Studi di Trieste.

Myocarditis is an infectious-inflammatory disease often superimposed to individual genetic background which could favour or inhibit its progression into a chronic heart muscle disorder (most often dilated cardiomyopathy, rarely arrhythmogenic, or right-sided cardiomyopathy). Post-myocarditis cardiomyopathy is likely caused by a complex interaction between the viral infection and an individual predisposition. Some viruses are able to highlight a clinical phenotype replicating a model similar to the genetically determined conditions, while other can affect the resolution or the progressive remodelling of the left ventricle after the infectious process. Read More

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http://dx.doi.org/10.1093/eurheartj/suz033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439912PMC
March 2019
1 Read

Epicardial ablation in genetic cardiomyopathies: a new frontier.

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B61-B66. Epub 2019 Mar 29.

Arrhythmology and Electrophysiology Department, IRCCS Policlinico San Donato, San Donato Milanese, Milano, Italy.

Brugada syndrome (BrS) and several cardiomyopathies, including dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction (LVNC), and hypertrophic cardiomyopathy (HCM), share common genetic mutations and are associated with an arrhythmogenic substrate (AS) and increased risk of sudden cardiac death (SCD) due to malignant ventricular arrhythmias. We report a family in which a SCN5A mutation was found in both a father and daughter who presented with different phenotypes: the father with LVNC and the daughter with BrS, suggesting SCN5A may be important in cases of overlap between BrS and these various other cardiomyopathies and arrhythmias. Additionally, we report a family in which a MYBPC3 mutation was found in a father, daughter, and son, but they also presented with different phenotypes: the father with HCM and the daughter and son with BrS, suggesting patients with cardiomyopathies or BrS exhibiting sarcomeric mutations may have common genetic pathways that ultimately diverge into different phenotypes. Read More

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https://academic.oup.com/eurheartjsupp/article/21/Supplement
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http://dx.doi.org/10.1093/eurheartj/suz028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439905PMC
March 2019
3 Reads

Defibrillator and non-ischaemic dilated cardiomyopathy: a never ending story.

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B5-B6. Epub 2019 Mar 29.

Department of Cardiology and Cardiovascular Pathophysiology, Azienda Ospedaliera Universitaria di Perugia, Italy.

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http://dx.doi.org/10.1093/eurheartj/suz005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439895PMC
March 2019
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Small RNA-sequencing identified the potential roles of neuron differentiation and MAPK signaling pathway in dilated cardiomyopathy.

Biomed Pharmacother 2019 Apr 1:108826. Epub 2019 Apr 1.

Department of Cardiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. Electronic address:

Dilated cardiomyopathy is a severe disease characterized by ventricular enlargement and subsequent cardiac dysfunction. MiRNAs plays multiple roles in cardiovascular disease. However, diagnosis values and therapeutic effects of miRNAs in dilated cardiomyopathy are yet poorly understood. Read More

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http://dx.doi.org/10.1016/j.biopha.2019.108826DOI Listing
April 2019
2 Reads

Cardiac Phenotypes, Genetics, and Risks in Familial Noncompaction Cardiomyopathy.

J Am Coll Cardiol 2019 Apr;73(13):1601-1611

Department of Clinical Genetics, Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address:

Background: There is overlap in genetic causes and cardiac features in noncompaction cardiomyopathy (NCCM), hypertrophic cardiomyopathy (HCM), and dilated cardiomyopathy (DCM).

Objectives: The goal of this study was to predict phenotype and outcome in relatives according to the clinical features and genotype of NCCM index cases.

Methods: Retrospective DNA and cardiac screening of relatives of 113 families from 143 index patients were used to classify NCCM cases according to the cardiac phenotype. Read More

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http://dx.doi.org/10.1016/j.jacc.2018.12.085DOI Listing
April 2019
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Heart transplantation in a patient with Myotonic Dystrophy type 1 and end-stage dilated cardiomyopathy: a short term follow-up.

Acta Myol 2018 Dec 1;37(4):267-271. Epub 2018 Dec 1.

Cardiomyology and Medical Genetics, Department of Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.

Myotonic dystrophy type 1 (DM1) or Steinert's disease is the most common muscular dystrophy in adult life with an estimated prevalence of 1:8000. Cardiac involvement, including arrhythmias and conduction disorders, contributes significantly to the morbidity and mortality of the disease. Mild ventricular dysfunction has also been reported associated with conduction disorders, but severe ventricular systolic dysfunction is not a frequent feature and usually occurs late in the course of the disease. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416698PMC
December 2018
2 Reads

Left ventricular apical hypertrophy in a transplanted heart: a case report.

BMC Cardiovasc Disord 2019 Apr 3;19(1):81. Epub 2019 Apr 3.

