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Hypertrophic cardiomyopathies

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https://www.readbyqxmd.com/read/28239655/alterations-in-sarcomere-function-modify-the-hyperplastic-to-hypertrophic-transition-phase-of-mammalian-cardiomyocyte-development
#1
Benjamin R Nixon, Alexandra F Williams, Michael S Glennon, Alejandro E de Feria, Sara C Sebag, H Scott Baldwin, Jason R Becker
It remains unclear how perturbations in cardiomyocyte sarcomere function alter postnatal heart development. We utilized murine models that allowed manipulation of cardiac myosin-binding protein C (MYBPC3) expression at critical stages of cardiac ontogeny to study the response of the postnatal heart to disrupted sarcomere function. We discovered that the hyperplastic to hypertrophic transition phase of mammalian heart development was altered in mice lacking MYBPC3 and this was the critical period for subsequent development of cardiomyopathy...
February 23, 2017: JCI Insight
https://www.readbyqxmd.com/read/28235781/pathogenesis-of-depression-and-anxiety-like-behavior-in-an-animal-model-of-hypertrophic-cardiomyopathy
#2
Amanda M Dossat, Marcos A Sanchez-Gonzalez, Andrew P Koutnik, Stefano Leitner, Edda L Ruiz, Brittany Griffin, Jens T Rosenberg, Samuel C Grant, Francis D Fincham, Jose R Pinto, Mohamed Kabbaj
Cardiovascular dysfunction is highly comorbid with mood disorders, such as anxiety and depression. However, the mechanisms linking cardiovascular dysfunction with the core behavioral features of mood disorder remain poorly understood. In this study, we used mice bearing a knock-in sarcomeric mutation, which is exhibited in human hypertrophic cardiomyopathy (HCM), to investigate the influence of HCM over the development of anxiety and depression. We employed behavioral, MRI, and biochemical techniques in young (3-4 mo) and aged adult (7-8 mo) female mice to examine the effects of HCM on the development of anxiety- and depression-like behaviors...
February 24, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28228389/stress-cardiomyopathy-in-a-patient-with-hypertrophic-cardiomyopathy-and-myocardial-bridging
#3
Miguel Benavides, Juan M Vinardell, Ivan Arenas, Orlando Santana
Stress cardiomyopathy is an acquired cardiomyopathy of unknown aetiology. It usually occurs in women over the age of 70 who have experienced physical or emotional stress. It is most commonly characterised by a transient, left ventricular systolic dysfunction in the apical portion and hyperkinesia in the basal segments, without obstructive coronary artery disease. Its association with obstructive hypertrophic cardiomyopathy and myocardial bridging is rare. Herein, we present such a case.
February 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28225973/do-you-know-this-syndrome-leopard-syndrome
#4
Flávio Heleno da Silva Queiroz Cançado, Luis Candido Pinto da Silva, Paulo Franco Taitson, Ana Carolina Dias Viana de Andrade, Matheus Melo Pithon, Dauro Douglas Oliveira
Hypertrophic cardiomyopathy is known as Leopard syndrome, which is a mnemonic rule for multiple lentigines (L), electrocardiographic conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), abnormalities of genitalia (A), retardation of growth (R), and deafness (D). We report the case of a 12-year-old patient with some of the abovementioned characteristics: hypertelorism, macroglossia, lentigines, hypospadias, cryptorchidism, subaortic stenosis, growth retardation, and hearing impairment...
January 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28223477/znf307-zinc-finger-protein-307-acts-as-a-negative-regulator-of-pressure-overload-induced-cardiac-hypertrophy
#5
Chang-Jiang Yu, Chen Liang, Yu-Xia Li, Qing-Qing Hu, Wei-Wan Zheng, Na Niu, Xu Yang, Zi-Rui Wang, Xiao-Di Yu, Bao-Long Zhang, Bin-Lin Song, Zhi-Ren Zhang
Pathological cardiac hypertrophy is a key risk factor for heart failure. We found that the protein expression levels of the ZNF307 (zinc finger protein 307) were significantly increased in heart samples from both human patients with dilated cardiomyopathy and mice subjected to aortic banding. Therefore, we aimed to elucidate the role of ZNF307 in the development of cardiac hypertrophy and to explore the signal transduction events that mediate the effect of ZNF307 on cardiac hypertrophy, using cardiac-specific ZNF307 transgenic (ZNF307-TG) mice and ZNF307 global knockout (ZNF307-KO) mice...
February 21, 2017: Hypertension
https://www.readbyqxmd.com/read/28223422/a-potential-oligogenic-etiology-of-hypertrophic-cardiomyopathy-a-classic-single-gene-disorder
#6
Lili Li, Matthew N Bainbridge, Yanli Tan, James T Willerson, Ali J Marian
Rationale: Hypertrophic cardiomyopathy (HCM) is a prototypic single gene disease caused mainly by mutations in genes encoding sarcomere proteins. Despite the remarkable advances, the causal genes in about 40% of the HCM cases remain unknown, typically in small families and sporadic cases, wherein co-segregation could not be established. Objective: To test the hypothesis that the "missing causal genes" in HCM is, in part, because of an oligogenic etiology, wherein the pathogenic variants do not co-segregate with the phenotype...
