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MedEdPORTAL 2020 May 29;16:10904. Epub 2020 May 29.

Professor of Cardiology, Department of Cardiology, Lehigh Valley Health Network.

Introduction: Physical examination skills are receiving less attention in curricula and clinical practice, being supplanted by imaging and other technologies. We developed an online module to introduce auscultation of common cardiac murmurs to second-year medical students.

Methods: The Murmur Online Learning Experience (MOLE) curriculum focused on nine common, unique, or highly testable cardiac murmurs, chosen collaboratively by the authors. Read More

J Card Surg 2020 Jul 11. Epub 2020 Jul 11.

Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK.

Objective: Affecting 1 in 500 individuals; hypertrophic cardiomyopathy is an autosomal dominant cardiovascular disorder which is prevalent throughout the world. Surgical myectomy (SM) and alcohol septal ablation (ASA) are two methods currently used for the management of drug refractory hypertrophic obstructive cardiomyopathy (HOCM). ASA may prove to be a useful, less invasive treatment in patients with HOCM METHODS: Electronic literature search was conducted to identify articles that discussed methods to treat drug refractory HOCM. Read More

J Am Coll Cardiol 2020 Jul;76(2):186-197

Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro, Madrid, Spain; Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart; Universidad Francisco de Vitoria, Pozuelo de Alarcon, Spain. Electronic address:

Background: PRKAG2 gene variants cause a syndrome characterized by cardiomyopathy, conduction disease, and ventricular pre-excitation. Only a small number of cases have been reported to date, and the natural history of the disease is poorly understood.

Objectives: The aim of this study was to describe phenotype and natural history of PRKAG2 variants in a large multicenter European cohort. Read More

BMC Med Imaging 2020 Jul 1;20(1):73. Epub 2020 Jul 1.

HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, PO Box 340, FI-00029, Helsinki, HUS, Finland.

Background: Left ventricle rotation and torsion are fundamental components of myocardial function, and several software packages have been developed for analysis of these components. The purpose of this study was to compare the suitability of two software packages with different technical principles for analysis of rotation and torsion of the left ventricle during systole.

Methods: A group of hypertrophic cardiomyopathy (HCM) patients (N = 14, age 43 ± 11 years), mutation carriers without hypertrophy (N = 10, age 34 ± 13 years), and healthy relatives (N = 12, age 43 ± 17 years) underwent a cardiovascular magnetic resonance examination, including spatial modulation of magnetization tagging sequences in basal and apical planes of the left ventricle. Read More

Circ Cardiovasc Imaging 2020 Jul 1;13(7):e010379. Epub 2020 Jul 1.

Department of Cardiovascular, Respiratory, Nephrologic, Anesthesiologic and Geriatric Sciences (A.F., M.A., C.C.), Sapienza University, Rome.

Int J Cardiovasc Imaging 2020 Jun 29. Epub 2020 Jun 29.

Department of Cardiovascular Ultrasound, The First Affiliated Hospital of China Medical University, 155 North Nanjing Street, Heping District, Shenyang, 110001, China.

We conducted this study to investigate left ventricle (LV) systolic function in endocardial, mid-myocardial, and epicardial layers by two-dimensional (2D) speckle tracking echocardiography (STE) in hypertrophic cardiomyopathy (HCM) patients with myocardial injury indexed by elevated serum cardiac troponin I (cTnI). Twenty-nine HCM patients with myocardial injury, thirty-five HCM patients without myocardial injury, and ninty-one healthy controls were enrolled in this study. Serum cTnI > 0. Read More

Eur Radiol 2020 Jun 25. Epub 2020 Jun 25.

PET-CT/MR Department, Harbin Medical University Cancer Hospital, Harbin, China.

Objectives: This study aimed to evaluate the feasibility and reproducibility of using cardiovascular magnetic resonance feature tracking (CMR-FT) for analysis of bi-ventricular strain and strain rate (SR) in hypertrophic cardiomyopathy (HCM) patients as well as to explore the correlation between right ventricular (RV) and left ventricular (LV) deformation.

Methods: A total of 60 HCM patients and 48 controls were studied. Global and segmental peak values of bi-ventricular longitudinal, circumferential, radial strain, and systolic SR were analyzed. Read More

Eur Heart J Cardiovasc Imaging 2020 Jun 26. Epub 2020 Jun 26.

Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2300 RC Leiden, The Netherlands.

