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https://www.readbyqxmd.com/read/29453593/risk-marker-profiles-in-patients-treated-with-percutaneous-septal-ablation-for-symptomatic-hypertrophic-obstructive-cardiomyopathy
#1
Amelie Burghardt, Frank van Buuren, Zisis Dimitriadis, Tim Grübbel, Hubert Seggewiss, Smita Scholtz, Dieter Horstkotte, Lothar Faber
BACKGROUND AND STUDY OBJECTIVE: Different studies have shown a good long-term survival with improvement of symptoms and hemodynamics after percutaneous septal ablation (PTSMA), similar to myectomy considered to be the therapeutic "gold standard" for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Furthermore, desobliteration of left ventricular (LV) outflow may modify features of the disease considered to be risk-relevant, such as magnitude of LV hypertrophy, outflow gradient, and left atrial (LA) size...
February 16, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/29453001/transmitral-septal-myectomy-for-hypertrophic-obstructive-cardiomyopathy
#2
Brody Wehman, Mehrdad Ghoreishi, Nathaniel Foster, Libin Wang, Michael N D'Ambra, Nathan Maassel, Sam Maghami, Rachael Quinn, Murtaza Dawood, Stacy Fisher, James S Gammie
BACKGROUND: Intrinsic abnormalities of the mitral valve are common in patients with hypertrophic cardiomyopathy and may need to be addressed at operation. METHODS: Consecutive patients undergoing transmitral septal myectomy were retrospectively reviewed. The ventricular septum was exposed through a left atriotomy, and the anterior leaflet of the mitral valve was detached from its annulus. An extended myectomy was performed to the base of the papillary muscles. After myectomy, the anterior leaflet was reattached and concomitant mitral valve repair or replacement was performed...
February 13, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29452394/treatment-of-hypertrophic-cardiomyopathy-caused-by-cardiospecific-variants-of-fabry-disease-with-chaperone-therapy
#3
Jonas Müntze, Tim Salinger, Daniel Gensler, Christoph Wanner, Peter Nordbeck
No abstract text is available yet for this article.
February 14, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29452157/hypertrophic-cardiomyopathy-mutation-r58q-in-the-myosin-regulatory-light-chain-perturbs-thick-filament-based-regulation-in-cardiac-muscle
#4
Thomas Kampourakis, Saraswathi Ponnam, Malcolm Irving
Hypertrophic cardiomyopathy (HCM) is frequently linked to mutations in the protein components of the myosin-containing thick filaments leading to contractile dysfunction and ultimately heart failure. However, the molecular structure-function relationships that underlie these pathological effects remain largely obscure. Here we chose an example mutation (R58Q) in the myosin regulatory light chain (RLC) that is associated with a severe HCM phenotype and combined the results from a wide range of in vitro and in situ structural and functional studies on isolated protein components, myofibrils and ventricular trabeculae to create an extensive map of structure-function relationships...
February 13, 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/29451820/the-hcm-linked-w792r-mutation-in-cardiac-myosin-binding-protein-c-reduces-c6-fniii-domain-stability
#5
Dan F Smelter, Willem J De Lange, Wenxuan Cai, Ying Ge, John C Ralphe
Cardiac myosin binding protein-C (cMyBP-C) is a functional sarcomeric protein that regulates contractility in response to contractile demand, and many mutations in cMyBP-C lead to hypertrophic cardiomyopathy (HCM). To gain insight into the effects of disease-causing cMyBP-C missense mutations on contractile function, we expressed the pathogenic W792R mutation in mouse cardiomyocytes lacking endogenous cMyBP-C and studied the functional effects using three-dimensional engineered cardiac tissue (mECT) constructs...
February 16, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29450893/dofetilide-for-suppression-of-atrial-fibrillation-in-hypertrophic-cardiomyopathy-a-case-series-and-literature-review
#6
JoEllyn C Moore, Lena Trager, Lucille E Anzia, Walid Saliba, Mohamed Bassiouny, Mandeep Bhargava, Mina Chung, Milind Desai, Ross Garberich, Harry Lever, Bruce D Lindsay, Jay Sengupta, Patrick Tchou, Oussama Wazni, Bruce L Wilkoff
BACKGROUND: Limited medical options are available for rhythm control in patients with atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM). There are no published reports of dofetilide use in this population. METHODS: A retrospective chart review was conducted on1,404 patients loaded on dofetilide for AF suppression at the Cleveland Clinic from 2008-2012, 25 of whom were found to have HCM. RESULTS: The HCM cohort was 32% female, 76% with persistent AF, mean age of 59 ± 10 years and mean ejection fraction (EF) of 54 ± 9 %...
