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Hypertrophe cardiomyopathy

Mouaz H Al-Mallah
No abstract text is available yet for this article.
March 8, 2019: JACC. Cardiovascular Imaging
Avinash Mani, Arun Gopalakrishnan, Anoop Ayyappan, Ajitkumar Valaparambil
A 5-year-old boy with an incidentally detected cardiac murmur was referred for evaluation. Tall R waves were noted in the electrocardiogram in leads V3 and V4. Transthoracic echocardiography suggested asymmetric septal hypertrophy with diffuse thickening of the inter-ventricular septum with normal thickness of the posterior left ventricular wall. Upon closer interrogation, a masquerading sessile cardiac mass was identified adherent to the left ventricular side of the inter-ventricular septum which appeared to contract with each cardiac cycle, mimicking hypertrophic cardiomyopathy...
March 2019: Clinical Medicine: Journal of the Royal College of Physicians of London
Alexandra Sousa, Paulo Canedo, Olga Azevedo, Luís Lopes, Teresa Pinho, Márcia Baixia, Francisco Rocha-Gonçalves, Lino Gonçalves, José Silva Cardoso, José Carlos Machado, Elisabete Martins
INTRODUCTION: Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilatation and impaired systolic function. Familial forms account for 30-50% of cases. Autosomal dominant inheritance is the predominant pattern of transmission. Causal genetic variants have been identified in several genes and molecular diagnosis has implications for genetic counseling and risk stratification. OBJECTIVE: We aimed to estimate the frequency of genetic variants and the molecular basis of DCM in Portugal...
March 11, 2019: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
Ladislav Valkovič, William T Clarke, Albrecht I Schmid, Betty Raman, Jane Ellis, Hugh Watkins, Matthew D Robson, Stefan Neubauer, Christopher T Rodgers
BACKGROUND: Cardiovascular phosphorus MR spectroscopy (31 P-CMRS) is a powerful tool for probing energetics in the human heart, through quantification of phosphocreatine (PCr) to adenosine triphosphate (ATP) ratio. In principle, 31 P-CMRS can also measure cardiac intracellular pH (pHi ) and the free energy of ATP hydrolysis (ΔGATP ). However, these require determination of the inorganic phosphate (Pi) signal frequency and amplitude that are currently not robustly accessible because blood signals often obscure the Pi resonance...
March 14, 2019: Journal of Cardiovascular Magnetic Resonance
Narayana Sarma V Singam, Marcus F Stoddard
Asian hypertrophic cardiomyopathy (AHCM) is a rare variant of hypertrophic cardiomyopathy (HCM) that is more prevalent in the Asian population. There is significant overlap between AHCM, mid-cavitary obstruction, and apical aneurysms. Although more benign compared to HCM, the course of AHCM is not clearly defined. We present an interesting case of an African American male with known AHCM who develops symptomatic mid-cavitary obstruction and apical aneurysm 11 years after initial diagnosis.
March 13, 2019: Echocardiography
Hisanori Horiuchi, Tsuyoshi Doman, Koichi Kokame, Yoshikatsu Saiki, Masanori Matsumoto
The blood glycoprotein von Willebrand factor (VWF) plays an important role in hemostasis and thrombosis.VWF is produced and secreted as large multimers by endothelial cells and megakaryocytes. It is then cleaved in a sheer-stress dependent manner by a specific protease, ADAMTS13, into multimers consisting of 2-80 subunits. Among VWF multimers, high molecular weight (HMW) multimers play important roles in platelet aggregation. Therefore, their loss induces a hemostatic disorder known as von Willebrand disease (VWD) type 2A...
March 12, 2019: Journal of Atherosclerosis and Thrombosis
Elena Severinova, Saleena Alikunju, Wei Deng, Puneet Dhawan, Nazish Sayed, Danish Sayed
Background An increase in serum cortisol has been identified as a risk factor for cardiac failure, which highlights the impact of glucocorticoid signaling in cardiomyocytes and its influence in the progression of failure. Dexamethasone, a synthetic glucocorticoid, is sufficient for induction of cardiomyocyte hypertrophy, but little is known of the glucocorticoid receptor (GR) genome-binding and -dependent transcriptional changes that mediate this phenotype. Methods and Results In this study using high-resolution sequencing, we identified genomic targets of GR and associated change in the transcriptome after 1 and 24 hours of dexamethasone treatment...
March 19, 2019: Journal of the American Heart Association
Özge Turgay Yıldırım, Ercan Akşit, Fatih Aydın, Ayşe Hüseyinoğlu Aydın
: Left ventricular thrombus (LVT) is a commonly seen complication of myocardial infarction and it also can be seen because of hypertrophic cardiomyopathies, nonischemic dilated cardiomyopathies, malignancies, and so on. Guidelines suggest the use of warfarin for left ventricular thrombi, but recent case reports show that direct oral anticoagulants (DOACs) are beginning to be used for this complication. DOACs are strong alternatives for warfarin because of their efficacy and safety even though there is no randomized controlled trial that proves the effect of DOACs against LVT...