Department of Cardiology, St Francis Hospital and Medical Center, 114 Woodland St, Hartford, CT, 06105, USA.

Background: Left ventricular apical hypertrophic cardiomyopathy is a rare presentation of hypertrophic cardiomyopathy associated with thickening of the apical segment of the left ventricle. It was initially described in Japan in 1976 and is characterized by electrocardiogram findings showing giant T wave inversions in the precordial leads as well as a spade shaped appearance of the apical cavity on imaging (Abugroun et al., Cardiol Res 8:265-268, 2017). Read More

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http://dx.doi.org/10.1186/s12872-019-1069-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446306PMC
April 2019
4 Reads

Complex multisystem phenotype associated with the mitochondrial DNA m.5522G>A mutation.

Neurol Sci 2019 Apr 1. Epub 2019 Apr 1.

Molecular Medicine for Neurodegenerative and Neuromuscular Diseases Unit, IRCCS Stella Maris Foundation, via dei Giacinti 2, 56128, Pisa, Italy.

Mitochondrial tRNAs are responsible for more than half of pathogenic point mutations in the mitochondrial genome (mtDNA). Different mutations give rise to widely differing phenotypes, ranging from isolated organ-specific diseases to multisystem conditions. Herein, we report a 40-year-old woman presenting with a complex multisystem phenotype including sensorineural hearing loss, retinopathy, severe dilated cardiomyopathy, non-insulin dependent diabetes mellitus, and renal failure. Read More

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http://link.springer.com/10.1007/s10072-019-03864-w
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http://dx.doi.org/10.1007/s10072-019-03864-wDOI Listing
April 2019
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Percutaneous Biventricular Mechanical Heart Support in Cardiogenic Shock: A Nursing Case Report.

Crit Care Nurse 2019 Apr;39(2):15-28

Bridget K. Dittman is a clinical nurse practice leader in the intensive care unit at Einstein Center Montgomery in East Norriton, Pennsylvania.

Introduction: Only a few cases of biventricular cardiogenic shock have been treated with Impella circulatory assist devices in the United States.

Clinical Findings: A 29-year-old man came to the emergency department because of cough, shortness of breath, fever, and chills. Initial assessment revealed hypotension; an elevated creatinine level of 2. Read More

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http://dx.doi.org/10.4037/ccn2019477DOI Listing
April 2019
2 Reads

Evaluation and management of ventricular tachycardia in patients with dilated cardiomyopathy.

Heart Rhythm 2019 Apr 26;16(4):624-631. Epub 2018 Oct 26.

Department of Electrophysiology, HELIOS Heart Center - University of Leipzig, Leipzig, Germany.

Recurrent ventricular tachycardia (VT) is an important cause of increased morbidity and mortality in patients with non-ischemic dilated cardiomyopathy (DCM). Current recommendations for the treatment of VT in patients with structural heart disease mainly rely on data derived from patients with ischemic cardiomyopathy. Unlike postinfarction ischemic cardiomyopathy, DCM comprises multiple different etiologies with variable disease progression and prognosis, which often requires an individualized approach to risk stratification and treatment. Read More

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http://dx.doi.org/10.1016/j.hrthm.2018.10.028DOI Listing
April 2019
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Management of primary graft failure after heart transplantation: Preoperative risks, perioperative events, and postoperative decisions.

Clin Transplant 2019 Apr 1:e13557. Epub 2019 Apr 1.

Division of Cardiology, Department of Medicine, Columbia University Irving Medical Center, New York, New York.

Primary graft failure (PGF) after heart transplantation (HT) is a devastating and unexpected event characterized by failure of the graft to adequately support recipient circulation necessitating high doses of vasopressors and inotropes and/or temporary mechanical circulatory support. Although it represents an increasingly common event in the current era, there remains a high degree of variability in prevalence, reported risk factors, and approach to this clinical entity. The purpose of the current review is to highlight preoperative considerations including known incidence and risk factors, perioperative issues involving the identification and management of PGF, and postoperative decisions related to weaning of mechanical circulatory support and titration of immunosuppressive therapy. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/ctr.13557
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http://dx.doi.org/10.1111/ctr.13557DOI Listing
April 2019
5 Reads

Evidence for the impact of BAG3 on electrophysiological activity of primary culture of neonatal cardiomyocytes.

J Cell Physiol 2019 Apr 1. Epub 2019 Apr 1.

Department of Neuroscience, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania.

