19,116 results match your criteria Cardiomyopathy Hypertrophic


The detection of apical variant of hypertrophic cardiomyopathy in myocardial perfusion imaging.

Acta Cardiol 2019 Jan 16:1-3. Epub 2019 Jan 16.

a Department of Nuclear Medicine , Ankara Training and Research Hospital, University of Health Sciences , Ankara , Turkey.

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http://dx.doi.org/10.1080/00015385.2018.1537959DOI Listing
January 2019

Exploration of time sequential, patient specific 3D heart unlocks clinical understanding.

3D Print Med 2018 Dec 6;4(1):15. Epub 2018 Dec 6.

University of Illinois College of Medicine, 1 Illini Drive, Peoria, IL, 61605, USA.

Objectives: The purpose was to create a time sequential three-dimensional virtual reality model, also referred to as a four-dimensional model, to explore its possible benefit and clinical applications. We hypothesized that this novel solution allows for the visuospatial benefits of the 3D model and the dynamic benefits of other existing imaging modalities.

Background: We have seen how 3D models hold great value in medical decision making by eliminating the variable visuospatial skills of practitioners. Read More

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http://dx.doi.org/10.1186/s41205-018-0034-7DOI Listing
December 2018

Histopathological Aspects of the Myocardium in Dilated Cardiomyopathy.

Curr Health Sci J 2018 Jul-Sep;44(3):243-249. Epub 2018 Jul 15.

Department of Research Methodology, University of Medicine and Pharmacy of Craiova.

Dilated cardiomyopathy is the most common form of cardiac muscle disease, accounting for approximately 60% of all cardiomyopathies. We proposed to identify histopathological changes of the myocardium in dilative cardiomyopathy. This study comprised a total of 19 cases, represented by myocardial fragments from deceased patients with diagnosis of dilated cardiomyopathy. Read More

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http://dx.doi.org/10.12865/CHSJ.44.03.07DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311227PMC

Apical hypertrophic cardiomyopathy: diagnosis, medical and surgical treatment.

Kardiochir Torakochirurgia Pol 2018 Dec 31;15(4):246-253. Epub 2018 Dec 31.

Cardiology Institute and Clinic, Poznan University of Medical Sciences, Poznan, Poland.

Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy, occasionally resulting in severe complications. The paper covers the etiology and pathogenesis of AHCM, different imaging methods and characteristic appearance of the disease in each of them. Echocardiography and cardiovascular magnetic resonance imaging (CMR) are known to be the most valuable imaging methods. Read More

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http://dx.doi.org/10.5114/kitp.2018.80922DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329883PMC
December 2018

Young adult with Friedreich ataxia.

Heart 2019 Jan 15. Epub 2019 Jan 15.

CMR Unit, Bristol Heart Institute, University of Bristol and University Hospitals Bristol NHS Foundation Trust, Bristol, UK.

Clinical Introduction: A young adult with Friedreich ataxia complaining of exertional breathlessness underwent a cardiological evaluation. On physical examination, high blood pressure and a loud systolic murmur were noted. ECG showed sinus rhythm with voltage criteria for left ventricular hypertrophy (LVH) and T-wave changes in the inferolateral leads. Read More

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http://dx.doi.org/10.1136/heartjnl-2018-314387DOI Listing
January 2019

Key Value of RNA Analysis of MYBPC3 Splice-Site Variants in Hypertrophic Cardiomyopathy.

Circ Genom Precis Med 2019 Jan;12(1):e002368

Agnes Ginges Centre for Molecular Cardiology Centenary Institute, The University of Sydney, Australia (E.S.S., J.I., C.S., R.D.B.).

Background: MYBPC3 splicing errors are a common cause of hypertrophic cardiomyopathy (HCM). Variants affecting essential splice-site dinucleotides inhibit splicing, whereas the impact of variants at conserved flanking nucleotides is less clear. We evaluated the contribution of MYBPC3 splice-site variants in a large cohort of patients with HCM and assessed the impact on splicing with RNA analysis. Read More

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http://dx.doi.org/10.1161/CIRCGEN.118.002368DOI Listing
January 2019

Region-resolved proteomics profiling of monkey heart.

J Cell Physiol 2019 Jan 15. Epub 2019 Jan 15.

Key Laboratory of Animal Models and Human Disease Mechanisms of Chinese Academy of Sciences &Yunnan Province, Kunming Institute of Zoology, Chinese Academy of Sciences (CAS), Kunming, Yunnan, China.

Nonhuman primates (NHPs) play an indispensable role in biomedical research because of their similarities in genetics, physiological, and neurological function to humans. Proteomics profiling of monkey heart could reveal significant cardiac biomarkers and help us to gain a better understanding of the pathogenesis of heart disease. However, the proteomic study of monkey heart is relatively lacking. Read More

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http://dx.doi.org/10.1002/jcp.28052DOI Listing
January 2019

Left ventricular contractile reserve in stress echocardiography: the bright side of the force.

