19,273 results match your criteria Cardiomyopathy Hypertrophic


Radiomics in Hypertrophic Cardiomyopathy: The New Tool.

JACC Cardiovasc Imaging 2019 Mar 8. Epub 2019 Mar 8.

Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas. Electronic address:

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http://dx.doi.org/10.1016/j.jcmg.2019.02.004DOI Listing

Image of the month: Ventricular myxoma mimicking hypertrophic cardiomyopathy.

Clin Med (Lond) 2019 Mar;19(2):131-132

Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India; Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India.

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. Read More

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http://dx.doi.org/10.7861/clinmedicine.19-2-131DOI Listing

Molecular characterization of Portuguese patients with dilated cardiomyopathy.

Rev Port Cardiol 2019 Mar 11. Epub 2019 Mar 11.

Department of Medicine, Faculty of Medicine, University of Porto, Portugal; I3S - Institute for Innovation and Health Research, Portugal; Department of Cardiology, São João Hospital Center, Portugal.

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. Read More

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http://dx.doi.org/10.1016/j.repc.2018.10.010DOI Listing

Measuring inorganic phosphate and intracellular pH in the healthy and hypertrophic cardiomyopathy hearts by in vivo 7T P-cardiovascular magnetic resonance spectroscopy.

J Cardiovasc Magn Reson 2019 Mar 14;21(1):19. Epub 2019 Mar 14.

Oxford Centre for Clinical Magnetic Resonance Research (OCMR), Division of Cardiovascular Medicine, BHF Centre of Research Excellence, University of Oxford, Oxford, UK.

Background: Cardiovascular phosphorus MR spectroscopy (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, P-CMRS can also measure cardiac intracellular pH (pH) and the free energy of ATP hydrolysis (ΔG). 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. Read More

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http://dx.doi.org/10.1186/s12968-019-0529-4DOI Listing

The evolution of apical hypertrophic cardiomyopathy: Development of mid-ventricular obstruction and apical aneurysm 11 years after initial diagnosis.

Echocardiography 2019 Mar 13. Epub 2019 Mar 13.

Division of Cardiology, University of Louisville, Louisville, Kentucky.

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. Read More

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http://dx.doi.org/10.1111/echo.14310DOI Listing

Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases.

J Atheroscler Thromb 2019 Mar 12. Epub 2019 Mar 12.

Department of Blood Transfusion Medicine, Nara Medical University.

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. Read More

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http://dx.doi.org/10.5551/jat.RV17031DOI Listing

Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy: A Contemporary Reappraisal.

EuroIntervention 2019 Mar 12. Epub 2019 Mar 12.

Department of Cardiovascular Sciences, Sapienza University, Rome, Italy.

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. Read More

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http://dx.doi.org/10.4244/EIJ-D-18-00959DOI Listing

Danon disease: Gender differences in presentation and outcomes.

Int J Cardiol 2019 Feb 16. Epub 2019 Feb 16.

Division of Cardiology, University of California San Diego, San Diego, CA, USA. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.ijcard.2019.01.020DOI Listing
February 2019

Comparative outcomes of subcutaneous and transvenous cardioverter-defibrillators.

Chin Med J (Engl) 2019 Mar;132(6):631-637

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA.

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. Read More

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http://dx.doi.org/10.1097/CM9.0000000000000133DOI Listing
March 2019
2 Reads

Hypertrophic Obstructive Cardiomyopathy.

Dtsch Arztebl Int 2019 Jan;116(4):47-53

Department of Cardiology, Klinikum Würzburg-Mitte, Juliusspital, Würzburg; Department of Thoracic and Cardiovascular Surgery, Saarland University Hospital, Homburg/Saar; University of Paris Descartes, Sorbonne Paris Cité, Paris, France.

Background: Hypertrophic cardiomyopathy (HCM) is caused by mutations in a number of genes. Its prevalence is 0.2% to 0. Read More

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http://dx.doi.org/10.3238/arztebl.2019.0047DOI Listing
January 2019

Hypertrophic cardiomyopathy: an updated review on diagnosis, prognosis, and treatment.

Heart Fail Rev 2019 Mar 9. Epub 2019 Mar 9.

2nd Cardiology Department, National and Kapodistrian University of Athens, Attikon Hospital, Rimini 1, 12462, Haidari, Greece.

