22,683 results match your criteria Cardiomyopathy Hypertrophic

Clinical characteristics and survival of children with hypertrophic cardiomyopathy in China: A multicentre retrospective cohort study.

EClinicalMedicine 2022 Jul 27;49:101466. Epub 2022 May 27.

Department of Cardiology, Shanghai Children's Medical Centre, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.

Background: Few data on paediatric hypertrophic cardiomyopathy (HCM) are available in developing countries. A multicentre, retrospective, cohort study was conducted to profile the clinical characteristics and survival of children with HCM in China.

Methods: We collected longitudinal data on children with HCM aged 0-18 years at three participating institutions between January 1, 2010 and December 31, 2019. Read More

View Article and Full-Text PDF

Apical Hypertrophic Cardiomyopathy Prompting Aneurysm, Thrombus, and Cardiac Arrest in a 56-Year-Old Female.

Cureus 2022 Jun 18;14(6):e26067. Epub 2022 Jun 18.

Cardiology, Lincoln Medical Center, New York City, USA.

Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic cardiac disease while apical hypertrophic cardiomyopathy (apHCM) is a rare subset of HCM. The significance of this case report is to present apHCM, its chronological course, and its association with left ventricular aneurysm, thrombosis, and cardiac arrest. We present the case of a 56-year-old female with a past medical history of apHCM who was admitted for substernal chest pain, developed a ventricular storm (VT), and subsequently suffered cardiac arrest; resuscitation of spontaneous circulation (ROSC) was eventually achieved after 10 minutes. Read More

View Article and Full-Text PDF

Hypertrophic cardiomyopathy and exercise restrictions: time to let the shackles off?

Br J Cardiol 2020 20;27(2):11. Epub 2020 May 20.

Consultant Cardiologist Institute of Cardiovascular Medicine and Science, Liverpool Heart and Chest Hospital, Thomas Drive, Liverpool, L14 3PE.

The health benefits of physical activity are well documented. Patients with hypertrophic cardiomyopathy (HCM) are often discouraged from participating in physical activity due to a perceived increase in the risk of sudden cardiac death (SCD). As a result, only 45% of patients with HCM meet the minimum guidelines for physical activity, and many report an intentional reduction in exercise following diagnosis. Read More

View Article and Full-Text PDF

Dynamical Behavior of Disordered Regions in Disease-Related Proteins Revealed by Quasielastic Neutron Scattering.

Satoru Fujiwara

Medicina (Kaunas) 2022 Jun 13;58(6). Epub 2022 Jun 13.

Institute for Quantum Biology, National Institutes for Quantum Science and Technology, 2-4 Shirakata, Tokai, Ibaraki 319-1106, Japan.

: Intrinsically disordered proteins (IDPs) and proteins containing intrinsically disordered regions (IDRs) are known to be involved in various human diseases. Since the IDPs/IDRs are fluctuating between many structural substrates, the dynamical behavior of the disease-related IDPs/IDRs needs to be characterized to elucidate the mechanisms of the pathogenesis of the diseases. As protein motions have a hierarchy ranging from local side-chain motions, through segmental motions of loops or disordered regions, to diffusive motions of entire molecules, segmental motions, as well as local motions, need to be characterized. Read More

View Article and Full-Text PDF

Association of the Genetic Variation in the Long Non-Coding RNA FENDRR with the Risk of Developing Hypertrophic Cardiomyopathy.

Life (Basel) 2022 May 30;12(6). Epub 2022 May 30.

Hospital Universitario Central de Asturias (HUCA), 33011 Oviedo, Spain.

In around 40-60% of Hypertrophic Cardiomyopathy (HCM) cases pathogenic variants are not identified. Our aim was to evaluate the possible association of lncRNAs with the risk of developing HCM. We sequenced 10 lncRNAs coding genes that have been associated with cardiovascular disease in a discovery cohort (238 HCM patients and 212 controls) by NGS, and genotyped rs74035787 G>A and rs1424019 A>G polymorphism in a validation cohort (962 HCM patients and 923 controls). Read More

View Article and Full-Text PDF

Review of Contemporary Invasive Treatment Approaches and Critical Appraisal of Guidelines on Hypertrophic Obstructive Cardiomyopathy: State-of-the-Art Review.

J Clin Med 2022 Jun 14;11(12). Epub 2022 Jun 14.

Department for the Treatment and Study of Cardiothoracic Diseases and Cardiothoracic Transplantation, IRCCS-ISMETT, 90127 Palermo, Italy.

Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a heterogeneous disease with different clinical presentations, albeit producing similar dismal long-term outcomes if left untreated. Several approaches are available for the treatment of HOCM; e.g. Read More

View Article and Full-Text PDF

Myogenic Determination and Differentiation of Chicken Bone Marrow-Derived Mesenchymal Stem Cells under Different Inductive Agents.

Animals (Basel) 2022 Jun 13;12(12). Epub 2022 Jun 13.

State Key Laboratory for Conservation and Utilization of Subtropical Agro-Bioresources, Lingnan Guangdong Laboratory of Agriculture, College of Animal Science, South China Agricultural University, Guangzhou 510642, China.

Poultry plays an important role in the meat consumer market and is significant to further understanding the potential mechanism of muscle development in the broiler. Bone marrow-derived mesenchymal stem cells (BM-MSCs) can provide critical insight into muscle development due to their multi-lineage differentiation potential. To our knowledge, chicken BM-MSCs demonstrate limited myogenic differentiation potential under the treatment with dexamethasone (DXMS) and hydrocortisone (HC). Read More

View Article and Full-Text PDF

Explaining unexplained syncope in hypertrophic cardiomyopathy: A clinical dilemma.

Int J Cardiol 2022 Jun 20. Epub 2022 Jun 20.

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, United States of America.

View Article and Full-Text PDF

Efficacy and Safety of a Combined Aerobic, Strength and Flexibility Exercise Training Program in Patients with Implantable Cardiac Devices.

J Cardiovasc Dev Dis 2022 Jun 6;9(6). Epub 2022 Jun 6.

Institute of Sports Medicine, Sport e Salute, 00197 Rome, Italy.

: The "FIDE Project" (Fitness Implantable DEvice) was organized by the Institute of Sports Medicine and Science and the World Society of Arrhythmias with the aim of demonstrating the usefulness of exercise training in improving functional capacity in patients with implantable cardiac devices. : Thirty sedentary patients were selected for the project (25 males and 5 females), with a mean age of 73 ± 5 years (range 44-94 years). Twenty-five were implanted with a Pacemaker (PM) and five with an Implantable Cardioverter Defibrillator (ICD). Read More

View Article and Full-Text PDF

Contemporary Diagnosis and Management of Hypertrophic Cardiomyopathy: The Role of Echocardiography and Multimodality Imaging.

J Cardiovasc Dev Dis 2022 May 25;9(6). Epub 2022 May 25.

National Cerebral and Cardiovascular Center, Department of Cardiovascular Medicine, Suita 564-8565, Japan.

Hypertrophic cardiomyopathy (HCM) is an underdiagnosed genetic heart disease with an estimated prevalence of 0.2-0.5%. Read More

View Article and Full-Text PDF

Single-nucleus profiling of human dilated and hypertrophic cardiomyopathy.

Nature 2022 Jun 22. Epub 2022 Jun 22.

Precision Cardiology Laboratory and the Cardiovascular Disease Initiative, The Broad Institute, Cambridge, MA, USA.

Heart failure encompasses a heterogeneous set of clinical features that converge on impaired cardiac contractile function and presents a growing public health concern. Previous work has highlighted changes in both transcription and protein expression in failing hearts, but may overlook molecular changes in less prevalent cell types. Here we identify extensive molecular alterations in failing hearts at single-cell resolution by performing single-nucleus RNA sequencing of nearly 600,000 nuclei in left ventricle samples from 11 hearts with dilated cardiomyopathy and 15 hearts with hypertrophic cardiomyopathy as well as 16 non-failing hearts. Read More

View Article and Full-Text PDF

Prevalence and Clinical Outcomes of Transthyretin Amyloidosis: A Systematic Review and Meta-analysis.

Eur J Heart Fail 2022 Jun 22. Epub 2022 Jun 22.

1st Cardiology Department, National and Kapodistrian University of Athens, Hippokration University Hospital, Athens, Greece.

Background: Systematic evidence on the prevalence and clinical outcome of transthyretin amyloidosis (ATTR) is missing. We explored: a) the prevalence of cardiac amyloidosis in various patient subgroups, b) survival estimates for ATTR subtypes and c) the effects of novel therapeutics on the natural course of disease.

Methods: A systematic review of literature published in Medline before 31/12/2021 was performed for the prevalence of cardiac amyloidosis & all-cause mortality of ATTR patients. Read More

View Article and Full-Text PDF

Response by Liang and Shimada to Letter Regarding Article "Comprehensive Proteomics Profiling Reveals Circulating Biomarkers of Hypertrophic Cardiomyopathy".