Kardiol Pol 2019 Jan 15. Epub 2019 Jan 15.

Institute of Clinical Physiology, National Council Research, Pisa, Italy.

Stress echocardiography (SE) is based on the detection of regional wall motion abnormalities (RWMA) mirroring a physiologically critical epicardial artery stenosis determining subendocardial underperfusion. Recently the core protocol of SE was enriched by adding left ventricular contractile reserve (LVCR) based on Force. An altered Force can be due to microvascular and/or epicardial coronary artery disease, but also to myocardial scar, necrosis and/or sub-epicardial layer disease. Read More

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http://dx.doi.org/10.5603/KP.a2019.0002DOI Listing
January 2019

A case of hypertrophic cardiomyopathy combined with muscular ventricular septal defect and abnormal origin of right coronary artery.

BMC Cardiovasc Disord 2019 Jan 14;19(1):16. Epub 2019 Jan 14.

Department of ultrasound, Renmin Hospital, Hubei University of Medicine, Shiyan, 442000, Hubei, China.

Background: Hypertrophic cardiomyopathy (HCM) is a myocardial disease. However, the coexistence of HCM with muscular ventricular septal defect (VSD), especially those with both incomplete spontaneous closure and coronary abnormal origin, is relatively rare.

Case Presentation: We report herein a unique case of HCM accompanied with incomplete spontaneous closure of muscular VSD and abnormal origin of right coronary artery (RCA) in a 26-year-old man, which was diagnosed by combination of transthoracic 2-dimensional (2D), color Doppler, Contrast-enhanced echocardiography and computed tomography angiography (CTA). Read More

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http://dx.doi.org/10.1186/s12872-018-0997-8DOI Listing
January 2019

Septal myectomy with versus without subvalvular apparatus intervention in patients with hypertrophic obstructive cardiomyopathy: a prospective randomized study.

Semin Thorac Cardiovasc Surg 2019 Jan 11. Epub 2019 Jan 11.

Heart Valve Surgery Department, Meshalkin National Medical Research Center, Novosibirsk, Russian Federation.

Objective: Surgical septal myectomy is a standard treatment option for patients with hypertrophic obstructive cardiomyopathy. Subvalvular abnormalities of the mitral valve might play an important role in residual left ventricular outflow tract obstruction. This randomized study aimed to compare the surgical outcomes of septal myectomy with versus without subvalvular interventions. Read More

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http://dx.doi.org/10.1053/j.semtcvs.2019.01.011DOI Listing
January 2019

Mid-term outcomes of simultaneous coronary artery bypass graft surgery and septal myectomy in patients with hypertrophic obstructive cardiomyopathy: A case-controlled study.

J Card Surg 2019 Jan 12. Epub 2019 Jan 12.

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

Background: The mid-term outcome of patients with hypertrophic obstructive cardiomyopathy (HOCM) undergoing coronary artery bypass graft (CABG) is unclear.

Materials And Methods: We studied 44 patients with HOCM and coronary artery disease (CAD) who underwent septal myectomy and CABG) between 2011 and 2017. The control group was matched in a ratio of 4:1 based on age, sex, body mass index, hypertension, and chest pain. Read More

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http://dx.doi.org/10.1111/jocs.13988DOI Listing
January 2019

Transplacental Delivery of Genome Editing Components Causes Mutations in Embryonic Cardiomyocytes of Mid-Gestational Murine Fetuses.

IUBMB Life 2019 Jan 11. Epub 2019 Jan 11.

Section of Gene Expression Regulation, Frontier Science Research Center, Kagoshima University, Kagoshima, Kagoshima, 890-8544, Japan.

Genome editing, as exemplified by CRISPR/Cas9, is now recognized as a powerful tool for the engineering of endogenous target genes. It employs only two components, namely, Cas9 in the form of DNA, mRNA, or protein; and guide RNA (gRNA), which is specific to a target gene. When these components are transferred to cells, they create insertion/deletion mutations (indels) within a target gene. Read More

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http://dx.doi.org/10.1002/iub.2004DOI Listing
January 2019
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Ferroptosis as a novel therapeutic target for Friedreich's ataxia.

J Pharmacol Exp Ther 2019 Jan 11. Epub 2019 Jan 11.

Children's Hospital of Philadelphia;

Friedreich ataxia (FRDA) is a progressive neuro- and cardio-degenerative disorder characterized by ataxia, sensory loss, and hypertrophic cardiomyopathy. In most cases, the disorder is caused by GAA repeat expansions in the first introns of both alleles of the FXN gene, resulting in decreased expression of the encoded protein, frataxin. Frataxin localizes to the mitochondrial matrix and is required for iron-sulfur-cluster biosynthesis. Read More

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http://dx.doi.org/10.1124/jpet.118.252759DOI Listing
January 2019
1 Read

Atrial fibrillation history impact on catheter ablation outcome. Findings from the ESC-EHRA atrial fibrillation ablation long term registry.

