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"hypertrophic cardiomyopathy"

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https://www.readbyqxmd.com/read/29353047/clinical-profile-of-nonresponders-to-surgical-myectomy-with-obstructive-hypertrophic-cardiomyopathy
#1
Sophie Wells, Ethan J Rowin, Griffin Boll, Hassan Rastegar, Wendy Wang, Martin S Maron, Barry J Maron
BACKGROUND: In the vast majority of patients with obstructive hypertrophic cardiomyopathy and drug-refractory heart failure, surgical myectomy results in reversal of heart failure symptoms. However, a small patient subgroup fails to experience sustained postoperative improvement despite relief of left ventricular outflow obstruction. Clinical profile of such patients has not been well defined. METHODS: Consecutive obstructive hypertrophic cardiomyopathy patients undergoing myectomy at Tufts Medical Center for drug refractory NYHA III/IV heart failure symptoms, 2004 to 2017, were followed postoperatively for 2...
January 15, 2018: American Journal of Medicine
https://www.readbyqxmd.com/read/29348686/defects-in-the-mitochondrial-trna-modification-enzymes-mto1-and-gtpbp3-promote-different-metabolic-reprogramming-through-a-hif-ppar%C3%AE-ucp2-ampk-axis
#2
Rachid Boutoual, Salvador Meseguer, Magda Villarroya, Elena Martín-Hernández, Mohammed Errami, Miguel A Martín, Marta Casado, M-Eugenia Armengod
Human proteins MTO1 and GTPBP3 are thought to jointly catalyze the modification of the wobble uridine in mitochondrial tRNAs. Defects in each protein cause infantile hypertrophic cardiomyopathy with lactic acidosis. However, the underlying mechanisms are mostly unknown. Using fibroblasts from an MTO1 patient and MTO1 silenced cells, we found that the MTO1 deficiency is associated with a metabolic reprogramming mediated by inactivation of AMPK, down regulation of the uncoupling protein 2 (UCP2) and transcription factor PPARγ, and activation of the hypoxia inducible factor 1 (HIF-1)...
January 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29343710/phenotypic-diversity-identified-by-cardiac-magnetic-resonance-in-a-large-hypertrophic-cardiomyopathy-family-with-a-single-myh7-mutation
#3
Jie Wang, Ke Wan, Jiayu Sun, Weihao Li, Hong Liu, Yuchi Han, Yucheng Chen
Limited data is available on phenotypic variations with the same genotype in hypertrophic cardiomyopathy (HCM). The present study aims to explore the relationship between genotype and phenotype characterized by cardiovascular magnetic resonance (CMR) in a large Chinese family. A proband diagnosed with HCM from a multigenerational family underwent next-generation sequencing based on a custom sureSelect panel, including 117 candidate pathogenic genes associated with cardiomyopathies. All genetic results were confirmed by the Sanger sequencing method...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29343659/cardiac-mr-imaging-of-hypertrophic-cardiomyopathy-techniques-findings-and-clinical-relevance
#4
Yasuo Amano, Mitsunobu Kitamura, Hitoshi Takano, Fumi Yanagisawa, Masaki Tachi, Yasuyuki Suzuki, Shinichiro Kumita, Morimasa Takayama
Hypertrophic cardiomyopathy (HCM) is a relatively common myocardial genetic disease having a wide variety of symptoms and prognoses. The most serious complications of HCM are sudden cardiac death induced by ventricular arrhythmia or inappropriate changes in blood pressure, and heart failure. Cardiac MR imaging is a valuable imaging method for detecting HCM because of its accurate measurement of wall thickness and myocardial mass without limited view and the unique ability of late gadolinium enhancement (LGE) to identify myocardial fibrosis related to the prognosis of HCM...