March 8, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Francesco Pelliccia, Giampaolo Niccoli, Felice Gragnano, Giuseppe Limongelli, Elisabetta Moscarella, Giuseppe Andò, Augusto Esposito, Eugenio Stabile, Gian Paolo Ussia, Giuseppe Tarantini, Juan Ramon Gimeno, Perry Elliott, Paolo Calabrò
Percutaneous alcohol septal ablation (ASA) is an effective and minimally invasive therapeutic strategy to resolve left ventricular outflow tract obstruction (LVOTO) in patients with hypertrophic cardiomy-opathy who remain symptomatic on maximally tolerated medical therapy. Firstly performed by Sig-wart in 1994, the procedure consists in determining an iatrogenic infarction of basal interventricular septum to reduce LVOTO and alleviate symptoms. Since its first description, numerous studies demonstrated its efficacy and safety, proposing ASA as a valid and attractive alternative to surgical septal myectomy...
March 12, 2019: EuroIntervention
Michela Brambatti, Oren Caspi, Alessandro Maolo, Elliott Koshi, Barry Greenberg, Matthew R G Taylor, Eric D Adler
BACKGROUND: Danon disease (DD) is a rare X-linked autophagic vacuolar myopathy, characterized by high penetrance and severe cardiomyopathy. Because of its rarity, the natural history (NH) is uncertain. OBJECTIVES: We aimed to describe disease variability and outcomes through a systematic review of all published DD cases. METHODS: Among 83 manuscripts in MEDLINE and EMBASE on DD cases published until October 2017, we identified 146 patients with positive genetic testing for DD or positive muscle biopsy in a relative of a genetically diagnosed proband...
February 16, 2019: International Journal of Cardiology
Jin-Jun Liang, Hideo Okamura, Roshini Asirvatham, Andrew Schneider, David O Hodge, Mei Yang, Xu-Ping Li, Ming-Yan Dai, Ying Tian, Pei Zhang, Bryan C Cannon, Cong-Xin Huang, Paul A Friedman, Yong-Mei Cha
BACKGROUND: The comparative outcomes of subcutaneous implantable cardioverter-defibrillator (S-ICD) and transvenous ICD (T-ICD) have not been well studied. The aim of this study was to evaluate the safety and efficacy of currently available S-ICD and T-ICD. METHODS: The study included 86 patients who received an S-ICD and 1:1 matched to those who received single-chamber T-ICD by gender, age, diagnosis, left ventricular ejection fraction (LVEF), and implant year...
March 20, 2019: Chinese Medical Journal
Angelika Batzner, Hans-Joachim Schäfers, Konstantin V Borisov, Hubert Seggewiß
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is caused by mutations in a number of genes. Its prevalence is 0.2% to 0.6%. METHODS: This review is based on publications retrieved by a selective literature search and on the authors' clinical experi- ence. RESULTS: 70% of patients with HCM suffer from the obstructive type of the condition, clinically characterized by highly dynamic and variable manifestations in the form of dyspnea, angina pectoris, and stress-dependent presyncope and syn- cope...
January 25, 2019: Deutsches Ärzteblatt International
George Makavos, Chris Κairis, Maria-Eirini Tselegkidi, Theodoros Karamitsos, Angelos G Rigopoulos, Michel Noutsias, Ignatios Ikonomidis
Hypertrophic cardiomyopathy (HCM) represents a phenotype of left ventricular hypertrophy unexplained by abnormal loading conditions. The definition is based on clinical criteria; however, there are numerous underlying etiologic factors. The MOGE(S) classification provides a standardized approach for multimodal characterization of HCM. HCM is associated with increased morbidity and mortality, and especially the assessment of the risk of sudden cardiac death is of paramount importance. In this review, we summarize essential knowledge and recently published data on clinical presentation, diagnosis, genetic analyses, differential diagnosis, prognosis, and treatment options that are necessary for understanding and management of HCM...
March 9, 2019: Heart Failure Reviews
Ernesto Jimenez, Darrell Wu, Shuab Omer, Lorraine Cornwell, Paulino Nunez, Todd K Rosengart, Joseph S Coselli
OBJECTIVE: Between 2000 and 2008, the mitral valve (MV) repair rate in patients with severe mitral regurgitation (MR) at our low-volume Veterans Affairs hospital was 21%. After instituting a multidisciplinary valve team in 2009, we determined whether this rate increased and characterized the outcomes of patients with degenerative disease. METHODS: We retrospectively reviewed data from 103 MV operations performed at our hospital between 1/2009 and 8/2016. Mitral valve pathology was categorized as degenerative, rheumatic, endocarditis, ischemic, hypertrophic cardiomyopathy, or failed prior MV repair...