Pacing Clin Electrophysiol 2019 Jan 10. Epub 2019 Jan 10.

Clinica Mediterranea, Napoli, Italy.

Background: Atrial fibrillation (AF) promotes atrial remodeling that in turn promote AF perpetuation. The aim of our study was to investigate the impact of AF history length on one-year outcome of AF catheter ablation in a cohort of patients enrolled in the Atrial Fibrillation Ablation Registry.

Methods: We described the real-life clinical epidemiology, therapeutic strategies and the short- and mid-term outcomes of 1948 patients (71. Read More

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http://doi.wiley.com/10.1111/pace.13600
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http://dx.doi.org/10.1111/pace.13600DOI Listing
January 2019
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The hypertrophic cardiomyopathy paradox: better with age.

Eur Heart J 2019 Jan 9. Epub 2019 Jan 9.

Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, USA.

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https://academic.oup.com/eurheartj/advance-article/doi/10.10
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http://dx.doi.org/10.1093/eurheartj/ehy889DOI Listing
January 2019
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Associations Between Multiple Circulating Biomarkers and the Presence of Atrial Fibrillation in Hypertrophic Cardiomyopathy with or Without Left Ventricular Outflow Tract Obstruction.

Int Heart J 2019 Jan 10. Epub 2019 Jan 10.

Clinical EP Lab & Arrhythmia Center, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College.

Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). Data regarding the correlations of biomarkers and AF in HCM patients are rather limited. We sought to explore the associations between the presence of AF and circulating biomarkers reflecting cardiovascular function (N-terminal pro-brain natriuretic peptide, NT-pro BNP), endothelial function (big endothelin-1, big ET-1), inflammation (high-sensitivity C-reactive protein), and myocardial damage (cardiac troponin I, cTnI) in HCM patients with and without left ventricular outflow tract obstruction (LVOTO). Read More

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https://www.jstage.jst.go.jp/article/ihj/advpub/0/advpub_18-
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http://dx.doi.org/10.1536/ihj.18-438DOI Listing
January 2019
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Prevalence, determinants, and prognostic significance of exercise-induced pulmonary hypertension in patients with hypertrophic cardiomyopathy.

Int J Cardiovasc Imaging 2019 Jan 8. Epub 2019 Jan 8.

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.

Exercise-induced pulmonary hypertension (EIPH) is associated with worse outcomes in patients with heart failure or valvular heart disease. However, little is known regarding the implications of EIPH in hypertrophic cardiomyopathy (HCM) patients. We retrospectively reviewed data of consecutive HCM patients who underwent clinically indicated exercise echocardiography using a semi-supine bicycle ergometer at our hospital. Read More

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http://dx.doi.org/10.1007/s10554-018-01522-4DOI Listing
January 2019

Correction to: Cardiovascular causes of sudden unexpected death in children and adolescents (0-17 years) : A nationwide autopsy study in the Netherlands.

Neth Heart J 2019 Jan 8. Epub 2019 Jan 8.

Department of Pathology, University Medical Center, Utrecht, The Netherlands.

Correction to:Neth Heart J 2018 https://doi.org/10.1007/s12471-018-1152-y In the version of the article originally published online, there was an error in the 'Methods and results' section of the Abstract. Read More

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http://dx.doi.org/10.1007/s12471-018-1219-9DOI Listing
January 2019

Novel Adult-Onset Systolic Cardiomyopathy Due to MYH7 E848G Mutation in Patient-Derived Induced Pluripotent Stem Cells.

JACC Basic Transl Sci 2018 Dec 31;3(6):728-740. Epub 2018 Dec 31.

Department of Medicine/Cardiology, University of Washington, Seattle, Washington.

A novel myosin heavy chain 7 mutation (E848G) identified in a familial cardiomyopathy was studied in patient-specific induced pluripotent stem cell-derived cardiomyocytes. The cardiomyopathic human induced pluripotent stem cell-derived cardiomyocytes exhibited reduced contractile function as single cells and engineered heart tissues, and genome-edited isogenic cells confirmed the pathogenic nature of the E848G mutation. Reduced contractility may result from impaired interaction between myosin heavy chain 7 and cardiac myosin binding protein C. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S2452302X183022
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http://dx.doi.org/10.1016/j.jacbts.2018.08.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314962PMC
December 2018
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Characterization of the L29Q hypertrophic cardiomyopathy mutation in cardiac Troponin C by Paramagnetic Relaxation Enhancement NMR.

Biochemistry 2019 Jan 8. Epub 2019 Jan 8.

