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idiopathic dilated cardiomyopathy

ByungSu Yoo
Hypertension is the most common risk factor for systolic and diastolic heart failure. Based on population-attributable risks, hypertension has the greatest impact on the development of heart failure, accounting for 39% of HF events in men and 59% in women. Higher blood pressure, longer duration of hypertension and older age are associated with higher incidence of heart failure however, long term control of hypertension reduces the risk of heart failure. Thus current guideline pointed the hypertension as the single most important modifiable risk factor for heart failure...
September 2016: Journal of Hypertension
Alexandra Pérez-Serra, Rocio Toro, Georgia Sarquella-Brugada, David de Gonzalo-Calvo, Sergi Cesar, Esther Carro, Vicenta Llorente-Cortes, Anna Iglesias, Josep Brugada, Ramon Brugada, Oscar Campuzano
Dilated cardiomyopathy is a rare cardiac disease characterized by left ventricular dilatation and systolic dysfunction leading to heart failure and sudden cardiac death. Currently, despite several conditions have been reported as aetiologies of the disease, a large number of cases remain classified as idiopathic. Recent studies determine that nearly 60% of cases are inherited, therefore due to a genetic cause. Progressive technological advances in genetic analysis have identified over 60 genes associated with this entity, being TTN the main gene, so far...
September 21, 2016: International Journal of Cardiology
Natalia Y Osovska, Natalia V Kuzminova
INTRODUCTION: isolated left ventricular non-compaction (LVNC) is a heart disease with rather distinct morphologic and clinical manifestations. Available in the literature information about LVNC considering multiple left ventricle abnormal chords (LVAC) as one of its criterion motivated us to review the results obtained in the study of young patients with this pathology. The aim of the research was to demonstrate different clinical variants of left ventricular non-compaction course in adult patients and to clarify some pathogenetic aspects of this pathology...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Dilek Colak, Ayodele A Alaiya, Namik Kaya, Nzioka P Muiya, Olfat AlHarazi, Zakia Shinwari, Editha Andres, Nduna Dzimiri
AIMS: The disease pathways leading to idiopathic dilated cardiomyopathy (DCM) are still elusive. The present study investigated integrated global transcriptional and translational changes in human DCM for disease biomarker discovery. METHODS: We used identical myocardial tissues from five DCM hearts compared to five non-failing (NF) donor hearts for both transcriptome profiling using the ABI high-density oligonucleotide microarrays and proteome expression with One-Dimensional Nano Acquity liquid chromatography coupled with tandem mass spectrometry on the Synapt G2 system...
2016: PloS One
Susanna L den Boer, Dimitris Rizopoulos, Gideon J du Marchie Sarvaas, Ad P C M Backx, Arend D J Ten Harkel, Gabriëlle G van Iperen, Lukas A J Rammeloo, Ronald B Tanke, Eric Boersma, Willem A Helbing, Michiel Dalinghaus
N-terminal pro-B-type natriuretic peptide (NT-proBNP) is an important predictor of outcome in adults with heart failure. In children with heart failure secondary to dilated cardiomyopathy (DC) markers that reliably predict disease progression and outcome during follow-up are scarce. We investigated whether serial NT-proBNP measurements were predictive for outcome in children with DC. All available NT-proBNP measurements in children with DC were analyzed. Linear mixed-effect models and Cox regression were used to analyze the predictive value of NT-proBNP on the end point of cardiac death (death, heart transplantation, or mechanical circulatory support)...
August 30, 2016: American Journal of Cardiology
Fanny Tarando, Damien Coisne, Elena Galli, Chloé Rousseau, Frédéric Viera, Christian Bosseau, Gilbert Habib, Mathieu Lederlin, Frédéric Schnell, Erwan Donal
Left ventricular non-compaction (LV NC) is characterized by abnormal trabeculations that are mainly at the LV apex. Distinction between LV NC and non-specific dilated cardiomyopathies (DCMs) remains often challenging. We sought to find additive tools comparing the longitudinal strain characteristics of LVNC versus idiopathic DCM in a cohort of patients. 48 cases of LVNC (derivation cohort) were compared with 45 cases of DCM. Global and regional multi-layer (sub-endocardial, mid-wall, and sub-epicardial) LV longitudinal strain analysis was performed...
September 22, 2016: International Journal of Cardiovascular Imaging
ByungSu Yoo
Hypertension is the most common risk factor for systolic and diastolic heart failure. Based on population-attributable risks, hypertension has the greatest impact on the development of heart failure, accounting for 39% of HF events in men and 59% in women. Higher blood pressure, longer duration of hypertension and older age are associated with higher incidence of heart failure however, long term control of hypertension reduces the risk of heart failure. Thus current guideline pointed the hypertension as the single most important modifiable risk factor for heart failure...