February 16, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29450766/septal-myectomy-for-hypertrophic-cardiomyopathy-important-surgical-knowledge-and-technical-tips-in-the-era-of-increasing-alcohol-septal-ablation
#7
REVIEW
Yuting P Chiang, Yuichi J Shimada, Jonathan Ginns, Shepard D Weiner, Hiroo Takayama
Hypertrophic cardiomyopathy (HCM) is the most commonly inherited cardiac disease-recent studies suggest a prevalence as high as 1 in 200. For symptomatic patients with obstructive HCM who are refractory to medical therapy, septal reduction is indicated. Septal myectomy (SM) is considered the gold standard septal reduction technique. However, due to a shortage of surgeons who are experienced in this technique, alcohol septal ablation (ASA) has overtaken SM as the most commonly performed procedure for obstructive HCM...
February 15, 2018: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29449957/early-detection-of-myocardial-dysfunction-using-two-dimensional-speckle-tracking-echocardiography-in-a-young-cat-with-hypertrophic-cardiomyopathy
#8
Ryohei Suzuki, Yohei Mochizuki, Hiroki Yoshimatsu, Ayaka Niina, Takahiro Teshima, Hirotaka Matsumoto, Hidekazu Koyama
Case summary: A 5-month-old intact female Scottish Fold cat was presented for cardiac evaluation. Careful auscultation detected a slight systolic murmur (Levine I/VI). The findings of electrocardiography, thoracic radiography, non-invasive blood pressure measurements and conventional echocardiographic studies were unremarkable. However, two-dimensional speckle tracking echocardiography revealed abnormalities in myocardial deformations, including decreased early-to-late diastolic strain rate ratios in longitudinal, radial and circumferential directions, and deteriorated segmental systolic longitudinal strain...
January 2018: JFMS Open Reports
https://www.readbyqxmd.com/read/29444294/prognosis-of-adult-obstructive-hypertrophic-cardiomyopathy-patients-with-different-morphological-types-after-surgical-myectomy
#9
Bing Tang, Yunhu Song, Hao Cui, Keshan Ji, Qinjun Yu, Changsheng Zhu, Shihua Zhao, Shuiyun Wang
OBJECTIVES: This study aims to assess the impact of morphological type on the prognosis of obstructive hypertrophic cardiomyopathy patients who underwent myectomy. METHODS: We recruited 469 obstructive hypertrophic cardiomyopathy patients refractory to medicinal treatment who have undergone surgical myectomy at a nationwide referral centre. All patients were divided into 3 groups based on the morphological classification of left ventricular hypertrophy and were followed up by telephone every year...
February 12, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29440459/mir-139-5p-inhibits-isoproterenol-induced-cardiac-hypertrophy-by-targeting-c-jun
#10
Su Ming, Wang Shui-Yun, Qiu Wei, Li Jian-Hui, Hui Ru-Tai, Song Lei, Jia Mei, Wang Hui, Wang Ji-Zheng
Hypertrophic cardiomyopathy (HCM) is a serious monogenic disease characterized by cardiac hypertrophy, fibrosis, sudden cardiac death and heart failure. Previously, we identified that miR-139-5p was downregulated in HCM patients. However, the regulatory effects of miR-139-5p remain unclear. Thus, we investigated the role of miR-139-5p in the regulation of cardiac hypertrophy. The expression of miR-139-5p in left ventricular tissues in HCM patients and mice subjected to transverse aortic constriction (TAC) was significantly downregulated...
February 12, 2018: Bioscience Reports
https://www.readbyqxmd.com/read/29440008/novel-%C3%AE-actin-gene-mutation-p-ala21val-causing-familial-hypertrophic-cardiomyopathy-myocardial-noncompaction-and-transmural-crypts-clinical-pathologic-correlation
#11
Andrea Frustaci, Alessandro De Luca, Valentina Guida, Tommaso Biagini, Tommaso Mazza, Carlo Gaudio, Claudio Letizia, Matteo Antonio Russo, Nicola Galea, Cristina Chimenti
BACKGROUND: Mutations of α-actin gene (ACTC1) have been phenotypically related to various cardiac anomalies, including hypertrophic cardiomyopathy and dilated cardiomyopathy and left ventricular (LV) myocardial noncompaction. A novel ACTC mutation is reported as cosegregating for familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts. METHODS AND RESULTS: In an Italian family of 7 subjects, 4 aged 10 (II-1), 14 (II-2), 43 (I-4) and 46 years (I-5), presenting abnormal ECG changes, dyspnea and palpitation (II-2, I-4, and I-5), and recurrent cerebral ischemic attack (I-5), underwent 2-dimensional echo, cardiac magnetic resonance, Holter monitoring, and next-generation sequencing gene analysis...
February 10, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29438530/hypertrophic-obstructive-cardiomyopathy-what-when-why-for-whom
#12
Carlos A Mestres, Thomas Bartel, Antonio Sorgente, Silvana Müller, Christiane Gruner, Joseph Dearani, Eduard Quintana
Hypertrophic cardiomyopathy is the most common genetic cardiovascular disorder and is associated with symptoms of heart failure and increased risk of sudden cardiac death. The most common condition is obstruction of the left ventricular outflow tract. Surgical septal myectomy and alcohol septal ablation are the 2 accepted modes of septal reduction therapy and are indicated when there are advanced symptoms and a peak left ventricular outflow gradient ≥50 mmHg. Advantages of alcohol septal ablation are limited groin approach, reduction of obstruction of the left ventricular outflow tract and functional improvement, but there are higher chances for intracardiac device implantation and residual obstruction...
