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idiopathic cardiomyopathy

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https://www.readbyqxmd.com/read/28902616/mef2c-loss-of-function-mutation-associated-with-familial-dilated-cardiomyopathy
#1
Fang Yuan, Zhao-Hui Qiu, Xing-Hua Wang, Yu-Min Sun, Jun Wang, Ruo-Gu Li, Hua Liu, Min Zhang, Hong-Yu Shi, Liang Zhao, Wei-Feng Jiang, Xu Liu, Xing-Biao Qiu, Xin-Kai Qu, Yi-Qing Yang
BACKGROUND: The MADS-box transcription factor myocyte enhancer factor 2C (MEF2C) is required for the cardiac development and postnatal adaptation and in mice-targeted disruption of the MEF2C gene results in dilated cardiomyopathy (DCM). However, in humans, the association of MEF2C variation with DCM remains to be investigated. METHODS: The coding regions and splicing boundaries of the MEF2C gene were sequenced in 172 unrelated patients with idiopathic DCM. The available close relatives of the index patient harboring an identified MEF2C mutation and 300 unrelated, ethnically matched healthy individuals used as controls were genotyped for MEF2C...
September 13, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28885342/clinical-features-of-idiopathic-restrictive-cardiomyopathy-a-retrospective-multicenter-cohort-study-over-2-decades
#2
MULTICENTER STUDY
Jung Ae Hong, Min-Seok Kim, Min-Su Cho, Hyo In Choi, Duk-Hyun Kang, Sang-Eun Lee, Ga-Yeon Lee, Eun-Seok Jeon, Jae-Yeong Cho, Kye-Hun Kim, Byung-Su Yoo, Jong-Young Lee, Won-Jang Kim, Kyung-Hee Kim, Wook-Jin Chung, Ju-Hee Lee, Myeong-Chan Cho, Jae-Joong Kim
Idiopathic restrictive cardiomyopathy (RCMP) has not been fully understood because this disease is difficult to diagnose. The present study aimed to assess the clinical profile and outcome of idiopathic RCMP from a multicenter cohort.This investigation is a retrospective study of consecutive patients with idiopathic RCMP at 10 centers in Korea between 1990 and 2010. We evaluated the clinical characteristics of the patients and prognostic factors associated with mortality using multivariate Cox proportional hazards regression analyses...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28883920/exogenous-t3-toxicosis-following-consumption-of-a-contaminated-weight-loss-supplement
#3
R D'Arcy, M McDonnell, K Spence, C H Courtney
A 42-year-old male presented with a one-week history of palpitations and sweating episodes. The only significant history was of longstanding idiopathic dilated cardiomyopathy. Initial ECG demonstrated a sinus tachycardia. Thyroid function testing, undertaken as part of the diagnostic workup, revealed an un-measureable thyroid-stimulating hormone (TSH) and free thyroxine (T4). Upon questioning the patient reported classical thyrotoxic symptoms over the preceding weeks. Given the persistence of symptoms free tri-iodothyronine (T3) was measured and found to be markedly elevated at 48...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28877291/survival-and-cardiovascular-outcomes-of-patients-with-secondary-mitral-regurgitation-a-systematic-review-and-meta-analysis
#4
Anna Sannino, Robert L Smith, Gabriele G Schiattarella, Bruno Trimarco, Giovanni Esposito, Paul A Grayburn
Importance: The outcomes of patients with left ventricular (LV) dysfunction and secondary mitral regurgitation (SMR) are still controversial. Objective: To clarify the role of SMR in the outcomes of patients with ischemic or idiopathic cardiomyopathies. Data Sources: MEDLINE, ISI Web of Science, and Scopus databases were searched for studies published up to March 2017. Study Selection: Studies reporting data on outcomes in patients with SMR were included...
September 6, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28868530/addressing-health-disparities-in-idiopathic-dilated-cardiomyopathy
#5
Argun D Can, Steven S Coughlin
No abstract text is available yet for this article.