The key events in regulating muscle contraction involve the Troponin (Tn) heterotrimeric protein complex where the binding to and release of Ca from the highly conserved Troponin C (TnC) subunit triggers a series of structural changes within Tn, and the other thin filament proteins, to result in contraction. In the heart, the control of contraction and relaxation events can be altered by many single point mutations which may result in cardiomyopathy and sometimes sudden cardiac death. Here we have examined the structural effects of one hypertrophic cardiomyopathy mutation, L29Q, on Ca induced structural transitions within whole TnC. Read More

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http://dx.doi.org/10.1021/acs.biochem.8b01140DOI Listing
January 2019
3.015 Impact Factor

Review on sudden death risk reduction after septal reduction therapies in hypertrophic obstructive cardiomyopathy.

Heart Fail Rev 2019 Jan 8. Epub 2019 Jan 8.

Mid-German Heart Center, Department of Internal Medicine III, Division of Cardiology, Angiology and Intensive Medical Care, University Hospital Halle, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Strasse 40, 06120, Halle (Saale), Germany.

Treatment of left ventricular outflow tract (LVOT) obstruction in hypertrophic obstructive cardiomyopathy (HOCM) with septal reduction, either with myectomy or alcohol septal ablation, is aiming to reduce the LVOT gradient and improve symptoms in patients who are refractory to or do not tolerate medical treatment. Apart from contributing to the evolution to heart failure, LVOT obstruction is considered a risk factor for sudden cardiac death (SCD). Both septal reduction treatments have been proven effective in reducing symptoms and seem to improve survival, which has been shown equal to the expected in the normal population. Read More

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http://dx.doi.org/10.1007/s10741-018-09767-wDOI Listing
January 2019

Progressive nature of myocardial fibrosis in pediatric hypertrophic cardiomyopathy: from mutation carrier to myocardial fibrosis.

Ann Transl Med 2018 Nov;6(Suppl 1):S52

Division of Cardiology, Cardiovascular Center, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.

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http://dx.doi.org/10.21037/atm.2018.10.07DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291561PMC
November 2018

Predictors of β-blocker adherence in cardiac inherited disease.

Open Heart 2018 16;5(2):e000877. Epub 2018 Dec 16.

Department of Psychological Medicine, The University of Auckland, Auckland, New Zealand.

Objective: The cardiac inherited disease (CID) population has suboptimal adherence to long-term β-blocker therapy, which is known to be a risk for sudden cardiac death. This study aimed to identify the clinical and psychosocial variables associated with non-adherence in this population.

Methods: 130 individuals (aged 16-81 years, median: 54) from the New Zealand Cardiac Inherited Disease Registry taking β-blockers participated: 65 (50%) long QT syndrome, 42 (32%) hypertrophic cardiomyopathy and 23 (18%) other. Read More

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http://openheart.bmj.com/lookup/doi/10.1136/openhrt-2018-000
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http://dx.doi.org/10.1136/openhrt-2018-000877DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307606PMC
December 2018
1 Read

The HCM-causing Y235S cMyBPC mutation accelerates contractile function by altering C1 domain structure.

Biochim Biophys Acta Mol Basis Dis 2019 Jan 3;1865(3):661-677. Epub 2019 Jan 3.

Department of Physiology and Biophysics, School of Medicine, Case Western Reserve University, Cleveland, OH, USA. Electronic address:

Mutations in cardiac myosin binding protein C (cMyBPC) are a major cause of hypertrophic cardiomyopathy (HCM). In particular, a single amino acid substitution of tyrosine to serine at residue 237 in humans (residue 235 in mice) has been linked to HCM with strong disease association. Although cMyBPC truncations, deletions and insertions, and frame shift mutations have been studied, relatively little is known about the functional consequences of missense mutations in cMyBPC. Read More

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http://dx.doi.org/10.1016/j.bbadis.2019.01.007DOI Listing
January 2019

Cost Efficacy of α-Galactosidase A Enzyme Screening for Fabry Disease.

Mayo Clin Proc 2019 Jan;94(1):84-88

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN; Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN; Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN.

The prevalence of Fabry disease (FD) in adult patients with suspected hypertrophic cardiomyopathy (HCM) has been reported between 0.3% and 4%. Fabry disease-specific therapy necessitates early diagnosis; however, the optimal screening strategy and cost efficacy of routine α-galactosidase A (α-gal A) vs comprehensive galactosidase alpha gene (GLA) testing remain poorly understood. Read More

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http://dx.doi.org/10.1016/j.mayocp.2018.08.009DOI Listing
January 2019

Septal Myectomy in Hypertrophic Cardiomyopathy: National Outcomes of Concomitant Mitral Surgery.