February 21, 2017: Circulation Research
https://www.readbyqxmd.com/read/28220574/selective-use-of-the-electrocardiogram-in-pediatric-preparticipation-athletic-examinations-among-pediatric-primary-care-providers
#7
Bradley C Clark, Joshua M Hayman, Charles I Berul, Kristin M Burns, Jonathan R Kaltman
OBJECTIVES: Recent literature examining insurance administrative data suggests that a selective approach, with concurrent history and physical exam (H&P), for obtaining an electrocardiogram (ECG) as a part of a preparticipation examination (PPE) for pediatric athletes is commonly used in the primary care setting demonstrating a high rate of disease detection. We sought to understand practice patterns of providers with regard to usage of ECG as a part of PPE. METHODS: Utilizing an online survey, we queried primary care providers regarding their practice patterns, rationale, and concerns regarding use of ECGs as a part of the PPE...
February 21, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28219794/atrial-fibrillation-in-hypertrophic-cardiomyopathy-a-turning-point-towards-increased-morbidity-and-mortality
#8
Thomas Zegkos, Georgios K Efthimiadis, Despoina G Parcharidou, Thomas D Gossios, Georgios Giannakoulas, Dimitris Ntelios, Antonis Ziakas, Stelios Paraskevaidis, Haralambos I Karvounis
BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmic event in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to identify the clinical and the prognostic impact of the arrhythmia on a large cohort of patients with HCM. METHODS: The echocardiographic and clinical correlates, the predictors of AF and thromboembolic stroke and the prognostic significance of the arrhythmia were evaluated in 509 patients with an established diagnosis of HCM...
February 17, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28215181/effect-of-cellular-and-extracellular-pathology-assessed-by-t1-mapping-on-regional-contractile-function-in-hypertrophic-cardiomyopathy
#9
Peter P Swoboda, Adam K McDiarmid, Bara Erhayiem, Graham R Law, Pankaj Garg, David A Broadbent, David P Ripley, Tarique A Musa, Laura E Dobson, James R Foley, Graham J Fent, Stephen P Page, John P Greenwood, Sven Plein
BACKGROUND: Regional contractile dysfunction is a frequent finding in hypertrophic cardiomyopathy (HCM). We aimed to investigate the contribution of different tissue characteristics in HCM to regional contractile dysfunction. METHODS: We prospectively recruited 50 patients with HCM who underwent cardiovascular magnetic resonance (CMR) studies at 3.0 T including cine imaging, T1 mapping and late gadolinium enhancement (LGE) imaging. For each segment of the American Heart Association model segment thickness, native T1, extracellular volume (ECV), presence of LGE and regional strain (by feature tracking and tissue tagging) were assessed...
February 20, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28214155/genetic-tests-in-the-assessment-of-patients-and-at-risk-relatives-the-example-of-hypertrophic-cardiomyopathy
#10
Jorge Manuel Saraiva
No abstract text is available yet for this article.
February 14, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28214152/clinical-and-genetic-diagnosis-of-familial-hypertrophic-cardiomyopathy-results-in-pediatric-cardiology
#11
Bárbara Cardoso, Inês Gomes, Petra Loureiro, Conceição Trigo, Fátima Ferreira Pinto
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is most often of autosomal dominant inheritance with incomplete penetrance and variable expression. The main purpose of family screening is to identify relatives with unrecognized HCM and to monitor those at risk for disease, in order to minimize complications and to assess risk of sudden cardiac death. The ESC and ACCF/AHA guidelines on the diagnosis and management of HCM recommend the screening of child relatives from the age of 10-12 years...
February 14, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28214001/comparison-of-pulmonary-venous-and-left-atrial-remodeling-in-patients-with-atrial-fibrillation-with-hypertrophic-cardiomyopathy-versus-with-hypertensive-heart-disease
#12
Kentaro Yoshida, Hideyuki Hasebe, Yasuaki Tsumagari, Hidekazu Tsuneoka, Mari Ebine, Yoshiko Uehara, Yoshihiro Seo, Kazutaka Aonuma, Noriyuki Takeyasu
Left ventricular diastolic dysfunction in hypertrophic cardiomyopathy (HC) increases susceptibility to atrial fibrillation. Although phenotypical characteristics of the hypertrophied left ventricle are clear, left atrial (LA) and pulmonary venous (PV) remodeling has rarely been investigated. This study aimed to identify differences in LA and PV remodeling between HC and hypertensive heart disease (HHD) using 3-dimensional computed tomography. Included were 33 consecutive patients with HC, 25 with HHD, and 29 without any co-morbidities who were referred for catheter ablation of atrial fibrillation...