June 2017: Jacobs Journal of Community Medicine
https://www.readbyqxmd.com/read/28859293/impaired-calcium-homeostasis-is-associated-with-sudden-cardiac-death-and-arrhythmias-in-a-genetic-equivalent-mouse-model-of-the-human-hrc-ser96ala-variant
#6
Christos Tzimas, Daniel M Johnson, Demetrio J Santiago, Elizabeth Vafiadaki, Demetrios A Arvanitis, Constantinos H Davos, Aimilia Varela, Nikolaos C Athanasiadis, Constantinos Dimitriou, Michalis Katsimpoulas, Stephan Sonntag, Mariya Kryzhanovska, Doron Shmerling, Stephan E Lehnart, Karin R Sipido, Evangelia G Kranias, Despina Sanoudou
Aims: The histidine-rich calcium-binding protein (HRC) Ser96Ala variant has previously been identified as a potential biomarker for ventricular arrhythmias and sudden cardiac death in patients with idiopathic dilated cardiomyopathy. Herein, the role of this variant in cardiac pathophysiology is delineated through a novel mouse model, carrying the human mutation in the homologous mouse position. Methods and results: The mouse HRC serine 81, homologous to human HRC serine 96, was mutated to alanine, using knock-in gene targeting...
September 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28855115/catheter-ablation-versus-medical-rate-control-in-atrial-fibrillation-and-systolic-dysfunction-camera-mri
#7
Sandeep Prabhu, Andrew J Taylor, Ben T Costello, David M Kaye, Alex Aj McLellan, Aleksandr Voskoboinik, Hariharan Sugumar, Siobhan M Lockwood, Michael B Stokes, Bhupesh Pathik, Chrishan J Nalliah, Geoff R Wong, Sonia M Azzopardi, Sarah Gutman, Geoffrey Lee, Jamie Layland, Justin A Mariani, Liang-Han Ling, Jonathan M Kalman, Peter M Kistler
BACKGROUND: Atrial fibrillation (AF) and left ventricular systolic dysfunction (LVSD) frequently co-exist despite adequate rate-control. Existing randomized studies of AF and LVSD of varying etiologies have demonstrated modest benefits with a rhythm control strategy. OBJECTIVE: To determine whether catheter ablation (CA) for AF could improve LVSD compared to medical rate-control (MRC) where the etiology of the LVSD was unexplained, apart from the presence of AF...
August 22, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28831623/structural-consequences-of-mutations-associated-with-idiopathic-restrictive-cardiomyopathy
#8
Svetlana Tarnovskaya, Artem Kiselev, Anna Kostareva, Dmitrij Frishman
Idiopathic restrictive cardiomyopathy (RCM, MIM# 115210) is the least common type of cardiomyopathies, often of genetic origin. Recently we described a spectrum of variants-classified as pathogenic, likely pathogenic and variants of unknown significance-in 24 patients suffering from idiopathic RCM. Pathogenic variants, detected in half of the RCM cases, were found in sarcomeric and cytoskeletal genes that have a predominant role in the development of RCM. Here we have analyzed the structural consequences of these missense variants and predicted their effect on the function of three large groups of domains: intrinsically disordered regions (IDRs), fibronectin-type III (FnIII) domains, and immunoglobulin-like (Ig) domains...
August 22, 2017: Amino Acids
https://www.readbyqxmd.com/read/28821499/primary-ventricular-tachycardia-in-paediatric-population-in-a-tertiary-centre
#9
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Wei-Chieh Tseng, Kun-Lang Wu, Jou-Kou Wang, Mei-Hwan Wu
OBJECTIVE: To delineate the outcome of ventricular tachycardia (VT) in the paediatric population. METHODS: Patients who developed sustained VT between the ages of 0 and 18 years in a referral centre from 1991 to 2015 were enrolled. RESULTS: A total of 116 patients (67 male/49 female) had documented VT, and 53 (46%) had associated heart disease, namely cardiomyopathy in 20 (17%), structural heart disease in 19 (16%) and channelopathy in 14 (12%), and some of them presented with two types of associated heart disease...
August 18, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28818336/association-between-premature-ventricular-complexes-during-exercise-long-term-occurrence-of-life-threatening-arrhythmia-and-mortality
#10
Jorge Aviles-Rosales, Hermes Ilarraza-Lomeli, Marianna Garcia-Saldivia, Jessica Rojano-Castillo, Maria-Dolores Rius-Suarez, Juan-Pablo Nunez-Urquiza, Pedro Iturralde
OBJECTIVE: Exercise-induced premature ventricular complexes (EiPVCs) are often considered as benign arrhythmias, nevertheless they are associated with a high risk of all-cause death in general healthy population. However, an intermediate pathophysiological process remains unclear, particularly in patients with known cardiovascular disease. The aim of this study was to find an association between EiPVCs, the occurrence of life-threatening ventricular arrhythmias (LACO) and all-cause mortality in patients with cardiovascular disease...
