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idiopathic dilated cardiomyopathy

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https://www.readbyqxmd.com/read/27890770/fibrosis-and-fibrotic-gene-expression-in-pediatric-and-adult-patients-with-idiopathic-dilated-cardiomyopathy
#1
Kathleen C Woulfe, Austine K Siomos, Hieu Nguyen, Megan SooHoo, Csaba Galambo, Brian L Stauffer, Carmen Sucharov, Shelley Miyamoto
BACKGROUND: While fibrosis seems to be prognostic for adverse outcomes in adults with idiopathic dilated cardiomyopathy (IDC), little is known about the prevalence and development of fibrosis in pediatric IDC hearts. We hypothesize there is less activation of fibrosis at a molecular level in pediatric IDC hearts than in the failing adult heart. METHODS AND RESULTS: Pediatric hearts were analyzed histologically to determine the prevalence of fibrosis. Left ventricular tissue from adult and pediatric IDC hearts and adult and pediatric non-failing (NF) hearts were subjected to qRT-PCR to study the expression of important mRNAs that affect fibrosis...
November 24, 2016: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/27889554/familial-dilated-cardiomyopathy-a-multidisciplinary-entity-from-basic-screening-to-novel-circulating-biomarkers
#2
REVIEW
D de Gonzalo-Calvo, M Quezada, O Campuzano, A Perez-Serra, J Broncano, R Ayala, M Ramos, V Llorente-Cortes, S Blasco-Turrión, F J Morales, P Gonzalez, R Brugada, A Mangas, R Toro
Idiopathic dilated cardiomyopathy has become one of the most prevalent inherited cardiomyopathies over the past decades. Genetic screening of first-degree relatives has revealed that 30-50% of the cases have a familial origin. Similar to other heart diseases, familial dilated cardiomyopathy is characterized by a high genetic heterogeneity that complicates family studies. Cli'nical screening, 12-lead electrocardiogram and transthoracic echocardiogram are recommended for patients and first-degree family members...
November 8, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27886618/massive-parallel-sequencing-questions-the-pathogenic-role-of-missense-variants-in-dilated-cardiomyopathy
#3
Martin G Dalin, Pär G Engström, Emil G Ivarsson, Per Unneberg, Sara Light, Maria Schaufelberger, Thomas Gilljam, Bert Andersson, Martin O Bergo
BACKGROUND: Germline genetic variants are an important cause of dilated cardiomyopathy (DCM). However, recent sequencing studies have revealed rare variants in DCM-associated genes also in individuals without known heart disease. In this study, we investigate variant prevalence and genotype-phenotype correlations in Swedish DCM patients, and compare their genetic variants to those detected in reference cohorts. METHODS AND RESULTS: We sequenced the coding regions of 41 DCM-associated genes in 176 unrelated patients with idiopathic DCM and found 102 protein-altering variants with an allele frequency of <0...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27871237/a-unique-association-of-arrhythmogenic-right-ventricular-dysplasia-and-acute-myocarditis-as-assessed-by-cardiac-mri-a-case-report
#4
Andrea Ponsiglione, Marta Puglia, Carmine Morisco, Luigi Barbuto, Antonio Rapacciuolo, Mario Santoro, Letizia Spinelli, Bruno Trimarco, Alberto Cuocolo, Massimo Imbriaco
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms...
November 21, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27862767/dynamics-of-cerebral-blood-flow-in-patients-with-mild-non-ischaemic-heart-failure
#5
Christian D Erkelens, Haye H van der Wal, Bauke M de Jong, Jan-Willem Elting, Remco Renken, Marleen Gerritsen, Peter Jan van Laar, Vincent M van Deursen, Peter van der Meer, Dirk J van Veldhuisen, Adriaan A Voors, Gert-Jan Luijckx
AIMS: Heart failure (HF) is associated with tissue hypoperfusion and congestion leading to organ dysfunction. Although cerebral blood flow (CBF) is preserved over a wide range of perfusion pressures in healthy subjects, it is impaired in end-stage HF. We aimed to compare CBF, autoregulation, and cognitive function in patients with mild non-ischaemic HF with healthy controls. METHODS AND RESULTS: Fifteen patients with mild idiopathic dilated cardiomyopathy and 15 matched healthy controls were studied...
November 14, 2016: European Journal of Heart Failure
https://www.readbyqxmd.com/read/27832288/screening-first-degree-relatives-of-patients-with-idiopathic-dilated-cardiomyopathy
#6
M Sefa Okten, K Tuluce, S Yakar Tuluce, S Kilic, H Soner Kemal, A Sayin, O Vuran, B Yagmur, I Mutlu, E Simsek, C Soydas Cinar, C Gurgun
BACKGROUND: This study evaluated whether subclinical myocardial dysfunction occurs in first-degree relatives of patients with idiopathic dilated cardiomyopathy (IDCM), using strain echocardiographic imaging, before apparent left ventricular (LV) failure is observed. PATIENTS AND METHODS: The study comprised 77 subjects aged 16-63 years who had first-degree relatives with a previous or new diagnosis of IDCM. LV myocardial deformation parameters of the first-degree relatives with normal LVEF (≥55%) values, as assessed using 2D echocardiography, were evaluated...
