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idiopathic cardiomyopathy

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https://www.readbyqxmd.com/read/27909515/myocardial-biopsy-in-idiopathic%C3%A2-atrial-fibrillation-and-other-arrhythmias-nosological-diagnosis-clinical-and-morphological-parallels-and-treatment
#1
O V Blagova, A V Nedostup, E A Kogan, V A Sulimov, S A Abugov, A G Kupriyanova, V A Zaydenov, A E Donnikov, E V Zaklyazminskaya, E A Okisheva
BACKGROUND: The nosological nature of "idiopathic" arrhythmias and the effect of etiotropic and pathogenetic treatment are often unknown. METHODS AND RESULTS: 19 patients (42.6±11.3 years, 9 women) with atrial fibrillation (n = 16), supraventricular (n = 10) and ventricular (n = 4) premature beats, supraventricular (n = 2) and ventricular tachycardia (n = 1), left bundle branch block (n= 2), AV block (n = 2) without structural heart changes. Viruses were identified (polymerase chain reaction, PCR) along with measurement of anti-heart antibodies (AHA) and endomyocardial biopsy (EMB)...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27890770/fibrosis-and-fibrotic-gene-expression-in-pediatric-and-adult-patients-with-idiopathic-dilated-cardiomyopathy
#2
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
#3
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
#4
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
#5
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
#6
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/27860029/different-correlates-but-similar-prognostic-implications-for-right-ventricular-dysfunction-in-heart-failure-patients-with-reduced-or-preserved-ejection-fraction
#7
Stefano Ghio, Marco Guazzi, Angela Beatrice Scardovi, Catherine Klersy, Francesco Clemenza, Erberto Carluccio, Pier Luigi Temporelli, Andrea Rossi, Pompilio Faggiano, Egidio Traversi, Olga Vriz, Frank Lloyd Dini
AIMS: To evaluate whether the clinical and echocardiographic correlates and the prognostic significance of right ventricular (RV) dysfunction are different in heart failure patients with reduced (HFrEF), mid-range (HFmrEF), or preserved (HFpEF) left ventricular ejection fraction. METHODS AND RESULTS: The study included 1663 patients with heart failure caused by ischaemic or hypertensive heart disease or by idiopathic cardiomyopathy. Left ventricular ejection fraction was <40% in 1123 patients (HFrEF), 40-49% in 156 patients (HFmrEF) and ≥50% in 384 patients (HFpEF)...
November 17, 2016: European Journal of Heart Failure
https://www.readbyqxmd.com/read/27832288/screening-first-degree-relatives-of-patients-with-idiopathic-dilated-cardiomyopathy
#8
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/27818482/high-dose-%C3%AE-blocker-therapy-triggers-additional-reverse-remodeling-in-patients-with-idiopathic-non-ischemic-cardiomyopathy
#9
Daisuke Nitta, Koichiro Kinugawa, Teruhiko Imamura, Naoko P Kato, Issei Komuro
Carvedilol has established its evidence to improve prognosis and facilitate left ventricular reverse remodeling (LVRR) in heart failure patients with reduced left ventricular ejection fraction (LVEF), and many studies have supported its dose-dependency. However, there are few studies demonstrating the effect of high dose carvedilol in Japan. We enrolled 23 patients with idiopathic non-ischemic cardiomyopathy, in whom LVEF remained 45% or less despite 20 mg/ day of carvedilol therapy for > 3 months. After high dose (40 mg/day) carvedilol therapy for > 3 months, LVEF improved (+9...