February 9, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29437742/implantable-cardioverter-defibrillators-in-the-context-of-hypertrophic-cardiomyopathy-a-lesson-in-patient-autonomy
#13
Jonathan James Hyett Bray, Chiara Bucciarelli-Ducci, Graham Stuart
Hypertrophiccardiomyopathy (HCM) is common, whereas the decision not to have an implantable cardioverterdefibrillator (ICD) when probably falling into a 'high-risk' category is not. A solicitor aged 45 years attended the inherited cardiac conditions clinic for review of her HCM and discussion about ICD implantation for primary prevention of sudden cardiac death (SCD). Despite a predicted 7% risk of SCD within the next 5 years, according to the European Society of Cardiology endorsed HCM Risk-SCD risk stratification tool, the patient opted against implantation of an ICD and comprehensively justifies her decision...
February 5, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29435968/-a-new-phenotype-of-infantile-onset-pompe-disease
#14
A Nascimento, E Villalobos-Pinto
INTRODUCTION: Infantile-onset Pompe disease is a kind of glycogenosis resulting from a deficit of the enzyme acid alpha-glucosidase. Before specific enzyme replacement therapy (ERT) became available, the classic form was fatal during the first two years of life. ERT increases survival and improves cardiac, respiratory and motor functioning. CASE REPORTS: Case 1: 2-month-old infant with predominantly axial hypotonia who required the use of a nasogastric tube as a result of difficulties in sucking and swallowing...
February 16, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29435772/heart-failure-interventions-targeting-impaired-left-ventricles-in-structural-heart-disease
#15
REVIEW
Mitsunobu Kitamura, Tobias Schmidt, Karl-Heinz Kuck, Christian Frerker
PURPOSE OF REVIEW: Interventional techniques have been developed for a wide spectrum of mechanisms of heart failure (HF), especially in valvular heart disease and cardiomyopathies (ischaemic cardiomyopathy and hypertrophic cardiomyopathy). In this article, we review recent reports on catheter interventions to treat patients with HF. RECENT FINDINGS: Direct modification using the Parachute device and the REVIVENT-TC device for patients with impaired left ventricle with large infarct scars improves geometry and haemodynamic efficiency, resulting in a reduction of HF symptoms...
February 12, 2018: Current Cardiology Reports
https://www.readbyqxmd.com/read/29431406/hypertrophic-cardiomyopathy-an-often-silent-and-delayed-diagnosis
#16
Robert Gauer
No abstract text is available yet for this article.
December 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/29431384/cardiomyopathy-an-overview
#17
Jay Brieler, Matthew A Breeden, Jane Tucker
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Dilated cardiomyopathy can be genetic or acquired and typically presents with classic symptoms of heart failure with reduced ejection fraction...
November 15, 2017: American Family Physician
https://www.readbyqxmd.com/read/29428048/a-new-look-into-hypertrophic-cardiomyopathy-based-on-clinical-evidence
#18
Gláucia Maria Moraes de Oliveira
No abstract text is available yet for this article.
January 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29426649/rest-and-stress-longitudinal-systolic-left-ventricular-mechanics-in-hypertrophic-cardiomyopathy-implications-for-prognostication
#19
Iraklis Pozios, Aurelio Pinheiro, Celia Corona-Villalobos, Lars L Sorensen, Zeina Dardari, Hong-Yun Liu, Kenneth Cresswell, Susan Phillip, David A Bluemke, Stefan L Zimmerman, M Roselle Abraham, Theodore P Abraham
BACKGROUND: Exercise intolerance is the most common symptom in hypertrophic cardiomyopathy (HCM). We examined whether inability to augment myocardial mechanics during exercise would influence functional performance and clinical outcomes in HCM. METHODS: Ninety-five HCM patients (32 nonobstructive, 32 labile-obstructive, 31 obstructive) and 26 controls of similar age and gender distribution were recruited prospectively. They underwent rest and treadmill stress strain echocardiography, and 61 of them underwent magnetic resonance imaging...
February 6, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29425530/ventricular-assist-devices-for-neonates-and-infants
#20
REVIEW
Katsuhide Maeda, David N Rosenthal, Olaf Reinhartz
Heart transplant waitlist survival in pediatric patients has been substantially improved since the introduction of pediatric-specific ventricular assist device. In neonates and infants, however, the waitlist mortality remains very high. The only long-term device currently approved for use in the United States is the Berlin Heart EXCOR, but this device has several important limitations because of the paracorporeal, pulsatile nature of the underlying technology. We reviewed Stanford ventricular assist experience on patients less than 1 year old since 2004...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
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