Mayo Clin Proc 2019 Jan;94(1):66-73

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN. Electronic address:

Objectives: To assess the frequency and implications of mitral valve (MV) surgery at the time of septal myectomy (SM) for hypertrophic cardiomyopathy (HCM) in a national cohort.

Patients And Methods: The National Inpatient Sample (NIS) was used to analyze surgical outcomes in patients with HCM undergoing SM from January 1, 2003, through December 31, 2014. Univariate analyses were used to compare patients undergoing SM with vs without concomitant procedures, and logistic regression was used to determine factors associated with prolonged length of stay (LOS) and in-hospital mortality. Read More

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http://dx.doi.org/10.1016/j.mayocp.2018.07.022DOI Listing
January 2019

Rare Association of Mucolipidosis III alpha/beta with Dilated Cardiomyopathy.

Ann Clin Lab Sci 2018 Nov;48(6):785-789

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea

Mucolipidosis III alpha/beta (ML III alpha/beta) is an autosomal recessive lysosomal storage disorder caused by N-acetylglucosamine-1-phosphotransferase (GlcNAc-phosphotransferase) deficiency. It is characterized by coarse facial features, developmental delay, short stature, and skeletal deformities. Its cardiovascular symptoms include valvular thickening or hypertrophic cardiomyopathy. Read More

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November 2018
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Incidence, Clinical Characteristics, and Long-term Outcome of the Dilated Phase of Hypertrophic Cardiomyopathy.

Keio J Med 2018 Dec 29. Epub 2018 Dec 29.

Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.

Some patients with hypertrophic cardiomyopathy (HCM) develop systolic dysfunction, called the dilated phase of HCM (d-HCM), which is associated with increased morbidity and mortality. We conducted a retrospective study using an HCM database to clarify the incidence, clinical characteristics, and long-term outcomes of d-HCM. We analyzed an HCM cohort consisting of 434 patients (273 with apical HCM and 161 with non-apical HCM; 18 had obstructive HCM, 16 had dilated HCM, and 127 had other HCM) diagnosed by echocardiography in our hospital between 1991 and 2010. Read More

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http://dx.doi.org/10.2302/kjm.2018-0004-OADOI Listing
December 2018

Holter monitoring demonstrates that ventricular arrhythmias are common in cats with decompensated and compensated hypertrophic cardiomyopathy.

Vet J 2019 Jan 12;243:21-25. Epub 2018 Nov 12.

Division of Cardiology, Clinic for Small Animal Internal Medicine, Vetsuisse Faculty University of Zürich, Winterthurerstrasse 260, CH-8057 Zürich, Switzerland. Electronic address:

Arrhythmias can complicate cardiac disease in cats and are a potential cause of sudden death. The aim of this study was to evaluate the presence and nature of cardiac arrhythmias, and the potential correlation between plasma serum troponin I (cTnI) concentrations and the presence or severity of arrhythmias in cats with decompensated (dHCM) and compensated hypertrophic cardiomyopathy (cHCM). Forty one client-owned cats were studied: 16 with cHCM, 15 with dHCM and 10 healthy control cats. Read More

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http://dx.doi.org/10.1016/j.tvjl.2018.11.005DOI Listing
January 2019
1 Read

Modes of death and clinical outcomes in adult patients with hypertrophic cardiomyopathy in Thailand.

BMC Cardiovasc Disord 2019 Jan 3;19(1). Epub 2019 Jan 3.

Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Rama IV Road, Bangkok, 10330, Thailand.

Background: There are limited data about modes of death and major adverse cardiovascular events (MACEs) in patients with hypertrophic cardiomyopathy (HCM) in South East Asian population. The aim of the study was to examine modes of death and clinical outcomes in Thai patients with HCM.

Methods: Between January 1, 2009 and December 31, 2013, 166 consecutive patients with HCM diagnosed in our institution were evaluated. Read More

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http://dx.doi.org/10.1186/s12872-018-0984-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6318850PMC
January 2019
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Surgical Treatment for Hypertrophic Obstructive Cardiomyopathy with Concomitant Mitral Valve Abnormalities: A Cohort of 26 Cases.

Heart Surg Forum 2018 Nov 7;21(6):E443-E447. Epub 2018 Nov 7.

Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

Background: We sought to analyze the pathological characteristics of hypertrophic obstructive cardiomyopathy (HOCM) with concomitant mitral valve abnormalities and to discuss the surgical treatment strategies.

Methods: The clinical data of 26 HOCM patients treated from January 2014 to March 2016 were retrospectively analyzed. There were 19 males and 7 females with a mean age of 47 ± 16 years (range, 10-70 years). Read More

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http://dx.doi.org/10.1532/hsf.2013DOI Listing
November 2018
1 Read

Fragmented QRS on electrocardiography as a predictor of myocardial scar in patients with hypertrophic cardiomyopathy.