Circ Heart Fail 2022 Jun 20;15(6):e009568. Epub 2022 Apr 20.

Division of Cardiology, Department of Medicine, Columbia University Irving Medical Center, New York.

View Article and Full-Text PDF

Implantable defibrillators in primary prevention of genetic arrhythmias. A shocking choice?

Eur Heart J 2022 Jun 21. Epub 2022 Jun 21.

Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy.

Many previously unexplained life-threatening ventricular arrhythmias and sudden cardiac deaths (SCDs) in young individuals are now recognized to be genetic in nature and are ascribed to a growing number of distinct inherited arrhythmogenic diseases. These include hypertrophic cardiomyopathy, arrhythmogenic cardiomyopathy, long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia (VT), and short QT syndrome. Because of their lower frequency compared to coronary disease, risk factors for SCD are not very precise in patients with inherited arrhythmogenic diseases. Read More

View Article and Full-Text PDF

Image resampling and discretization effect on the estimate of myocardial radiomic features from T1 and T2 mapping in hypertrophic cardiomyopathy.

Sci Rep 2022 Jun 17;12(1):10186. Epub 2022 Jun 17.

Unit of Medical Physics, Pisa University Hospital "Azienda Ospedaliero-Universitaria Pisana", Via Roma 67, 56126, Pisa, Italy.

Radiomics is emerging as a promising and useful tool in cardiac magnetic resonance (CMR) imaging applications. Accordingly, the purpose of this study was to investigate, for the first time, the effect of image resampling/discretization and filtering on radiomic features estimation from quantitative CMR T1 and T2 mapping. Specifically, T1 and T2 maps of 26 patients with hypertrophic cardiomyopathy (HCM) were used to estimate 98 radiomic features for 7 different resampling voxel sizes (at fixed bin width), 9 different bin widths (at fixed resampling voxel size), and 7 different spatial filters (at fixed resampling voxel size/bin width). Read More

View Article and Full-Text PDF

Verification of the folkloric and anecdotal antidiabetic effects of Hypoxis hemerocallidea (Fisch., C.A. Mey. & Avé-Lall) and isolated, β-sitosterol using early-stage type II spontaneous diabetic mutant BKS-Lepr mice.

BMC Complement Med Ther 2022 Jun 20;22(1):163. Epub 2022 Jun 20.

Department of Paraclinical Sciences, Section of Pharmacology, Faculty of Veterinary Sciences, University of Pretoria, Pretoria, South Africa.

Background: Previous studies in our laboratory in ex vivo assays have demonstrated H. hemerocallidea extract as potential antidiabetic agent through increased insulin release from pancreatic beta cells. Thus, for this study the early stage type II spontaneous diabetic mutant mice model was used to evaluate and determine the degree of the antidiabetic efficacy of H. Read More

View Article and Full-Text PDF

Case Report: Treatment of Hypertrophic Cardiomyopathy With Stereotactic Body Radiotherapy.

Front Med (Lausanne) 2022 1;9:799310. Epub 2022 Jun 1.

Department of Oncology, Xiangya Hospital of Central South University, Changsha, China.

Patients with hypertrophic cardiomyopathy (HCM), which is characterized by left ventricular hypertrophy, is usually treated with medications such as calcium channel blockers or beta-blockers and invasive treatments such as transcatheter alcohol septal ablation, percutaneous radiofrequency ablation, or heart transplantation. However, non-invasive methods have not been employed for the management of patients with HCM. A 71-year-old male who presented with occasional chest pain for approximately 2 months and had been diagnosed with HCM since he was 39 years old due to occasional fainting was treated with a novel method for HCM using stereotactic body radiotherapy (SBRT). Read More

View Article and Full-Text PDF

Longitudinal Psychometric Analysis of the Hypertrophic Cardiomyopathy Symptom Questionnaire (HCMSQ) Using Outcomes from the Phase III EXPLORER-HCM Trial.

Pharmacoecon Open 2022 Jun 20. Epub 2022 Jun 20.

MyoKardia, Inc., a wholly owned subsidiary of Bristol Myers Squibb, Brisbane, CA, USA.

Background: Hypertrophic cardiomyopathy (HCM) symptoms include shortness of breath (SOB), fatigue, chest pain, palpitations, dizziness, and fainting. The HCM Symptom Questionnaire (HCMSQ), the only patient-reported outcome instrument designed to specifically measure HCM symptoms, yields four domain scores (SOB, tiredness, cardiovascular symptoms, syncope) and a total score. We evaluated the longitudinal psychometric properties of the HCMSQ using baseline to week 30 data from the phase III EXPLORER-HCM trial (NCT03470545). Read More

View Article and Full-Text PDF

Prognostic factors in hypertrophic cardiomyopathy in children: An MRI based study.