November 10, 2016: Herz
https://www.readbyqxmd.com/read/27813223/truncating-titin-mutations-are-associated-with-a-mild-and-treatable-form-of-dilated-cardiomyopathy
#7
Joeri A Jansweijer, Karin Nieuwhof, Francesco Russo, Edgar T Hoorntje, Jan D H Jongbloed, Ronald H Lekanne Deprez, Alex V Postma, Marieke Bronk, Ingrid A W van Rijsingen, Simone de Haij, Elena Biagini, Paul L van Haelst, Jan van Wijngaarden, Maarten P van den Berg, Arthur A M Wilde, Marcel M A M Mannens, Rudolf A de Boer, Karin Y van Spaendonck-Zwarts, J Peter van Tintelen, Yigal M Pinto
AIMS: Truncating titin mutations (tTTN) occur in 25% of dilated cardiomyopathy (DCM) cases, but the phenotype and severity of disease they cause have not yet been systematically studied. We studied whether tTTN variants are associated with a clinically distinguishable form of DCM. METHODS AND RESULTS: We compared clinical data on DCM probands and relatives with a tTTN mutation (n = 45, n = 73), LMNA mutation (n = 28, n = 29), and probands who tested negative for both genes [idiopathic DCM (iDCM); n = 60]...
November 3, 2016: European Journal of Heart Failure
https://www.readbyqxmd.com/read/27792707/-hypertrophic-miocardiopathy-an-historical-and-anatomopathological-review
#8
Manlio F Márquez, Teresita de Jesús Ruíz-Siller, Rosario Méndez-Ramos, Erick Karabut, Alberto Aranda-Fraustro, Silvia Jiménez-Becerra
Hypertrophic cardiomyopathy (HCM) is characterized by the presence of an abnormal hypertrophy of the left ventricle (LV), without dilation, and in the absence of any condition or another cardiac or systemic disease capable of inducing such hypertrophy. This primary or idiopathic hypertrophy can occur with or without dynamic obstruction (induced by exercise) of the LV outflow tract, so in its natural history two fundamental aspects are highlighted: the production of symptoms by blocking the LV outflow tract and the occurrence of sudden cardiac death secondary to ventricular arrhythmias...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27753837/sy-05-3-hypertension-and-bp-effect-in-hf-paradox-or-phenomenon
#9
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
https://www.readbyqxmd.com/read/27736720/genetic-basis-of-dilated-cardiomyopathy
#10
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...
December 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27717935/clinical-examples-of-left-ventricular-non-compaction-in-adults
#11
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
https://www.readbyqxmd.com/read/27711126/integrated-left-ventricular-global-transcriptome-and-proteome-profiling-in-human-end-stage-dilated-cardiomyopathy
#12
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
https://www.readbyqxmd.com/read/27692597/usefulness-of-serial-n-terminal-pro-b-type-natriuretic-peptide-measurements-to-predict-cardiac-death-in-acute-and-chronic-dilated-cardiomyopathy-in-children
#13
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
https://www.readbyqxmd.com/read/27659478/left-ventricular-non-compaction-and-idiopathic-dilated-cardiomyopathy-the-significant-diagnostic-value-of-longitudinal-strain
#14
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
https://www.readbyqxmd.com/read/27642890/sy-05-3-hypertension-and-bp-effect-in-hf-paradox-or-phenomenon
#15
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
https://www.readbyqxmd.com/read/27617087/arrhythmogenic-cardiomyopathy-electrical-and-structural-phenotypes
#16
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
https://www.readbyqxmd.com/read/27593399/j-waves-are-associated-with-the-increased-occurrence-of-life-threatening-ventricular-tachyarrhythmia-in-patients-with-nonischemic-cardiomyopathy
#17
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
https://www.readbyqxmd.com/read/27590129/t-cell-immunity-in-myocardial-inflammation-pathogenic-role-and-therapeutic-manipulation
#18
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
https://www.readbyqxmd.com/read/27561166/prognostic-value-of-cardiopulmonary-exercise-testing-in-idiopathic-dilated-cardiomyopathy
#19
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
https://www.readbyqxmd.com/read/27560983/monoclonal-antibodies-against-pools-of-mono-and-polyacetylated-peptides-selectively-recognize-acetylated-lysines-within-the-context-of-the-original-antigen
#20
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
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