November 4, 2016: International Heart Journal
https://www.readbyqxmd.com/read/27813223/truncating-titin-mutations-are-associated-with-a-mild-and-treatable-form-of-dilated-cardiomyopathy
#10
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/27810292/special-electrophysiological-characteristics-of-pediatric-idiopathic-ventricular-tachycardia
#11
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Kun-Lang Wu, Wei-Chieh Tseng, Ming-Tai Lin, Chien-Chih Chang, Jou-Kou Wang, Mei-Hwan Wu
BACKGROUND: Idiopathic ventricular tachycardia (VT) is an important pediatric arrhythmia but relevant studies in Asian cohort are rare. We determined the electrophysiological characteristics and outcomes of this rhythm disturbance. METHODS: From an institutional database, patients aged <18years without structural heart disease who have sustained monomorphic VT (longer than 30s) during 1995-2016 were enrolled. RESULTS: We enrolled 57 idiopathic VT patients (male:female=31:26)...
October 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27798053/relationship-between-aetiology-and-left-ventricular-systolic-dysfunction-in-hypertrophic-cardiomyopathy
#12
Stefania Rosmini, Elena Biagini, Costantinos O'Mahony, Heerajnarain Bulluck, Niccolo' Ruozi, Luis R Lopes, Oliver Guttmann, Patricia Reant, Cristina C Quarta, Antonis Pantazis, Maria Tome-Esteban, William J Mckenna, Claudio Rapezzi, Perry M Elliott
BACKGROUND: Severe left ventricular (LV) systolic dysfunction is an uncommon complication of hypertrophic cardiomyopathy (HCM) that is associated with poor prognosis. Small observational series suggest that patients with rare causes of HCM are more likely to develop systolic impairment than those with idiopathic disease or mutations in cardiac sarcomeric protein genes. The aim of this study was to test this hypothesis by comparing the prevalence of systolic dysfunction and its impact on prognosis in patients with different causes of HCM...
October 24, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27792707/-hypertrophic-miocardiopathy-an-historical-and-anatomopathological-review
#13
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/27788994/incessant-left-ventricular-tachycardia-of-unusual-etiology
#14
Praloy Chakraborty, H S Isser, Sudheer Arava, Kausik Mandal
Apart from Coronary artery disease, left ventricular tachycardia may result from cardiac sarcoidosis, left ventricular tumor, chagas disease and idiopathic left ventricular tachycardia. We report a rare case of incessant left ventricular tachycardia resulting from left dominant arrhythmogenic cardiomyopathy.
May 2016: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/27753837/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/27746287/nonobstructive-hypertrophic-cardiomyopathy-out-of-the-shadows-known-from-the-beginning-but-largely-ignored%C3%A2-until-now
#16
Barry J Maron, Ethan J Rowin, Martin S Maron, Eugene Braunwald
Hypertrophic cardiomyopathy was first recognized as a disease of obstruction to left ventricular outflow, hence its early names and acronyms such as idiopathic hypertrophic subaortic obstruction. The nonobstructive subset of patients, incapable of developing mechanical impedance to left ventricular outflow at rest or with physiologic exercise, was initially recognized by the Braunwald group at the National Institutes of Health >50 years ago in the pre-imaging era and is now recognized as comprising about one-third of hypertrophic cardiomyopathy patients...
October 13, 2016: American Journal of Medicine
https://www.readbyqxmd.com/read/27736720/genetic-basis-of-dilated-cardiomyopathy
#17
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
#18
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
#19
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/27692971/incidence-and-etiology-of-sudden-cardiac-arrest-and-death-in-high-school-athletes-in-the-united-states
#20
Kimberly G Harmon, Irfan M Asif, Joseph J Maleszewski, David S Owens, Jordan M Prutkin, Jack C Salerno, Monica L Zigman, Rachel Ellenbogen, Ashwin L Rao, Michael J Ackerman, Jonathan A Drezner
OBJECTIVE: To determine the incidence and etiology of sudden cardiac arrest and death (SCA/D) in US high school athletes. PATIENTS AND METHODS: A prospective media database of SCA/D was queried for cases aged 14 to 18 years from 7 states over 6 school years (September 1, 2007, to August 30, 2013). Event details were investigated to determine participation on a high school athletic team, sex, sport, and occurrence during school-sponsored activity or exertion. National sports participation numbers were used and a conversion factor was applied to account for multisport athletes...
November 2016: Mayo Clinic Proceedings
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