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. Read More

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http://link.springer.com/10.1007/s10741-019-09775-4
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http://dx.doi.org/10.1007/s10741-019-09775-4DOI Listing
March 2019
3 Reads

Mitral Valve Repair Rate at a Veterans Affairs Hospital Utilizing a Multidisciplinary Heart Team.

Semin Thorac Cardiovasc Surg 2019 Mar 5. Epub 2019 Mar 5.

Department of Cardiothoracic Surgery, Baylor College of Medicine/Texas Heart Institute, Houston, Tex; Division of Cardiothoracic Surgery, Michael E. DeBakey Veterans Affairs Hospital, Houston, Tex.

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. Read More

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http://dx.doi.org/10.1053/j.semtcvs.2019.02.028DOI Listing
March 2019
1 Read

Moving beyond simple answers to complex disorders in sarcomeric cardiomyopathies: the role of integrated systems.

Pflugers Arch 2019 Mar 8. Epub 2019 Mar 8.

Department of Biomedical Engineering, University of Arizona, Tucson, AZ, USA.

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. Read More

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http://dx.doi.org/10.1007/s00424-019-02269-0DOI Listing
March 2019
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Optimal choice of OSEM and SD reconstruction algorithms in CZT SPECT for hypertrophic cardiomyopathy patients.

J Nucl Cardiol 2019 Mar 7. Epub 2019 Mar 7.

Department of Quantum Medical Technology, Kanazawa University, Kanazawa, Japan.

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). Read More

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http://dx.doi.org/10.1007/s12350-019-01677-0DOI Listing

Therapeutic Genome Editing in Cardiovascular Diseases.

JACC Basic Transl Sci 2019 Feb 25;4(1):122-131. Epub 2019 Feb 25.

Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon.

A variety of genetic cardiovascular diseases may one day be curable using gene editing technology. Germline genome editing and correction promises to permanently remove monogenic cardiovascular disorders from the offspring and subsequent generations of affected families. Although technically feasible and likely to be ready for implementation in humans in the near future, this approach remains ethically controversial. Read More

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http://dx.doi.org/10.1016/j.jacbts.2018.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390678PMC
February 2019

Anti-arrhythmic therapy in patients with non-ischemic cardiomyopathy.

Pharmacol Res 2019 Mar 4;143:27-32. Epub 2019 Mar 4.

Division of Cardiology, McGill University Health Center, Montreal, Canada. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.phrs.2019.03.004DOI Listing
March 2019
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Cardiomyopathy Mutations in Metavinculin Disrupt Regulation of Vinculin-Induced F-Actin Assemblies.

J Mol Biol 2019 Mar 4. Epub 2019 Mar 4.

Department of Biochemistry and Biophysics and Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. Electronic address:

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). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00222836193010
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http://dx.doi.org/10.1016/j.jmb.2019.02.024DOI Listing
March 2019
2 Reads

TGR5 activation ameliorates hyperglycemia-induced cardiac hypertrophy in H9c2 cells.

Sci Rep 2019 Mar 6;9(1):3633. Epub 2019 Mar 6.

Department of Medical Research, Chi-Mei Medical Center, Yong Kang, Tainan City, 71003, Taiwan.

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. Read More

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http://dx.doi.org/10.1038/s41598-019-40002-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6403401PMC

Prevalence and morphology of myocardial crypts in normal and hypertrophied myocardium by computed tomography.

Int J Cardiovasc Imaging 2019 Mar 5. Epub 2019 Mar 5.

Department of Cardiology, Rabin Medical Center - Beilinson Hospital, 49100, Petach Tikva, Israel.

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. Read More

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http://dx.doi.org/10.1007/s10554-019-01543-7DOI Listing

Hypertrophic Obstructive Cardiomyopathy and Subvalvular Mitral Apparatus Remodeling.

Ann Thorac Surg 2019 Mar 2. Epub 2019 Mar 2.

Cardiac Surgery and Heart Transplantation Unit, IRCCS-ISMETT, Via Tricomi 5, 90127 Palermo Italy.

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http://dx.doi.org/10.1016/j.athoracsur.2019.01.056DOI Listing
March 2019
1 Read

Disruption of embryonic ROCK signaling reproduces the sarcomeric phenotype of hypertrophic cardiomyopathy.

JCI Insight 2019 Mar 5;5. Epub 2019 Mar 5.