Acta Cardiol 2019 Jan 3:1-5. Epub 2019 Jan 3.

a Department of Cardiology , Amrita Institute of Medical Sciences and Research Centre (AIMS) , Kochi , India.

Background: Fragmented QRS (fQRS) and Q waves are ECG findings in patients with myocardial scar. fQRS is more sensitive than pathological Q waves in detecting myocardial fibrosis in patients with coronary artery disease (CAD). Cardiac magnetic resonance (CMR) imaging is used for the diagnosis and for quantifying scar tissue in patients with HCM. Read More

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http://dx.doi.org/10.1080/00015385.2018.1547355DOI Listing
January 2019

Hypertrophic Cardiomyopathy in Patients with Atrial Fibrillation: Prevalence and Associated Stroke Risks in a Nationwide Cohort Study.

Thromb Haemost 2019 Jan 2. Epub 2019 Jan 2.

Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Severance Cardiovascular Hospital, Yonsei University, Seoul, Republic of Korea.

Background:  There have been no prior nationwide reports on the prevalence of hypertrophic cardiomyopathy (HCM) among patients suffering from atrial fibrillation (AF). It is also unclear how much stroke risk is attributable to HCM compared with other stroke risks in patients with AF. This study assessed the prevalence of HCM among non-valvular AF (NVAF) patients and to assess the magnitude of increase in stroke risk in NVAF patients with HCM, compared with those without HCM. Read More

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http://dx.doi.org/10.1055/s-0038-1676818DOI Listing
January 2019

Impaired left ventricular mechanics and functional reserve are associated with reduced exercise capacity in patients with hypertrophic cardiomyopathy.

Echocardiography 2019 Jan 1. Epub 2019 Jan 1.

Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing, China.

Background: Reduced metabolic equivalents (METs) are an indicator of exercise intolerance, which predicts poor prognosis in hypertrophic cardiomyopathy (HCM) patients. We sought to evaluate the changes in left ventricular (LV) mechanics and functional reserves, as well as their association with functional capacity in HCM patients.

Methods: Seventy HCM patients and thirty controls were included in this study. Read More

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http://doi.wiley.com/10.1111/echo.14241
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http://dx.doi.org/10.1111/echo.14241DOI Listing
January 2019
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Novel phenotype-genotype correlations of hypertrophic cardiomyopathy caused by mutation in α-actin and myosin-binding protein genes in three unrelated Chinese families.

J Cardiol 2018 Dec 29. Epub 2018 Dec 29.

Department of Ultrasound, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi, China. Electronic address:

Background: The correlations between genotype and phenotype in hypertrophic cardiomyopathy (HCM) have not been established. Mutation of α-actin gene (ACTC1) is a rare cause of HCM. This study aimed to explore novel genotype-phenotype correlations in HCM patients with the variants in ACTC1 and myosin-binding protein (MYBPC3) genes in three unrelated Chinese families. Read More

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http://dx.doi.org/10.1016/j.jjcc.2018.09.005DOI Listing
December 2018

Growth hormone therapy in patients with Noonan syndrome.

Ann Pediatr Endocrinol Metab 2018 Dec 31;23(4):176-181. Epub 2018 Dec 31.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Noonan syndrome (NS) is an autosomal dominant disorder that involves multiple organ systems, with short stature as the most common presentation (>70%). Possible mechanisms of short stature in NS include growth hormone (GH) deficiency, neurosecretory dysfunction, and GH resistance. Accordingly, GH therapy has been carried out for NS patients over the last three decades, and multiple studies have reported acceleration of growth velocity (GV) and increase of height standard deviation score (SDS) in both prepubertal and pubertal NS patients upon GH therapy. Read More

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http://dx.doi.org/10.6065/apem.2018.23.4.176DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312920PMC
December 2018

Accuracy of Jet Direction on Doppler Echocardiography in Identifying the Etiology of Mitral Regurgitation in Obstructive Hypertrophic Cardiomyopathy.

J Am Soc Echocardiogr 2018 Dec 28. Epub 2018 Dec 28.

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

Background: Mitral valve regurgitation (MR) mediated by systolic anterior motion (SAM) in obstructive hypertrophic cardiomyopathy (HCM) is traditionally characterized by a posteriorly directed jet on Doppler echocardiography. Many believe that MR in the absence of a posteriorly directed jet signals the presence of intrinsic mitral valve (MV) disease.

Methods: A total of 709 adult patients with obstructive HCM who underwent septal myectomy were evaluated; 330 of these patients had >2 + MR preoperatively and constituted the study group. Read More

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http://dx.doi.org/10.1016/j.echo.2018.10.011DOI Listing
December 2018

Skeletal alterations, developmental delay and new mutations in juvenile-onset Pompe disease.

Neuromuscul Disord 2018 Dec 4. Epub 2018 Dec 4.