August 14, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28808788/shocks-after-implantable-cardioverter-defibrillator-implantation-in-idiopathic-cardiomyopathy-patients-a-myocardial-biopsy-study
#11
Erdal Safak, Giuseppe D Ancona, Heinz-Peter Schultheiss, Uwe Kühl, Stephan Kische, Hilmi Kaplan, Hüseyin Ince, Jasmin Ortak
Prediction of follow-up shock is crucial to stratify patients with dilated cardiomyopathy (DCM) requiring implantable cardioverter defibrillator (ICD). The objective of the article is to assess the predictive value of endo-myocardial biopsy (EMB) towards ICD shock and follow-up mortality. A series of patients with DCM scheduled for ICD implantation underwent EMB to further determine the genesis of DCM. Presence of fibrosis and inflammation was documented and related to outcomes. A total of 240 patients were referred for ICD as primary (56%) and secondary (44%) prophylaxis...
August 14, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28803350/comparison-of-mitral-competence-after-mitral-repair-with-papillary-muscle-approximation-versus-papillary-muscle-relocation-for-functional-mitral-regurgitation
#12
Koji Furukawa, Mitsuhiro Yano, Eisaku Nakamura, Masakazu Matsuyama, Masanori Nishimura, Katsuya Kawagoe, Kunihide Nakamura
The purpose of this study was to evaluate the surgical results of papillary muscle approximation (PMA) and papillary muscle relocation (PMR) for functional mitral regurgitation (FMR) and to compare the effects of both procedures on the change in mitral regurgitation (MR) and echocardiogram parameters associated with tethering. Eighteen patients with moderate-to-severe FMR (MR grade ≥2) who underwent PMA or PMR were retrospectively analyzed. Underlying diseases were ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and aortic valve disease for seven, six, and five patients, respectively...
August 12, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28797313/intravenous-immunoglobulins-in-children-with-new-onset-dilated-cardiomyopathy
#13
Josephine F Heidendael, Suzanne L Den Boer, Joanne G Wildenbeest, Michiel Dalinghaus, Bart Straver, Dasja Pajkrt
BACKGROUND: Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded...
August 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28771538/a-novel-noninvasive-surface-ecg-analysis-using-interlead-qrs-dispersion-in-arrhythmogenic-right-ventricular-cardiomyopathy
#14
Wan-Hsin Hsieh, Chin-Yu Lin, Abigail Louise D Te, Men-Tzung Lo, Cheng-I Wu, Fa-Po Chung, Yi-Chung Chang, Shih-Lin Chang, Chen Lin, Li-Wei Lo, Yu-Feng Hu, Jo-Nan Liao, Yun-Yu Chen, Shih-Jie Jhuo, Sunu Budhi Raharjo, Yenn-Jiang Lin, Shih-Ann Chen
BACKGROUND: This study investigated the feasibility of using the precordial surface ECG lead interlead QRS dispersion (IQRSD) in the identification of abnormal ventricular substrate in arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: Seventy-one consecutive patients were enrolled and reclassified into 4 groups: definite ARVC with epicardial ablation (Group 1), ARVC with ventricular tachycardia (VT, Group 2), idiopathic right ventricular outflow tract VT without ARVC (Group 3), and controls without VT (Group 4)...
2017: PloS One
https://www.readbyqxmd.com/read/28744986/complete-recovery-of-fulminant-peripartum-cardiomyopathy-on-mechanical-circulatory-support-combined-with-high-dose-bromocriptine-therapy
#15
Patrick Horn, Diyar Saeed, Payam Akhyari, Denise Hilfiker-Kleiner, Malte Kelm, Ralf Westenfeld
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure due to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found. We report a case of a woman with PPCM who developed a critical cardiogenic shock with repeated cardiopulmonary resuscitation. We show for the first time that mechanical circulatory support combined with high-dose bromocriptine therapy to suppress systemic prolactin levels may serve as an effective therapeutic option in patients with fulminant PPCM and cardiogenic shock...
July 25, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28735322/viral-infection-of-the-heart-pathogenesis-and-diagnosis
#16
Mateusz Kuffner, Agnieszka Pawlak, Maciej Przybylski
Viral infections of the heart cause serious clinical problems, either as infectious myocarditis, which usually is a consequence of acute infection or as idiopathic dilated cardiomyopathy, resulting rather from a chronic infection. This minireview presents an up-to-date view on pathomechanisms of viral infection of the heart tissues, the role of immune system in controlling infectious process at its various stages and current possibilities of recognizing viral infection of the heart with use of both cardiological and virological methods...