March 5, 2019: Seminars in Thoracic and Cardiovascular Surgery
Andrea E Deranek, Matthew M Klass, Jil C Tardiff
The classic clinical definition of hypertrophic cardiomyopathy (HCM) as originally described by Teare is deceptively simple, "left ventricular hypertrophy in the absence of any identifiable cause." Longitudinal studies, however, including a seminal study performed by Frank and Braunwald in 1968, clearly described the disorder much as we know it today, a complex, progressive, and highly variable cardiomyopathy affecting ~ 1/500 individuals worldwide. Subsequent genetic linkage studies in the early 1990s identified mutations in virtually all of the protein components of the cardiac sarcomere as the primary molecular cause of HCM...
March 8, 2019: Pflügers Archiv: European Journal of Physiology
Kunihito Tsuboi, Akio Nagaki, Takayuki Shibutani, Masahisa Onoguchi
BACKGROUND: The spectrum dynamics (SD) algorithm is a cardiac reconstruction algorithm of D-SPECT, which improves spatial resolution compared with the ordered-subsets expectation maximization (OSEM) algorithm. We evaluated the wall thickness and left ventricular (LV) volume in patients with hypertrophic cardiomyopathy (HCM) using the SD algorithm. METHODS: In a phantom study, the myocardial wall was scanned with varying wall thicknesses (10-40 mm). In the clinical study, 30 and 27 normal and HCM patients underwent myocardial perfusion imaging...
March 7, 2019: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Riccardo Proietti, Vincenzo Russo, Ahmed AlTurki
Implantable cardiac defibrillators (ICD) are the foundation of therapy for the prevention of sudden cardiac death. While ICDs prevent SCD, they do not prevent the occurrence of ventricular arrhythmias which are usually symptomatic. Though catheter ablation has been successful in substrate modification of ventricular tachycardia in patients with ischemic cardiomyopathy, there is much less evidence to support its use in non-ischemic cardiomyopathy. Therefore, anti-arrhythmic drugs (AADs) are an essential adjunctive therapy for secondary prevention of ventricular arrhythmias in patients with non-ischemic cardiomyopathy...
March 4, 2019: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Muzaddid Sarker, Hyunna T Lee, Lin Mei, Andrey Krokhotin, Santiago Espinosa de Los Reyes, Laura Yen, Lindsey M Costantini, Jack Griffith, Nikolay V Dokholyan, Gregory M Alushin, Sharon L Campbell
Debilitating heart conditions, notably dilated and hypertrophic cardiomyopathies (CM), are associated with point mutations in metavinculin, a larger isoform of the essential cytoskeletal protein vinculin. Metavinculin is co-expressed with vinculin at sub-stoichiometric ratios in cardiac tissues. Cardiomyopathy mutations in the metavinculin tail domain (MVt) occur within the extra 68-residue insert that differentiates it from the vinculin tail domain (Vt). Vt binds actin filaments (F-actin) and promotes vinculin dimerization to bundle F-actin into thick fibers...
March 4, 2019: Journal of Molecular Biology
Kai-Chun Cheng, Wei-Ting Chang, Feng Yu Kuo, Zhih-Cherng Chen, Yingxiao Li, Juei-Tang Cheng
Left ventricular hypertrophy is an independent risk factor in diabetic patients. TGR5 is shown to express in hearts, but its functional role in diabetes-induced cardiac hypertrophy remained unclear. The current study investigated the role of TGR5 on high glucose-induced hypertrophy of H9C2 cells. After incubation with a high level of glucose, H9C2 cells showed hypertrophic responses. Activation of TGR5 by lithocholic acid (LCA) ameliorated cell hypertrophy and enhanced SERCA2a and phosphorylated phospholamban (PLN) expression in H9C2 cells...
March 6, 2019: Scientific Reports
Ziad Arow, Mithal Nassar, Daniel Monakier, Abid Assali, Hana Vaknin-Assa, Ran Kornowski, Ashraf Hamdan
Myocardial crypts can be recognized in patients with hypertrophic cardiomyopathy (HCM) using magnetic resonance imaging, but similar studies using computed tomography (CT) are sparse. The aim of the present study was to evaluate the prevalence and morphology of myocardial crypts in patients with HCM, arterial hypertension, and aortic valve stenosis using contrast-enhanced CT. We also investigated the added value of a finding of myocardial crypts on CT scan to the diagnosis of HCM. The study cohort included 73 patients with HCM, 100 patients with arterial hypertension, 120 patients with aortic valve stenosis, and 100 subjects without cardiovascular disease (normal control group)...
March 5, 2019: International Journal of Cardiovascular Imaging
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