Dept. Nursing, University of Valencia, Valencia, Spain. Electronic address:

Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase. In addition to the severe infantile form with cardiac involvement, late-onset variants can affect older children, adolescents (aged >1 year old) or adults. Patients with juvenile (a subgroup of late-onset type) Pompe disease typically do not have cardiac alterations e. Read More

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http://dx.doi.org/10.1016/j.nmd.2018.11.013DOI Listing
December 2018

Feasibility and Reliability of Nonexpert POCUS for Cardiovascular Preparticipation Screening of Varsity Athletes: The SHARP Protocol.

Can J Cardiol 2019 Jan 14;35(1):35-41. Epub 2018 Nov 14.

Department of Medicine, Queen's University, Kingston, Ontario, Canada. Electronic address:

Background: Point of care ultrasound (POCUS) is a potential adjunctive cardiovascular preparticipation screening modality for young competitive athletes. A novel cardiac POCUS screening protocol, Screening the Heart of the Athlete Research Program (SHARP), was developed for nonexpert examiners to assess common structural etiologies associated with sudden cardiac arrest/death (SCA/D).

Methods: Assessment of primary outcomes of feasibility, and reliability of obtained measurements, performed by comparison to formal transthoracic echocardiogram was undertaken. Read More

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http://dx.doi.org/10.1016/j.cjca.2018.11.003DOI Listing
January 2019

Lysosomal storage disorders affecting the heart: a review.

Cardiovasc Pathol 2018 Dec 1;39:12-24. Epub 2018 Dec 1.

Department of Pathology and Laboratory Medicine, Ottawa Hospital and Faculty of Medicine, University of Ottawa, 451 Smyth Road, Ottawa, Ontario, K1H 8M5, Canada.

Lysosomal storage disorders (LSD) comprise a group of diseases caused by a deficiency of lysosomal enzymes, membrane transporters or other proteins involved in lysosomal biology. Lysosomal storage disorders result from an accumulation of specific substrates, due to the inability to break them down. The diseases are classified according to the type of material that is accumulated; for example, lipid storage disorders, mucopolysaccharidoses and glycoproteinoses. Read More

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http://dx.doi.org/10.1016/j.carpath.2018.11.002DOI Listing
December 2018

Clinical diversity of MYH7-related cardiomyopathies: Insights into genotype-phenotype correlations.

Am J Med Genet A 2018 Dec 27. Epub 2018 Dec 27.

The Genetics Institute, Rambam Health Care Campus, Haifa, Israel.

MYH7-related disease (MRD) is the most common hereditary primary cardiomyopathy (CM), with pathogenic MYH7 variants accounting for approximately 40% of familial hypertrophic CMs. MRDs may also present as skeletal myopathies, with or without CM. Since pathogenic MYH7 variants result in highly variable clinical phenotypes, from mild to fatal forms of cardiac and skeletal myopathies, genotype-phenotype correlations are not always apparent, and translation of the genetic findings to clinical practice can be complicated. Read More

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http://dx.doi.org/10.1002/ajmg.a.61017DOI Listing
December 2018

Sideroblastic anemia associated with multisystem mitochondrial disorders.

Pediatr Blood Cancer 2018 Dec 26:e27591. Epub 2018 Dec 26.

Department of Paediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.

Background: Sideroblastic anemia represents a heterogeneous group of inherited or acquired diseases with disrupted erythroblast iron utilization, ineffective erythropoiesis, and variable systemic iron overload. In a cohort of 421 patients with multisystem mitochondrial diseases, refractory anemia was found in 8 children.

Results: Five children had sideroblastic anemia with increased numbers of ring sideroblasts >15%. Read More

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http://doi.wiley.com/10.1002/pbc.27591
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http://dx.doi.org/10.1002/pbc.27591DOI Listing
December 2018
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Chronic Calmodulin-Kinase II Activation Drives Disease Progression in Mutation-Specific Hypertrophic Cardiomyopathy.

Circulation 2018 Nov 29. Epub 2018 Nov 29.

Dept. of Medicine/Cellular and Molecular Med, University of Arizona, UNITED STATES.

Background: Although the genetic causes of Hypertrophic Cardiomyopathy (HCM) are widely recognized, considerable lag in the development of targeted therapeutics has limited interventions to symptom palliation. This is in part due to an incomplete understanding of how point mutations trigger pathogenic remodeling. As a further complication, similar mutations within sarcomeric genes can result in differential disease severity, highlighting the need to understand the mechanism of progression at the molecular level. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.034549DOI Listing
November 2018

A Premature Termination Codon Mutation in MYBPC3 Causes Hypertrophic Cardiomyopathy via Chronic Activation of Nonsense-Mediated Decay.

Circulation 2018 Oct 2. Epub 2018 Oct 2.

Stanford Cardiovascular Institute, Stanford University School of Medicine, UNITED STATES.