January 2, 2017: Polish Journal of Microbiology
https://www.readbyqxmd.com/read/28708714/sleep-medications-containing-melatonin-can-potentially-induce-ventricular-arrhythmias-in-structurally-normal-hearts-a-two-patient-report
#17
L J de Vries, T Géczy, T Szili-Torok
Idiopathic ventricular arrhythmias (IVAs) are relatively common in the general population and usually have a good prognosis. However, frequent PVCs can lower quality of life (in symptomatic cases) and can cause cardiomyopathy and sudden cardiac death. In this report, we demonstrate a novel trigger for IVAs. Melatonin use for treating sleep-disorders has increased significantly in recent years. We provide here the first human evidence of its pro-arrhythmic effect by presenting two patients (with normal myocardium) with symptomatic PVCs, while on melatonin...
July 13, 2017: Journal of Cardiovascular Pharmacology
https://www.readbyqxmd.com/read/28701682/prognostic-impact-of-segmental-wall-motion-abnormality-in-patients-with-idiopathic-dilated-cardiomyopathy
#18
Yuichiro Iida, Takayuki Inomata, Toyoji Kaida, Teppei Fujita, Yuki Ikeda, Takeru Nabeta, Shunsuke Ishii, Toshimi Koitabashi, Ichiro Takeuchi, Junya Ako
The clinical impact of left ventricular (LV) segmental wall motion abnormalities (SWMA) in patients with idiopathic dilated cardiomyopathy (IDCM) has not been well elucidated.Among 100 consecutive IDCM patients with follow-up visits, we enrolled 85 after excluding those with left bundle branch block and/or ventricular pacemaker implantation. LV wall motion was assessed using left ventriculography scored for 7 segments according to the American Heart Association classification as follows: 0, normokinesis; 1, hypokinesis; 2, akinesis; and 3, dyskinesis...
August 3, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28696268/personalizing-risk-stratification-for-sudden-death-in-dilated-cardiomyopathy-the-past-present-and-future
#19
REVIEW
Brian P Halliday, John G F Cleland, Jeffrey J Goldberger, Sanjay K Prasad
Results from the DANISH Study (Danish Study to Assess the Efficacy of ICDs in Patients With Non-Ischemic Systolic Heat Failure on Mortality) suggest that for many patients with dilated cardiomyopathy (DCM), implantable cardioverter-defibrillators do not increase longevity. Accurate identification of patients who are more likely to die of an arrhythmia and less likely to die of other causes is required to ensure improvement in outcomes and wise use of resources. Until now, left ventricular ejection fraction has been used as a key criterion for selecting patients with DCM for an implantable cardioverter-defibrillator for primary prevention purposes...
July 11, 2017: Circulation
https://www.readbyqxmd.com/read/28684747/mir-320a-as-a-potential-novel-circulating-biomarker-of-arrhythmogenic-cardiomyopathy
#20
Elena Sommariva, Yuri D'Alessandra, Floriana Maria Farina, Michela Casella, Fabio Cattaneo, Valentina Catto, Mattia Chiesa, Ilaria Stadiotti, Silvia Brambilla, Antonio Dello Russo, Corrado Carbucicchio, Giulia Vettor, Daniela Riggio, Maria Teresa Sandri, Andrea Barbuti, Gianluca Vernillo, Manuela Muratori, Matteo Dal Ferro, Gianfranco Sinagra, Silvia Moimas, Mauro Giacca, Gualtiero Ivanoe Colombo, Giulio Pompilio, Claudio Tondo
Diagnosis of Arrhythmogenic CardioMyopathy (ACM) is challenging and often late after disease onset. No circulating biomarkers are available to date. Given their involvement in several cardiovascular diseases, plasma microRNAs warranted investigation as potential non-invasive diagnostic tools in ACM. We sought to identify circulating microRNAs differentially expressed in ACM with respect to Healthy Controls (HC) and Idiopathic Ventricular Tachycardia patients (IVT), often in differential diagnosis. ACM and HC subjects were screened for plasmatic expression of 377 microRNAs and validation was performed in 36 ACM, 53 HC, 21 IVT...
July 6, 2017: Scientific Reports
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