Sarcomeric disarray is a hallmark of gene mutations in patients with Hypertrophic Cardiomyopathy (HCM). However, it is unknown when detrimental sarcomeric changes first occur and whether they originate in the developing embryonic heart. Furthermore, Rho Kinase (ROCK) is a serine threonine protein kinase that is critical for regulating the function of several sarcomeric proteins and therefore, our aim was to determine if disruption of ROCK signalling during the earliest stages of heart development would disrupt the integrity of sarcomeres altering heart development and function. Read More

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http://dx.doi.org/10.1172/jci.insight.125172DOI Listing

Cardiac hypertrophy and arrhythmia in mice induced by a mutation in ryanodine receptor 2.

JCI Insight 2019 Mar 5;5. Epub 2019 Mar 5.

Hypertrophic cardiomyopathy (HCM) is triggered mainly by mutations in genes encoding sarcomeric proteins, but a significant proportion of patients lack a genetic diagnosis. We identified a novel mutation in the ryanodine receptor 2, RyR2-P1124L, in a patient from a genotype-negative HCM cohort. The aim of this study was to determine whether RyR2-P1124L triggers functional and structural alterations in isolated RyR2 channels and whole hearts. Read More

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http://dx.doi.org/10.1172/jci.insight.126544DOI Listing

Dilated phase of hypertrophic cardiomyopathy: Cardiac magnetic resonance evolutive assessment.

Rev Clin Esp 2019 Mar 1. Epub 2019 Mar 1.

Servicio de Cardiología, Hospital Universitario de La Princesa, Madrid, España.

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http://dx.doi.org/10.1016/j.rce.2019.01.008DOI Listing
March 2019
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Does the Flow Know? Mitral Regurgitant Jet Direction and Need for Valve Repair in Hypertrophic Obstructive Cardiomyopathy.

J Am Soc Echocardiogr 2019 Mar;32(3):341-343

Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Boston, Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.echo.2019.01.005DOI Listing

Fabry disease in cardiology practice: Literature review and expert point of view.

Arch Cardiovasc Dis 2019 Feb 27. Epub 2019 Feb 27.

French Referral Centre for Fabry Disease, Division of Medical Genetics, hôpital Raymond-Poincare, AP-HP, 92380 Garches, France; Inserm U1179, University of Versailles, 78180 Montigny, France.

Fabry disease is an X-linked progressive multisystemic genetic sphingolipidosis caused by deficient activity of lysosomal α-galactosidase A. Men aged>30 years and women aged>40 years most often present with unexplained left ventricular hypertrophy, usually concentric and non-obstructive, but sometimes mimicking sarcomeric hypertrophic cardiomyopathy, particularly when isolated, as in the cardiac or late-onset variant of the disease. In hypertrophic cardiomyopathy cohorts, up to 1% of patients have been diagnosed with Fabry disease. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18752136193003
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http://dx.doi.org/10.1016/j.acvd.2019.01.002DOI Listing
February 2019
3 Reads

Myocardial adaptation after surgical therapy differs for aortic valve stenosis and hypertrophic obstructive cardiomyopathy.

Int J Cardiovasc Imaging 2019 Mar 1. Epub 2019 Mar 1.

Department of Cardiology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Cardiovascular Sciences, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands.

Surgical therapies in aortic valve stenosis (AVS) and hypertrophic obstructive cardiomyopathy (HOCM) aim to relief intraventricular pressure overload and improve clinical outcome. It is currently unknown to what extent myocardial adaptation concurs with restoration of intraventricular pressures, and whether this is similar in both patient groups. The aim of this study was to investigate changes in myocardial adaptation after surgical therapies for AVS and HOCM. Read More

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http://dx.doi.org/10.1007/s10554-019-01563-3DOI Listing
March 2019
1 Read

Diagnosis and Management of a Cardiac Amyloidosis Case Mimicking Hypertrophic Cardiomyopathy.

Cureus 2018 Dec 18;10(12):e3749. Epub 2018 Dec 18.

Internal Medicine, American University of Integrative Sciences, Tucker, BRB.

Cardiac amyloidosis is an acquired heart disease secondary to the deposition of β-pleated amyloid proteins in heart tissue. Amyloid light chain (AL) amyloidosis is usually secondary to multiple myeloma and can rapidly deteriorate cardiac function, with high mortality. Up to 50% of AL patients have cardiac involvement presenting as heart failure, conduction abnormalities, and cardiomyopathies. Read More

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http://dx.doi.org/10.7759/cureus.3749DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6388819PMC
December 2018
1 Read

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy.