Background: Hypertrophic cardiomyopathy (HCM) is frequently caused by mutations in myosin binding protein C3 ( MYBPC3) resulting in a premature termination codon (PTC). The underlying mechanisms of how PTC mutations in MYBPC3 lead to the onset and progression of HCM are poorly understood. This study's aim was to investigate the molecular mechanisms underlying the pathogenesis of HCM associated with MYBPC3 PTC mutations by utilizing human isogenic induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs). Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.034624DOI Listing
October 2018

Aborted sudden cardiac death in a young soldier with concomitant hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome.

Cardiol Young 2018 Dec 26:1-4. Epub 2018 Dec 26.

2Division of Cardiology, Department of Internal Medicine,The Armed Forces Capital Hospital,Seongnam,Republic of Korea.

We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Read More

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http://dx.doi.org/10.1017/S1047951118002123DOI Listing
December 2018
1 Read

[Evaluation of strain indexes and prognosis of patients with cardiac amyloidosis with preserved LVEF by three-dimensional speckle tracking imaging].

Zhonghua Yi Xue Za Zhi 2018 Dec;98(47):3842-3847

Department of Cardiology, the First Affiliated Hospital of Zhejiang University, College of Medicine, Hangzhou 310003, China.

To evaluate the patients with Cardiac amyloidosis (CA) and Hypertrophic cardiomyopathy (HCM) by using the strain indexes of three-dimensional speckle tracking imaging(3D-STI) technique, and to evaluate the prognosis. A total of 32 patients with pathologically confirmed cardiac amyloidosis and 34 patients with hypertrophic cardiomyopathy and 16 normal controls were enrolled from the First Affiliated Hospital of Zhejiang University College of Medicine from June, 2013 to January, 2018.The color Doppler echocardiography and three-dimensional speckle tracking echocardiography were used to measure strain indexes of each group, and the Logistic regression equation was used to analyze the index differences. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2018.47.006DOI Listing
December 2018

Combined mechanical mitral valve replacement and transmitral myectomy for hypertrophic obstructive cardiomyopathy treatment: An experience of over 20 years.

J Cardiol 2018 Dec 21. Epub 2018 Dec 21.

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.

Background: Although transaortic septal myectomy (TASM) is recognized as a standard procedure for treating hypertrophic obstructive cardiomyopathy (HOCM), occasionally the left ventricle (LV) intracavitary gradient remains postoperatively because of this technically demanding procedure. Mitral valve replacement (MVR) is sometimes chosen as an alternative option, but data on its long-term outcomes are lacking.

Methods And Results: Between 1991 and 2016, 29 patients [age, 14-82 (mean 58. Read More

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http://dx.doi.org/10.1016/j.jjcc.2018.12.012DOI Listing
December 2018

Apical Ballooning and Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy.

CASE (Phila) 2018 Dec 22;2(6):243. Epub 2018 Sep 22.

Department of Cardiovascular Surgery, Mount Sinai St. Luke's, New York, New York.

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http://dx.doi.org/10.1016/j.case.2018.07.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302028PMC
December 2018
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Papillary muscles of left ventricle-Morphological variations and it's clinical relevance.

Indian Heart J 2018 Nov - Dec;70(6):894-900. Epub 2017 Dec 11.

Department of Anatomy, Institute of Post Graduation Medical Education & Research, 244, A.J.C Bose Road, Kolkata-700020, West Bengal, India. Electronic address:

Introduction: The two left ventricular papillary muscles are small structures at sternocostal and inferior wall but are vital to mitral valve competence. Extra papillary muscles could be found. Partial or complete rupture, complicating acute myocardial infarction, causes severe or even catastrophic mitral regurgitation, potentially correctable by surgery. Read More

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http://dx.doi.org/10.1016/j.ihj.2017.12.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306352PMC
January 2019
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Impact of disease state on arrhythmic event detection by action potential modelling in cardiac safety pharmacology.

J Pharmacol Toxicol Methods 2018 Dec 21;96:15-26. Epub 2018 Dec 21.

Nova Research Laboratories LLC, 1441 Canal Street, New Orleans, LA 70112, USA. Electronic address:

Introduction: The use of in silico cardiac action potential simulations is one of the pillars of the CiPA initiative (Comprehensive in vitro Proarrhythmia Assay) currently under evaluation designed to detect more accurately proarrhythmic liabilities of new drug candidate. In order to take into account the variability of clinical situations, we propose to improve this method by studying the impact of various disease states on arrhythmic events induced by 30 torsadogenic or non-torsadogenic compounds.

Method: In silico modelling was done on the human myocytes using the Dutta revised O'Hara-Rudy algorithm. Read More

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http://dx.doi.org/10.1016/j.vascn.2018.12.004DOI Listing
December 2018