Homeostasis of proteins involved in contractility of individual cardiomyocytes and those coupling adjacent cells is of critical importance as any abnormalities in cardiac electrical conduction may result in cardiac irregular activity and heart failure. Bcl2-associated athanogene 3 (BAG3) is a stress-induced protein whose role in stabilizing myofibril proteins as well as protein quality control pathways, especially in the cardiac tissue, has captured much attention. Mutations of BAG3 have been implicated in the pathogenesis of cardiac complications such as dilated cardiomyopathy. Read More

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http://dx.doi.org/10.1002/jcp.28471DOI Listing
April 2019
2 Reads

Surgical Ablation of Atrial Fibrillation in Patients with Tachycardia-Induced Cardiomyopathy.

Ann Thorac Surg 2019 Mar 27. Epub 2019 Mar 27.

Department of Surgery, Division of Cardiothoracic Surgery, Washington University School of Medicine, Barnes-Jewish Hospital, St. Louis, Missouri. Electronic address:

Background: Atrial fibrillation (AF) is a common cause of tachycardia-induced cardiomyopathy (TIC). This study evaluated the outcomes of the Cox-Maze IV procedure (CMP IV) in patients with TIC and significant left ventricular dysfunction.

Methods: Between January 2002 and January 2017, 37 consecutive patients with LVEF≤40% underwent stand-alone surgical ablation of AF. Read More

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http://dx.doi.org/10.1016/j.athoracsur.2019.01.088DOI Listing
March 2019
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Selective C-reactive protein-apheresis in patients.

Ther Apher Dial 2019 Mar 29. Epub 2019 Mar 29.

Cardiovascular Center Oberallgäu-Kempten, Kempten, Germany.

Background: C-reactive protein (CRP), the prototype human acute-phase protein, is a well-known marker of inflammation. However, CRP may also mediate tissue damage in various human diseases like atherosclerosis, acute myocardial infarction, dilated cardiomyopathy, stroke and potentially autoimmune disease. Therefore, CRP elimination from human plasma may indeed be a widely usable therapeutic approach. Read More

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http://dx.doi.org/10.1111/1744-9987.12804DOI Listing
March 2019
2 Reads

Combinatorial interactions of genetic variants in human cardiomyopathy.

Nat Biomed Eng 2019 Feb 7;3(2):147-157. Epub 2019 Feb 7.

Division of Cardiology, Department of Medicine, University of California, San Diego, La Jolla, CA, USA.

Dilated cardiomyopathy (DCM) is a leading cause of morbidity and mortality worldwide; yet how genetic variation and environmental factors impact DCM heritability remains unclear. Here, we report that compound genetic interactions between DNA sequence variants contribute to the complex heritability of DCM. By using genetic data from a large family with a history of DCM, we discovered that heterozygous sequence variants in the () and () genes cose-gregate in individuals affected by DCM. Read More

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http://dx.doi.org/10.1038/s41551-019-0348-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433174PMC
February 2019
1 Read

[Mitral Regurgitation Repaired by Papillary Heads Optimization Technique].

Kyobu Geka 2019 Mar;72(3):184-189

Department of Cardiovascular Surgery, Kagoshima University Hospital, Kagoshima, Japan.

Functional mitral regurgitation( FMR) is a risk factor that increases the mortality rate and incidence of heart failure. Surgical intervention is important. Mitral valve (MV) reconstruction can preserve the valvular apparatus;no anticoagulation therapy is required, left ventricular function is preserved. Read More

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March 2019
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Advanced Heart Failure Treatment Modalities and Hospice Care: The Need for High Level Care Coordination.

Am J Hosp Palliat Care 2019 Mar 28:1049909119838250. Epub 2019 Mar 28.

3 Division of Cardiology, University of Washington School of Medicine, Seattle, WA, USA.

Advanced heart failure therapies such as ventricular assist devices and home inotrope use are becoming more common. Technology advances as well as increased indications for use of such therapies is leading to a higher percentage of patients with end-stage heart failure receiving these therapies at end of life. We present a case of a young man with dilated cardiomyopathy who undergoes advanced cardiac care in the setting of progressively declining cardiac function. Read More

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http://dx.doi.org/10.1177/1049909119838250DOI Listing
March 2019
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Metformin Enhances Autophagy and Provides Cardioprotection in δ-Sarcoglycan Deficiency-Induced Dilated Cardiomyopathy.

Circ Heart Fail 2019 Apr;12(4):e005418

Department of Cardiology (H.K., G.N., A.Y., S.M., T.W., T.K., Y.Y., A.M., M.K., G.T., S.M.), Gifu University Graduate School of Medicine, Japan.

Background: Metformin is a popular antidiabetic agent that is also used to treat heart failure patients with type 2 diabetes mellitus. Several reports suggest that metformin may also have cardioprotective effects in patients without diabetes mellitus. In the present study, we investigated the possible therapeutic effect of metformin in heart failure and its underlying molecular mechanisms using a δ-sarcoglycan-deficient mouse model of dilated cardiomyopathy. Read More

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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.118.005418DOI Listing
April 2019
1 Read