Cardiology 2019 Mar 1;141(4):202-211. Epub 2019 Mar 1.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing,

Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a myocardial disease characterized by fibrosis and microvascular ischemia. Microvessels play a critical role in myocardial fibrosis in HOCM. However, it remains unclear whether or not myocardial fibrosis is associated with microvascular density (MVD) changes. Read More

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http://dx.doi.org/10.1159/000493005DOI Listing

Siblings with lethal primary pulmonary hypoplasia and compound heterozygous variants in the gene: further delineation of the phenotypic spectrum.

Cold Spring Harb Mol Case Stud 2019 Feb 28. Epub 2019 Feb 28.

Yale University School of Medicine;

Variants in the mitochondrial alanyl-tRNA synthetase 2 gene (OMIM 612035) are associated with infantile mitochondrial cardiomyopathy or later-onset leukoencephalopathy with premature ovarian insufficiency. Here, we report two newborn siblings who died soon after birth with primary pulmonary hypoplasia without evidence of cardiomyopathy. Whole exome sequencing detected the same compound heterozygous variants in both siblings (c. Read More

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http://dx.doi.org/10.1101/mcs.a003699DOI Listing
February 2019
2 Reads

Update on alcohol septal ablation for hypertrophic obstructive cardiomyopathy.

Kardiol Pol 2019 ;77(2):160-161

Department of Cardiology, University Hospital Motol and 2nd Medical School, Charles University, Prague, Czech Republic.

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http://dx.doi.org/10.5603/KP.2019.0019DOI Listing
January 2019
1 Read

Survival Differences in Women and Men After Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy.

JAMA Cardiol 2019 Feb 27. Epub 2019 Feb 27.

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota.

Importance: Recent data indicate that women with hypertrophic cardiomyopathy (HCM) are older and more symptomatic at presentation and have worse clinical outcomes than men. However, to our knowledge, there are no large studies of the association of patient sex with outcomes after surgical myectomy.

Objective: To analyze preoperative characteristics and overall survival of women and men undergoing septal myectomy for obstructive HCM. Read More

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http://cardiology.jamanetwork.com/article.aspx?doi=10.1001/j
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http://dx.doi.org/10.1001/jamacardio.2019.0084DOI Listing
February 2019
3 Reads

Electroconvulsive therapy in a patient with hypertrophic obstructive cardiomyopathy.

Aust N Z J Psychiatry 2019 Feb 26:4867419831792. Epub 2019 Feb 26.

3 Department of Cardiology, Prince of Wales Hospital and Community Health Service, Randwick, NSW, Australia.

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http://dx.doi.org/10.1177/0004867419831792DOI Listing
February 2019
2 Reads

Exercising caution: is there a role for exercise testing in the hypertrophic cardiomyopathy population?

J Physiol 2019 Feb 26. Epub 2019 Feb 26.

Faculty of Kinesiology and Physical Education, University of Toronto, Toronto, ON, Canada.

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http://dx.doi.org/10.1113/JP277778DOI Listing
February 2019

Anesthetic Management in an Adult Patient With Costello Syndrome: A Case Report.

A A Pract 2019 Feb 21. Epub 2019 Feb 21.

From the Department of Anesthesiology, Shimane University Faculty of Medicine, Shimane, Japan.

Costello syndrome is a rare genetic disorder characterized by mental and growth retardation and distinctive coarse facies. A significant proportion of patients with Costello syndrome have hypertrophic cardiomyopathy, papillomata, and malignant tumors. General anesthesia practice, especially airway and cardiac management, in patients with Costello syndrome may be complicated by anatomical features and cardiac abnormalities. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000983DOI Listing
February 2019
1 Read

High T2-weighted signal intensity is associated with myocardial deformation in hypertrophic cardiomyopathy.

Sci Rep 2019 Feb 25;9(1):2644. Epub 2019 Feb 25.

Department of Radiology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

The association between global and segmental myocardial strain impairment and fibrosis extent in hypertrophic cardiomyopathy (HCM) is widely verified. The aim of this study was to investigate the contribution of high T2-weighted signal intensity (HighT2) to myocardial deformation in HCM. We prospectively recruited 57 patients with HCM examined by a 3. Read More

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http://dx.doi.org/10.1038/s41598-019-39456-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390098PMC
February 2019

Possibly Late-Onset Arrhythmogenic Right Ventricular Cardiomyopathy: Unique Triglyceride Deposition by Analysis of Lipid Contents.