September 2016: Journal of Hypertension
Deniz Akdis, Corinna Brunckhorst, Firat Duru, Ardan M Saguner
This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes...
August 2016: Arrhythmia & Electrophysiology Review
Yoshihisa Naruse, Akihiko Nogami, Yasutoshi Shinoda, Yuichi Hanaki, Yasuhiro Shirai, Shinya Kowase, Kenji Kurosaki, Takeshi Machino, Kenji Kuroki, Hiro Yamasaki, Miyako Igarashi, Yukio Sekiguchi, Kazutaka Aonuma
INTRODUCTION: Recent studies showed that J waves were associated with higher incidence of ventricular tachyarrhythmia (VT/VF) in patients with idiopathic ventricular fibrillation (VF) and myocardial infarction. We sought to assess the association between J waves and VT/VF in patients with nonischemic cardiomyopathy (NICM). METHODS AND RESULTS: We retrospectively enrolled 109 patients (79 men; mean age, 60 ± 15 years) with NICM who underwent implantable cardioverter defibrillator (ICD) implantation...
October 6, 2016: Journal of Cardiovascular Electrophysiology
E Stephenson, K Savvatis, S A Mohiddin, F M Marelli-Berg
T-cell-mediated immunity has been linked not only to a variety of heart diseases, including classic inflammatory diseases such as myocarditis and post-myocardial infarction (Dressler's) syndrome, but also to conditions without an obvious inflammatory component such as idiopathic dilated cardiomyopathy and hypertensive cardiomyopathy. It has been recently proposed that in all these conditions, the heart becomes the focus of T-cell-mediated autoimmune inflammation following ischaemic or infectious injury. For example, in acute myocarditis, an inflammatory disease of heart muscle, T-cell responses are thought to arise as a consequence of a viral infection...
September 2, 2016: British Journal of Pharmacology
Gianfranco Sinagra, Annamaria Iorio, Marco Merlo, Antonio Cannatà, Davide Stolfo, Elena Zambon, Concetta Di Nora, Stefania Paolillo, Giulia Barbati, Emanuela Berton, Cosimo Carriere, Damiano Magrì, Gaia Cattadori, Marco Confalonieri, Andrea Di Lenarda, Piergiuseppe Agostoni
BACKGROUND: Although cardiopulmonary exercise testing (CPET) is considered as an important tool in risk stratification of patients with heart failure (HF), prognostic data in the specific setting of Idiopathic Dilated Cardiomyopathy (iDCM) are still undetermined. The aim of the study was to test the prognostic value of CPET in a large cohort of iDCM patients. METHODS AND RESULTS: We analyzed 381 iDCM patients who consecutively performed CPET. The study end-point was a composite of cardiovascular death/urgent heart transplantation (CVD/HTx)...
November 15, 2016: International Journal of Cardiology
Annamaria Sandomenico, Annalia Focà, Luca Sanguigno, Andrea Caporale, Giuseppina Focà, Angelica Pignalosa, Giusy Corvino, Angela Caragnano, Antonio Paolo Beltrami, Giulia Antoniali, Gianluca Tell, Antonio Leonardi, Menotti Ruvo
Post-translational modifications (PTMs) strongly influence the structure and function of proteins. Lysine side chain acetylation is one of the most widespread PTMs, and it plays a major role in several physiological and pathological mechanisms. Protein acetylation may be detected by mass spectrometry (MS), but the use of monoclonal antibodies (mAbs) is a useful and cheaper option. Here, we explored the feasibility of generating mAbs against single or multiple acetylations within the context of a specific sequence...
August 25, 2016: MAbs
Yu Kang, Chen Chen, Xiaojing Chen, Xiaolin Sun, Fang Wang, Lingli Li, Qing Zhang, Yujia Liang
Adaptive enlargement of the mitral leaflet has been implied to participate in the pathogenesis of functional mitral regurgitation (FMR). The aim of the present study was to observe the elongation pattern of anterior mitral leaflets (AML) and posterior mitral leaflets (PML) in idiopathic dilated cardiomyopathy (DC) and to explore its relation with FMR. Forty normal controls (control group) and 97 patients with idiopathic DC (group DC 0-1+: 36 patients with no or only mild FMR; group DC >1+: 61 patients with more-than-mild FMR) were consecutively recruited...
October 1, 2016: American Journal of Cardiology
Matthew J Brody, Youngsook Lee
Leucine-rich repeat containing protein 10 (LRRC10) is a cardiomyocyte-specific member of the Leucine-rich repeat containing (LRRC) protein superfamily with critical roles in cardiac function and disease pathogenesis. Recent studies have identified LRRC10 mutations in human idiopathic dilated cardiomyopathy (DCM) and Lrrc10 homozygous knockout mice develop DCM, strongly linking LRRC10 to the molecular etiology of DCM. LRRC10 localizes to the dyad region in cardiomyocytes where it can interact with actin and α-actinin at the Z-disc and associate with T-tubule components...