January 18, 2018: Magnetic Resonance in Medical Sciences: MRMS
https://www.readbyqxmd.com/read/29332907/patients-characteristics-and-clinical-course-of-hypertrophic-cardiomyopathy-in-a-regional-japanese-cohort%C3%A3-results-from-kochi-ryoma-study
#5
Toru Kubo, Takayoshi Hirota, Yuichi Baba, Yuri Ochi, Asa Takahashi, Naohito Yamasaki, Naohisa Hamashige, Katsuhito Yamamoto, Fumiaki Kondo, Kanji Bando, Eisuke Yamada, Takashi Furuno, Toshikazu Yabe, Yoshinori L Doi, Hiroaki Kitaoka
BACKGROUND: There have been few studies on the clinical course of hypertrophic cardiomyopathy (HCM) in a community-based patient cohort in Japan.Methods and Results:In 2004, we established a cardiomyopathy registration network in Kochi Prefecture (the Kochi RYOMA study) that consisted of 9 hospitals, and finally, 293 patients with HCM were followed. The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were male. HCM-related deaths occurred in 23 patients during a mean follow-up period of 6...
January 13, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29332241/atrial-fibrillation-is-associated-with-sudden-cardiac-death-a-systematic-review-and-meta-analysis
#6
Pattara Rattanawong, Sikarin Upala, Tanawan Riangwiwat, Veeravich Jaruvongvanich, Anawin Sanguankeo, Wasawat Vutthikraivit, Eugene H Chung
PURPOSE: Recent studies suggest that atrial fibrillation (AF) is associated with increased cardiovascular risk and mortality including sudden cardiac death (SCD). According to the Cardiovascular Heath Study cohort, the incident rate of SCD was higher in the AF population (2.9 per 1000 per year) compared with non-AF controls (1.3 per 1000 per year). In this study, we performed a systematic review and meta-analysis to explore the association between AF and SCD. METHODS: We comprehensively searched the databases of MEDLINE and EMBASE from inception to January 2017...
January 13, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29331171/the-genotypic-and-phenotypic-spectrum-of-mto1-deficiency
#7
James J O'Byrne, Maja Tarailo-Graovac, Aisha Ghani, Michael Champion, Charu Deshpande, Ali Dursun, Riza K Ozgul, Peter Freisinger, Ian Garber, Tobias B Haack, Rita Horvath, Ivo Barić, Ralf A Husain, Leo A J Kluijtmans, Urania Kotzaeridou, Andrew A Morris, Colin J Ross, Saikat Santra, Jan Smeitink, Mark Tarnopolsky, Saskia B Wortmann, Johannes A Mayr, Michaela Brunner-Krainz, Holger Prokisch, Wyeth W Wasserman, Ron A Wevers, Udo F Engelke, Richard J Rodenburg, Teck Wah Ting, Robert McFarland, Robert W Taylor, Ramona Salvarinova, Clara D M van Karnebeek
BACKGROUND: Mitochondrial diseases, a group of multi-systemic disorders often characterized by tissue-specific phenotypes, are usually progressive and fatal disorders resulting from defects in oxidative phosphorylation. MTO1 (Mitochondrial tRNA Translation Optimization 1), an evolutionarily conserved protein expressed in high-energy demand tissues has been linked to human early-onset combined oxidative phosphorylation deficiency associated with hypertrophic cardiomyopathy, often referred to as combined oxidative phosphorylation deficiency-10 (COXPD10)...
January 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29325364/-the-efficacy-and-safety-of-coil-embolization-of-septal-branch-in-the-treatment-of-patients-with-obstructive-hypertrophic-cardiomyopathy
#8
H Liu, Q Wu, H W Tan, J Pang
Objective: To observe the clinical efficacy and safety of coil embolization of septal branch in the treatment of obstructive hypertrophic cardiomyopathy (HOCM). Methods: Eighteen patients with HOCM hospitalized in our department from September 2014 to October 2016 were enrolled in this study. There were 12 males and 6 females in this cohort and the age of patients ranged from 22 to 64 years old. Left ventricular outflow tract gradient (LVOTG) was derived from echocardiographic apical five-chamber view at pre-operation and at 48 hours and 6 months post operation...
December 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/29325362/-predicting-value-of-2014-european-guidelines-risk-prediction-model-for-sudden-cardiac-death-hcm-risk-scd-in-chinese-patients-with-hypertrophic-cardiomyopathy
#9
W X Li, L W Liu, J Wang, L Zuo, F Yang, N Kang, C H Lei
Objective: To evaluate the predicting value of the 2014 European Society of Cardiology (ESC) guidelines risk prediction model for sudden cardiac death (HCM Risk-SCD) in Chinese patients with hypertrophic cardiomyopathy (HCM), and to explore the predictors of adverse cardiovascular events in Chinese HCM patients. Methods: The study population consisted of a consecutive 207 HCM patients admitted in our center from October 2014 to October 2016. All patients were followed up to March 2017. The 5-year SCD probability of each patient was estimated using HCM Risk-SCD model based on electrocardiogram, echocardiography and cardiac magnetic resonance (CMR) examination results...