Clin Med Insights Case Rep 2019 15;12:1179547619828715. Epub 2019 Feb 15.

Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Japan.

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man's death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. Read More

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http://dx.doi.org/10.1177/1179547619828715DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378422PMC
February 2019

Impaired Right Ventricular Mechanics at Rest and During Exercise Are Associated With Exercise Capacity in Patients With Hypertrophic Cardiomyopathy.

J Am Heart Assoc 2019 Mar;8(5):e011269

1 Department of Echocardiography Heart Center Beijing ChaoYang Hospital Capital Medical University Beijing China.

Background Impaired right ventricular ( RV ) function indicates RV involvement in patients with hypertrophic cardiomyopathy ( HCM ). We aimed to assess RV function at rest and during exercise in HCM patients and to examine the association between impaired RV mechanics and exercise capacity. Methods and Results A total of 76 HCM patients (48 without and 28 with RV hypertrophy) and 30 age- and sex-matched controls were prospectively recruited. Read More

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https://www.ahajournals.org/doi/10.1161/JAHA.118.011269
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http://dx.doi.org/10.1161/JAHA.118.011269DOI Listing
March 2019
2 Reads

Targeted DNA Methylation Profiling of Human Cardiac Tissue Reveals Novel Epigenetic Traits and Gene Deregulation Across Different Heart Failure Patient Subtypes.

Circ Heart Fail 2019 Mar;12(3):e005765

UCD Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Ireland (N.G., B.M., A.R.-H., D.P.O., W.M.G., K.M., M.L., J.B., S.D., C.J.W.).

Background: Limited knowledge exists of the extent of epigenetic alterations, such as DNA methylation, in heart failure (HF). We conducted targeted DNA methylation sequencing to identify DNA methylation alterations in coding and noncoding RNA (ncRNA) across different etiological subtypes of HF.

Methods And Results: A targeted bisulfite sequence capture sequencing platform was applied to DNA extracted from cardiac interventricular septal tissue of 30 male HF patients encompassing causes including hypertrophic obstructive cardiomyopathy, ischemic cardiomyopathy, dilated cardiomyopathy, and 9 control patients with nonfailing hearts. Read More

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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.118.005765DOI Listing
March 2019
2 Reads

Sub for SAM: Mitral Subvalvular Interventions in Hypertrophic Cardiomyopathy.

Authors:
Tomasz A Timek

Semin Thorac Cardiovasc Surg 2019 Feb 20. Epub 2019 Feb 20.

Division of Cardiothoracic Surgery Spectrum Health Clinical Associate Professor Michigan State University College of Human Medicine, 100 Michigan Ave NE, Grand Rapids, Michigan. Electronic address:

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http://dx.doi.org/10.1053/j.semtcvs.2019.02.018DOI Listing
February 2019

Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Patient with MYL2-R58Q-Mediated Apical Hypertrophic Cardiomyopathy Show Hypertrophy, Myofibrillar Disarray, and Calcium Perturbations.

J Cardiovasc Transl Res 2019 Feb 22. Epub 2019 Feb 22.

Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN, 55905, USA.

Hypertrophic cardiomyopathy (HCM), characterized by unexplained left ventricular hypertrophy, is one of the most common heritable cardiovascular diseases. The myosin regulatory light chain (MYL2) mutation R58Q has been associated with severe cardiac hypertrophy and sudden cardiac death (SCD). Herein, we provide the first patient-specific, induced pluripotent stem cell-derived cardiomyocyte (iPSC-CM) model of MYL2-R58Q. Read More

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http://dx.doi.org/10.1007/s12265-019-09873-6DOI Listing
February 2019
1 Read

Clinical significance of evaluating coronary atherosclerosis in adult patients with hypertrophic cardiomyopathy who have chest pain.

Eur Radiol 2019 Feb 22. Epub 2019 Feb 22.

Department of Radiology, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, Gyeonggi-do, 13620, South Korea.

Objective: Chest pain is a common symptom in patients with hypertrophic cardiomyopathy (HCM), causing difficulty determining whether there is coexistent coronary artery disease (CAD). We investigated whether coronary computed tomography angiography (CCTA) can assess the prevalence and clinical significance of CAD in adult patients with HCM showing chest pain through longitudinal follow-up.