Int J Cardiol 2022 Jun 16. Epub 2022 Jun 16.

MD, Unité médico-chirurgicale de cardiologie congénitale et pédiatrique, centre de référence des maladies cardiaques congénitales complexes - M3C, Hôpital universitaire Necker-Enfants Malades, Université de Paris, France and Azienda Ospedaliero Universitaria Meyer, Florence, Italy. Electronic address:

Background: Clinical and prognostic role of cardiac magnetic resonance (CMR) in adult population with hypertrophic cardiomyopathy (HCM) have been largely assessed. We sought to investigate the role of CMR for predicting cardiovascular events in children with HCM.

Methods: CMR was performed in 116 patients with HCM (37 sarcomeric mutations, 31 other mutations, mean age 10. Read More

View Article and Full-Text PDF

MicroRNA expression profiles in familial hypertrophic cardiomyopathy with myosin-binding protein C3 (MYBPC3) gene mutations.

BMC Cardiovasc Disord 2022 Jun 18;22(1):278. Epub 2022 Jun 18.

Shengli Clinical Medical College of Fujian Medical University, Fuzhou, 350001, China.

Familial hypertrophic cardiomyopathy (FHCM) is an autosomal dominant inherited disease caused by mutations in genes encoding cardiac sarcomere proteins. MicroRNAs (miRNAs) play an important role in the pathogenesis of FHCM. In the present study, we aimed to determine the miRNA profile in FHCM patients with myosin-binding protein C3 (MYBPC3) gene mutations. Read More

View Article and Full-Text PDF

Left atrioventricular coupling index in hypertrophic cardiomyopathy and risk of new-onset atrial fibrillation.

Int J Cardiol 2022 Jun 15. Epub 2022 Jun 15.

Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands; Hospital University Germans Trias i Pujol, Fundació Institut d'Investigació en Ciències de la Salut Germans Trias i Pujol, Badalona, Spain. Electronic address:

Backgrounds: This study aimed to investigate the association between left atrioventricular coupling index (LACI) and the occurrence of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM).

Methods: A total of 373 patients with HCM and no history of AF were evaluated by transthoracic echocardiography. LACI was defined by the ratio of left atrial (LA) end-diastolic volume divided by left ventricular (LV) end-diastolic volume. Read More

View Article and Full-Text PDF

Effect of Obesity on Left Ventricular Remodeling and Clinical Outcome in Chinese Patients With Hypertrophic Cardiomyopathy: Assessed by Cardiac MRI.

J Magn Reson Imaging 2022 Jun 17. Epub 2022 Jun 17.

Department of Radiology, Functional and Molecular Imaging Key Laboratory of Sichuan Province, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Background: Obesity is highly prevalent in patients with hypertrophic cardiomyopathy (HCM) and believed to influence its phenotype.

Purpose: To explore the effects of obesity on left ventricular (LV) remodeling and long-term clinical course in Chinese patients with HCM.

Study Type: Longitudinal. Read More

View Article and Full-Text PDF

Porcine Circovirus type 2 infected myocardial tissue transcriptome signature.

Gene 2022 Jun 15;836:146670. Epub 2022 Jun 15.

ICAR - National Research Centre on Pig, Rani, Guwahati, Assam 781131, India. Electronic address:

The goal of this study was to compare the global gene expression profile in cardiac tissues of pig infected with porcine circovirus 2 (PCV2) to that of healthy cells. Since PCV2 infection causes severe cardiovascular lesions, the myocardial tissue model was chosen for this study. In High-throughput transcriptome analysis, DESeq2 and CLC genomics workbench analyses revealed a total of 196 significantly differentially expressed genes (DEGs) (p-value < 0. Read More

View Article and Full-Text PDF

Exercise training during childhood and adolescence is associated with favorable diastolic function in hypertrophic cardiomyopathy.

Int J Cardiol 2022 Jun 14. Epub 2022 Jun 14.

Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Institute for Experimental Medical Research, Oslo University Hospital Ullevål and University of Oslo, Oslo, Norway.; K. G. Jebsen Center for Cardiac Research, University of Oslo, Oslo, Norway; Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Electronic address:

Background: Diastolic dysfunction is an important part of the clinical phenotype in hypertrophic cardiomyopathy (HCM). While exercise training is known to improve left ventricular (LV) diastolic function in normal hearts, the effects of exercise training during childhood and adolescence in carriers of HCM-associated genetic variants are unknown.