2016: Frontiers in Physiology
Hak Ju Kim, Sungkyu Cho, Woong-Han Kim
Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most patients with heart failure treated with CRT are adults, middle-aged or older with idiopathic or ischemic dilated cardiomyopathy. We treated a 12-year-old boy, who was transferred after cardiac arrest, with dilated cardiomyopathy, left bundle-branch block, and ventricular tachycardia. We performed cardiac resynchronization therapy with a defibrillator (CRT-D). After CRT-D, left ventricular ejection fraction improved from 22% to 44% assessed by echocardiogram 1 year postoperatively...
August 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Yi-Meng Zhou, Xiao-Yong Dai, Ri-Tai Huang, Song Xue, Ying-Jia Xu, Xing-Biao Qiu, Yi-Qing Yang
Dilated cardiomyopathy (DCM) is the most prevalent form of primary cardiomyopathy in humans and is a leading cause of heart failure and sudden cardiac death. Genetic abnormalities have been demonstrated to be a major contributor to the development of DCM. However, DCM is a genetically heterogeneous disease, and the genetic basis underlying DCM in a significant proportion of patients remains unclear. In the current study, the coding exons and splicing junction sites of the T‑Box 20 (TBX20) gene, which encodes a T‑box transcription factor essential for cardiac morphogenesis and structural remodeling, were sequenced in 115 unrelated patients with idiopathic DCM, and a novel heterozygous mutation, p...
October 2016: Molecular Medicine Reports
Sheng-Na Li, Xin-Lin Zhang, Guo-Long Cai, Ruo-Wei Lin, He Jiang, Jian-Zhou Chen, Biao Xu, Wei Huang
BACKGROUND: Current risk stratification of idiopathic dilated cardiomyopathy (IDC) lacks sufficient sensitivity and specificity. The objective of this study was to investigate the predictive role of frontal QRS-T angles in IDC. METHODS: A prospective study with 509 IDC patients was performed from February 2008 to December 2013 in the Affiliated Drum Tower Hospital, Nanjing University School of Medicine. Baseline values and changes in QRS-T angles were recorded. Follow-up was conducted every 6 months...
August 20, 2016: Chinese Medical Journal
J K Nozynski, D Konecka-Mrowka, M Zakliczynski, E Zembala-Nozynska, D Lange, M Zembala
BACKGROUND: The role of BRCA1 in chronic ischemic episodes seems to be pivotal for adverse remodeling and development of ischemic cardiomyopathy, because of its role in DNA repair and apoptosis. The aim of this study was to investigate the role of BRCA-1 in idiopathic dilated cardiomyopathy (IDCM). MATERIAL AND METHODS: The study group (IDCM) comprised myocardial samples from hearts explanted before transplantation owing to IDCM in 10 males (age 44 ± 5.3 years) without clinical symptoms of ischemic heart disease...
June 2016: Transplantation Proceedings
Manuela Cabiati, Benedetta Svezia, Marco Matteucci, Luca Botta, Angela Pucci, Mauro Rinaldi, Chiara Caselli, Vincenzo Lionetti, Silvia Del Ry
Osteopontin (OPN) is a phosphoglycoprotein of cardiac extracellular matrix and it is still poorly defined whether its expression changes in failing heart of different origin. The full-length OPN-a and its isoforms (OPN-b, OPN-c) transcriptomic profile were evaluated in myocardium of patients with dilated or ischemic cardiomyopathy (DCM n = 8; LVEF% = 17.5±3; ICM n = 8; LVEF% = 19.5±5.2) and in auricle of valvular patients (VLP n = 5; LVEF%≥50), by Real-time PCR analysis. OPN-a and thrombin mRNA levels resulted significantly higher in DCM compared to ICM patients (DCM:31...
2016: PloS One
Ali Amr, Elham Kayvanpour, Farbod Sedaghat-Hamedani, Tiziano Passerini, Viorel Mihalef, Alan Lai, Dominik Neumann, Bogdan Georgescu, Sebastian Buss, Derliz Mereles, Edgar Zitron, Andreas E Posch, Maximilian Würstle, Tommaso Mansi, Hugo A Katus, Benjamin Meder
The search for a parameter representing left ventricular relaxation from non-invasive and invasive diagnostic tools has been extensive, since heart failure (HF) with preserved ejection fraction (HF-pEF) is a global health problem. We explore here the feasibility using patient-specific cardiac computer modeling to capture diastolic parameters in patients suffering from different degrees of systolic HF. Fifty eight patients with idiopathic dilated cardiomyopathy have undergone thorough clinical evaluation, including cardiac magnetic resonance imaging (MRI), heart catheterization, echocardiography, and cardiac biomarker assessment...
August 2016: Genomics, Proteomics & Bioinformatics
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