December 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/29325361/-guidelines-for-the-diagnosis-and-treatment-for-chinese-adult-patients-with-hypertrophic-cardiomyopathy
#10
(no author information available yet)
No abstract text is available yet for this article.
December 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/29325359/-new-strategies-for-the-diagnosis-and-treatemtn-of-hypertrophic-cardiomyopathy
#11
(no author information available yet)
No abstract text is available yet for this article.
December 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/29324812/identifying-unmet-clinical-need-in-hypertrophic-cardiomyopathy-using-national-electronic-health-records
#12
Mar Pujades-Rodriguez, Oliver P Guttmann, Arturo Gonzalez-Izquierdo, Bram Duyx, Constantinos O'Mahony, Perry Elliott, Harry Hemingway
INTRODUCTION: To evaluate unmet clinical need in unselected hypertrophic cardiomyopathy (HCM) patients to determine the risk of a wide range of subsequent cardiovascular disease endpoints and safety endpoints relevant for trial design. METHODS: Population based cohort (CALIBER, linked primary care, hospital and mortality records in England, period 1997-2010), all people diagnosed with HCM were identified and matched by age, sex and general practice with ten randomly selected people without HCM...
2018: PloS One
https://www.readbyqxmd.com/read/29321133/evolving-story-of-clinical-trials-in-hypertrophic-cardiomyopathy
#13
EDITORIAL
Perry M Elliott
No abstract text is available yet for this article.
January 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29321131/efficacy-of-ranolazine-in-patients-with-symptomatic-hypertrophic-cardiomyopathy-the-restyle-hcm-randomized-double-blind-placebo-controlled-study
#14
Iacopo Olivotto, Paolo G Camici, Piera Angelica Merlini, Claudio Rapezzi, Monica Patten, Vicent Climent, Gianfranco Sinagra, Benedetta Tomberli, Francisco Marin, Philipp Ehlermann, Lars S Maier, Alessandra Fornaro, Claudius Jacobshagen, Antonello Ganau, Luciano Moretti, Antonio Hernandez Madrid, Raffaele Coppini, Giorgio Reggiardo, Corrado Poggesi, Francesco Fattirolli, Luiz Belardinelli, Gianfranco Gensini, Alessandro Mugelli
BACKGROUND: The late sodium current inhibitor ranolazine reverses the main electrophysiological and mechanical abnormalities of human hypertrophic cardiomyopathy (HCM) cardiomyocytes in vitro, suggesting potential clinical benefit. We aimed to assess the effect of ranolazine on functional capacity, symptomatic status, diastolic function, and arrhythmias in HCM. METHODS AND RESULTS: In this multicenter, double-blind, phase 2 study, 80 adult patients with nonobstructive HCM (age 53±14 years, 34 women) were randomly assigned to placebo (n=40) or ranolazine 1000 mg bid (n=40) for 5 months...
January 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29321034/diagnostic-and-prognostic-utility-of-cardiovascular-magnetic-resonance-imaging-in-heart-failure-with-preserved-ejection-fraction-implications-for-clinical-trials
#15
Prathap Kanagala, Adrian S H Cheng, Anvesha Singh, John McAdam, Anna-Marie Marsh, Jayanth R Arnold, Iain B Squire, Leong L Ng, Gerry P McCann
BACKGROUND: Heart failure with preserved ejection fraction (HFpEF) is a poorly characterized condition. We aimed to phenotype patients with HFpEF using multiparametric stress cardiovascular magnetic resonance imaging (CMR) and to assess the relationship to clinical outcomes. METHODS: One hundred and fifty four patients (51% male, mean age 72 ± 10 years) with a diagnosis of HFpEF underwent transthoracic echocardiography and CMR during a single study visit. The CMR protocol comprised cine, stress/rest perfusion and late gadolinium enhancement imaging on a 3T scanner...