January 25, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28213270/myocardial-leptin-transcription-in-feline-hypertrophic-cardiomyopathy
#13
Sonja Fonfara, Sarah Kitz, Udo Hetzel, Anja Kipar
Leptin is an adipokine, which is in humans with cardiac disease suspected to be involved in myocardial remodeling and thrombus formation. In cats, however, it is not known whether leptin plays a role in cardiac disease, i.e. hypertrophic cardiomyopathy (HCM) and the presence of an atrial thrombus (AT). The objective of the study was therefore to establish whether leptin is transcribed in the feline myocardium and to compare myocardial leptin mRNA concentrations in cats with HCM with and without AT, and in cats without cardiac diseases...
February 13, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28212053/transthoracic-echocardiography-pitfalls-and-limitations-as-delineated-at-cardiac-ct-and-mr-imaging
#14
Sachin B Malik, Natalie Chen, Rex A Parker, Joe Y Hsu
Transthoracic echocardiography ( TTE transthoracic echocardiography ) is a critical tool in the field of clinical cardiology. It often serves as one of the first-line imaging modalities in the evaluation of cardiac disease owing to its low cost, portability, widespread availability, lack of ionizing radiation, and ability to evaluate both anatomy and function of the heart. Consequently, a large majority of patients undergoing a cardiac computed tomography (CT) or magnetic resonance (MR) imaging examination will have a TTE transthoracic echocardiography available for review...
February 17, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28209216/hypertrophic-cardiomyopathy-with-left%C3%A2-ventricular-apical-aneurysm-implications-for-risk-stratification-and-management
#15
Ethan J Rowin, Barry J Maron, Tammy S Haas, Ross F Garberich, Weijia Wang, Mark S Link, Martin S Maron
BACKGROUND: A previously under-recognized subset of hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) apical aneurysms is being identified with increasing frequency. However, risks associated with this subgroup are unknown. OBJECTIVES: The authors aimed to clarify clinical course and prognosis of a large cohort of HCM patients with LV apical aneurysms over long-term follow-up. METHODS: The authors retrospectively analyzed 1,940 consecutive HCM patients at 2 centers, 93 of which (4...
February 21, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28201496/other-side-of-the-coin-the-missing-heritability-in-hypertrophic-cardiomyopathy
#16
Adam S Helms, Sharlene M Day
No abstract text is available yet for this article.
February 13, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28197645/subclinical-decrease-in-myocardial-function-in-asymptomatic-infants-of-diabetic-mothers-a-tissue-doppler-study
#17
Jenny E Zablah, Dorota Gruber, Guillaume Stoffels, Estefania G Cabezas, Denise A Hayes
Infants of diabetic mothers (IDMs) with hypertrophic cardiomyopathy are recognized to have impaired myocardial performance, but less is known about ventricular function in IDMs without hypertrophy. We hypothesized that in asymptomatic newborns with normal two-dimensional echocardiographic evaluations, pulsed wave tissue Doppler imaging (TDI) would suggest a subclinical decrease in the cardiac function of IDMs compared to infants of non-diabetics (nIDMs). This is a retrospective cohort study of asymptomatic neonates ≥36 weeks gestation, at 0-7 days of life, with normal standard echocardiograms...
February 14, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28197289/cardiac-amyloidosis-masked-as-hypertrophic-cardiomyopathy-a-case-report
#18
Jing Wang, Amy Marzolf, Janet C L Zhang, Anjali Owens, Yuchi Han
It is well known that cardiac amyloidosis and hypertrophic cardiomyopathy (HCM) have different physiologies and pathologies. However, it might be difficult to differentiate cardiac amyloidosis from HCM in certain clinical situations.
October 2016: Cardiology Research
https://www.readbyqxmd.com/read/28194469/phenylephrine-induced-cardiac-hypertrophy-is-attenuated-by-a-histone-acetylase-inhibitor-anacardic-acid-in-mice
#19
Chang Peng, Xiaomei Luo, Shuo Li, Huichao Sun
Cardiac hypertrophy is a complex process involving highly coordinated but tight regulation of multiple elements, such as in epigenetics, which make an important contribution to myocardium remodeling and cardiac hypertrophy. Epigenetic regulations, particularly histone acetylation, have been implicated in cardiac hypertrophy, however, the exact mechanism is still largely unknown. In the present study, we explored the potential attenuating effects of Chinese herbal extract anacardic acid on phenylephrine-induced cardiac hypertrophy and the underlying mechanism...
February 14, 2017: Molecular BioSystems
https://www.readbyqxmd.com/read/28193615/phenotype-genotype-correlation-in-hypertrophic-cardiomyopathy-less-signal-more-noise
#20
EDITORIAL
Albree Tower-Rader, Milind Y Desai
No abstract text is available yet for this article.
February 2017: Circulation. Cardiovascular Imaging
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