Methods: In a cross-sectional and retrospective study, we combined clinical and echocardiographic data with history of exercise training from childhood to time of examination in 187 participants with HCM or an HCM-causative genotype. Read More

View Article and Full-Text PDF

Berardinelli Seip Syndrome: A rare case report.

J Pak Med Assoc 2022 May;72(5):969-971

Department of Pediatric Medicine, The Children's Hospital and Institute of Child Health, Lahore, Pakistan.

Berardinelli Seip Congenital Lipodystrophy (BSCL) or Congenital Generalized Lipodystrophy (CGL) is one of the four subgroups of lipodystrophy syndrome which is characterized by varying degrees of loss of adipose mass in the body. It is an extremely rare autosomal recessive disorder and commonly reported clinical presentations include muscular hypertrophy, gigantism, hepatomegaly, impaired glucose tolerance, acanthosis nigricans, hypertriglyceridaemia, cardiomyopathy, intellectual impairment, bone cysts and phlebomegaly. We present a case of a 4. Read More

View Article and Full-Text PDF

Electrocardiographic and Echocardiographic Predictors of Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy.

Front Cardiovasc Med 2022 27;9:905128. Epub 2022 May 27.

Cardiology Department, University of Medicine and Pharmacy "Carol Davila"- Euroecolab, Bucharest, Romania.

Background: Patients with hypertrophic cardiomyopathy (HCM) have an increased prevalence of atrial fibrillation (AF) compared to the general population, and left atrium (LA) remodeling is strongly correlated with the risk of AF. This prospective, monocentric study aimed to assess the role of LA electrocardiographic and echocardiographic (structural and functional) parameters in predicting the risk for incident AF in patients with HCM.

Methods And Results: The study population consisted of 126 HCM patients in sinus rhythm (52. Read More

View Article and Full-Text PDF

Aerobic Exercise Inhibited P2X7 Purinergic Receptors to Improve Cardiac Remodeling in Mice With Type 2 Diabetes.

Front Physiol 2022 31;13:828020. Epub 2022 May 31.

Department of Physical Education, Wenzhou Medical University, Wenzhou, China.

Diabetic cardiomyopathy (DCM), the main complication of diabetes mellitus, presents as cardiac dysfunction by ventricular remodeling. In addition, the inhibition of P2X7 purinergic receptors (P2X7R) alleviates cardiac fibrosis and apoptosis in Type 1 diabetes. However, whether exercise training improves cardiac remodeling by regulating P2X7R remains unknown. Read More

View Article and Full-Text PDF

Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy.

Interv Cardiol Clin 2022 Jul;11(3):245-255

Center for Valve and Structural Heart Disease, Minneapolis Heart Institute at Abbott Northwestern Hospital, 920 East, 28th Street, Minneapolis, MN 55417, USA; Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 East, 28th Street, Minneapolis, MN 55417, USA.

Over the past several decades, alcohol septal ablation has become an established therapy for selected patients, in whom there is clinical improvement in symptoms as well as objective functional capacity. Patient selection is essential to success, with continued emphasis on the procedure being performed by experienced operators as part of a multidisciplinary team. In many patients, the outcomes of alcohol septal ablation are comparable to the standard of surgical myectomy. Read More

View Article and Full-Text PDF

Hypertrophic Cardiomyopathy and Exercise: Mutually Exclusive or Beneficial?

Clin Sports Med 2022 Jul;41(3):473-484

Department of Cardiovascular Medicine, Atlantic Health, Morristown Medical Center, Morristown, NJ 07960, USA; Sports Cardiology and Hypertrophic Cardiomyopathy. Electronic address:

Individuals with HCM have historically been held from participation in sports beyond mild-intensity exercise. Exercise improves functional capacity and indices of cardiac function even in those with HCM. Emerging data have demonstrated the safety of exercise in individuals with HCM. Read More

View Article and Full-Text PDF

Exercise Stress Testing in Athletes.

Clin Sports Med 2022 Jul;41(3):441-454

Sports Cardiology Center, Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address:

Exercise stress testing (EST) is indicated for diagnostic and prognostic purposes in the general population. In athletes, stress tests can also be useful to inform the risk of high-intensity training and competition, to assess athletic conditioning, and to refine training regimens. Many specific indications for EST are unique to athletes. Read More

View Article and Full-Text PDF