January 11, 2018: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29319343/obesity-and-sudden-cardiac-death-in-the-young-clinical-and-pathological-insights-from-a-large-national-registry
#16
Gherardo Finocchiaro, Michael Papadakis, Harshil Dhutia, Della Cole, Elijah R Behr, Maite Tome, Sanjay Sharma, Mary N Sheppard
Aims Obesity is an increasing public health problem and a risk factor for cardiovascular diseases. The aim of the study was to determine the main features and aetiologies in a large cohort of sudden cardiac deaths that occurred in obese subjects. Methods Between 1994 and 2014, 3684 consecutive cases of unexpected sudden cardiac death were referred to our cardiac pathology centre. This study was confined to young individuals (age ≤ 35 years) for whom information about body mass index was available and consisted of 1033 cases...
January 1, 2018: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/29315424/cardiac-resynchronization-therapy-in-patients-with-end-stage-hypertrophic-cardiomyopathy
#17
Ammar M Killu, Jae-Yoon Park, Jaskanwal D Sara, David O Hodge, Bernard J Gersh, Rick A Nishimura, Samuel J Asirvatham, Christopher J McLeod
Aims: A dilated/end-stage phase of hypertrophic cardiomyopathy (HCM) is rare but well-recognized. The role for cardiac resynchronization therapy (CRT) in this subset of patients remains unexplored. We aimed to clarify the impact of bi-ventricular pacing CRT in dilated/end-stage HCM. Methods and results: The Mayo Clinic HCM database was interrogated to identify patients with ejection fraction (EF) <50% and CRT. Control subjects were identified in 1:1 manner...
January 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29313818/long-term-mortality-in-patients-with-severe-secondary-mitral-regurgitation-and-normal-left-ventricular-ejection-fraction-interventional-perspective
#18
Amr F Barakat, Mohammad Q Raza, Kinjal Banerjee, Rayji S Tsutsui, Amgad Mentias, Karim Abdur Rehman, Badal Thakkar, Sajjad Gul, Raquib Faruqui, Amar Krishnaswamy, Leonardo L Rodriguez, Richard Grimm, Brian Griffin, Emin Murat Tuzcu, Samir R Kapadia
AIMS: Patients with severe secondary mitral regurgitation (MR) and normal ejection fraction are being excluded from clinical trials evaluating transcatheter mitral devices. We sought to evaluate the long-term mortality with medical management alone in this patient population. METHODS AND RESULTS: We retrospectively evaluated patients diagnosed with ≥3+ mitral MR at our institution over 15 years. Only patients with ejection fraction ≥ 60% were included in the study...
January 9, 2018: EuroIntervention
https://www.readbyqxmd.com/read/29310338/genetic-polymorphism-of-angiotensin-converting-enzyme-and-hypertrophic-cardiomyopathy-risk-a-systematic-review-and-meta-analysis
#19
Ye Yuan, Lin Meng, Yan Zhou, Na Lu
BACKGROUND: Genetic factors in the pathogenesis of cardiomyopathies have received a lot of attention during the past 2 decades. Some studies have reported that angiotensin-converting enzyme (ACE) gene has been associated with hypertrophic cardiomyopathy (HCM). However, there have been inconsonant results among different studies. To clarify the influence of ACE on HCM, a systemic review and meta-analysis of case-control studies were performed. METHODS: The following databases were searched to indentify related studies: PubMed database, the Embase database, the Cochrane Central Register of Controlled Trials database, China National Knowledge Information database, and Chinese Scientific and Technological Journal database...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29298659/cardiac-manifestations-of-prkag2-mutation
#20
Pooya Banankhah, Gregory A Fishbein, Anthony Dota, Reza Ardehali
BACKGROUND: The Protein Kinase AMP-Activated Non-Catalytic Subunit Gamma 2 (PRKAG2) cardiac syndrome is characterized by glycogen accumulation in the cardiac tissue. The disease presents clinically with hypertrophic cardiomyopathy (HCM), and it is often associated with conduction abnormalities. CASE PRESENTATION: A 23 year-old female with history of Wolff-Parkinson-White (WPW) and HCM presented for evaluation after an episode of Non-ST Elevation Myocardial Infarction (NSTEMI)...
January 3, 2018: BMC Medical Genetics
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