Methods: In 238 adult patients with HCM, who underwent CCTA for chest pain, we analyzed the degree of stenosis and adverse plaque characteristics (APCs) as CCTA variables. Read More

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http://dx.doi.org/10.1007/s00330-018-5951-8DOI Listing
February 2019

Long non-coding RNA cytoskeleton regulator RNA (CYTOR) modulates pathological cardiac hypertrophy through miR-155-mediated IKKi signaling.

Biochim Biophys Acta Mol Basis Dis 2019 Feb 19. Epub 2019 Feb 19.

Department of Cardiology, Renmin Hospital of Wuhan University, Cardiovascular Research Institute of Wuhan University, Hubei Key Laboratory of Cardiology, Wuhan 430060, China; Department of Cardiology, The Fifth Affiliated Hospital of Xin Jiang Medical University, Urumchi 830001, China; Qianjiang Central Hospital of Hubei Province, Qianjiang 433100, China. Electronic address:

Pathological cardiac hypertrophy, which may lead to heart failure and sudden death, can be affected by multiple factors. In our previous study, we revealed that IKKi deficiency induced cardiac hypertrophy through the activation of the AKT and NF-kB signaling pathway in response to aortic banding (AB). Non-coding RNAs, mainly long non-coding RNAs (lncRNAs) and microRNAs (miRNAs), play a crucial role in normal developmental and pathological processes. Read More

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http://dx.doi.org/10.1016/j.bbadis.2019.02.014DOI Listing
February 2019
4.882 Impact Factor

Role of Preoperative Cardiovascular Magnetic Resonance in Planning Ventricular Septal Myectomy in Patients With Obstructive Hypertrophic Cardiomyopathy.

Am J Cardiol 2019 Feb 8. Epub 2019 Feb 8.

Policlinico di Monza, Monza, Italy.

In obstructive hypertrophic cardiomyopathy (HC), extreme heterogeneity of septal morphology makes septal myectomy particularly challenging. Although cardiovascular magnetic resonance (CMR) reconstructs ventricular anatomy with high spatial resolution, CMR is not used systematically to plan preoperatively septal myectomy. In this study, we report our results with using CMR to plan the extent of septal excision in 112 consecutive HC patients who subsequently underwent myectomy. Read More

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http://dx.doi.org/10.1016/j.amjcard.2019.01.041DOI Listing
February 2019
3 Reads

Left atrial fibrosis correlates with extent of left ventricular myocardial delayed enhancement and left ventricular strain in hypertrophic cardiomyopathy.

Int J Cardiovasc Imaging 2019 Feb 21. Epub 2019 Feb 21.

Department of Internal Medicine (Cardiology), Yale School of Medicine, 330 Cedar St, Boardman 110, P.O. Box 208056, New Haven, CT, 06520-8056, USA.

Hypertrophic cardiomyopathy (HCM) is associated with increased left ventricular (LV) mass, decreased myocardial strain, and the presence of LV fibrosis and scar. The relationship between LV scar and fibrosis with left atrial (LA) fibrosis in the setting of HCM has not been examined. The purpose of this study is to demonstrate a correlation between the degree of LA fibrosis and LV parameters in subjects with HCM. Read More

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http://dx.doi.org/10.1007/s10554-019-01551-7DOI Listing
February 2019
2 Reads

[Hypertrophic cardiomyopathy: current state of the problem].

Khirurgiia (Mosk) 2019 (1):83-88

Petrovsky Russian Research Center for Surgery, Moscow, Russia.

The main aspects of epidemiology, pathophysiology, medication and surgical treatment of hypertrophic cardiomyopathy (HCM) are reviewed in the article. The authors emphasize that surgical treatment is still the method of choice for HCM and can guarantee excellent immediate and long-term results with minimal risk. Read More

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http://dx.doi.org/10.17116/hirurgia201901183DOI Listing
January 2019

The spectrum of genetic variants and phenotypic features of Southeast Asian patients with Noonan syndrome.

Mol Genet Genomic Med 2019 Feb 19:e581. Epub 2019 Feb 19.

Genetics Service, Department of Paediatrics, KK Women's & Children's Hospital, Singapore.

Background: Noonan syndrome (NS) is an autosomal dominant disorder that belongs to a group of developmental disorders called RASopathies with overlapping features and multiple causative genes. The aim of the study was to identify mutations underlying this disorder in patients from Southeast Asia and characterize their clinical presentations.

Methods: Patients were identified from the hospital's Genetics clinics after assessment by attending clinical geneticists. Read More

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http://dx.doi.org/10.1002/mgg3.581DOI Listing
February 2019