Aims: Atrial fibrillation (AF) is frequently observed in hypertrophic cardiomyopathy (HCM) and is associated with poor clinical outcome. Total atrial conduction time, estimated by tissue Doppler imaging (TDI), the so-called PA-TDI duration, reflects the left atrial (LA) structural and electrical remodelling. The aim of this study was to evaluate the association between PA-TDI and new-onset AF in patients with HCM. Read More

Curr Probl Cardiol 2020 Jun 4:100641. Epub 2020 Jun 4.

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and the most common cause of sudden cardiac death in young population, especially in athletes under 35 years old. Mutations occur primarily in the β-myosin heavy chain gene and involve the cardiac myosin-binding protein C gene. In this review we would like to focus on the importance of the examination of mitral valve apparatus and the mitral valve abnormalities in patients with HCM. Read More

Cardiovasc Pathol 2020 May 19;49:107234. Epub 2020 May 19.

Centre for Heart Lung Innovation, St Paul's Hospital/University of British Columbia, Vancouver, BC, Canada; Departments of Pathology and Laboratory Medicine, Providence Health Care/University of British Columbia, Vancouver, BC, Canada.

Rev Port Cardiol 2020 Jun 18. Epub 2020 Jun 18.

Hospital da Luz - Inherited Cardiovascular Diseases & Hypertrophic Cardiomyopathy Center, Nova Medical School, Lisbon, Portugal.

Introduction And Objectives: Hypertrophic cardiomyopathy (HCM) is a genetically and phenotypically heterogeneous disease; there is still a large proportion of patients with no identified disease-causing mutation. Although the majority of mutations are found in the MYH7 and MYBPC3 genes, mutations in Z-disk-associated proteins have also been linked to HCM.

Methods: We assessed a small family with HCM based on family history, physical examination, 12-lead ECG, echocardiogram and magnetic resonance imaging. Read More

J Am Soc Echocardiogr 2020 Jun 18. Epub 2020 Jun 18.

Inherited Cardiac Diseases Unit and Cardiovascular Imaging Laboratory, Vall d'Hebron University Hospital, Barcelona, Spain.

Background: Left ventricular wall thickness (LVWT) measurement is key in the diagnostic and prognostic assessment of hypertrophic cardiomyopathy (HCM). Recent investigations have highlighted discrepancies in LVWT by cardiac magnetic resonance (CMR) and standard echocardiography (S-Echo) in this condition. The aim of this study was to elucidate the role of contrast-enhanced echocardiography (C-Echo) to optimize LVWT measurement in patients with HCM. Read More

Med Phys 2020 Jun 21. Epub 2020 Jun 21.

Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.

Purpose: Cardiac motion tracking enables quantitative evaluation of myocardial strain, which is clinically interesting in cardiovascular disease research. However, motion tracking is difficult to perform manually. In this paper, we aim to develop and compare two fully automated motion tracking methods for the steady state free precession (SSFP) cine magnetic resonance imaging (MRI), and explore their use in real clinical scenario with different patient groups. Read More

Heart Fail Rev 2020 Jun 20. Epub 2020 Jun 20.

Cardiovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), University of Trieste, Via Pietro Valdoni 7, 34149, Trieste, Italy.

Sudden cardiac death (SCD) is a pivotal health problem worldwide. The identification of subjects at increased risk of SCD is crucial for the accurate selection of candidates for implantable cardioverter defibrillator (ICD) therapy. Current strategies for arrhythmic stratification largely rely on left ventricular (LV) ejection fraction (EF), mostly measured by echocardiography, and New York Heart Association functional status for heart failure with reduced EF. Read More

J Magn Reson Imaging 2020 Jun 18. Epub 2020 Jun 18.

Biomedical Imaging Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, Leeds Teaching Hospitals NHS Trust, Leeds, UK.

Background: Hypertrophic cardiomyopathy (HCM) remains the commonest cause of sudden cardiac death among young athletes. Differentiating between physiologically adaptive left ventricular (LV) hypertrophy observed in athletes' hearts and pathological HCM remains challenging. By quantifying the diffusion of water molecules, diffusion tensor imaging (DTI) MRI allows voxelwise characterization of myocardial microstructure. Read More

BMC Cardiovasc Disord 2020 Jun 18;20(1):298. Epub 2020 Jun 18.

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: To investigate the relationship between fragmented QRS (fQRS) quantified by a new method and myocardial fibrosis (MF) and the diagnostic value of quantitative fQRS (Q-fQRS) to detect MF in hypertrophic obstructive cardiomyopathy (HOCM) patients based on histological validation.

Methods: We performed a retrospective study that included 69 patients with HOCM who underwent ventricular septal surgery. Nine individuals who died from accidents were studied as a control reference for the histological parameters. Read More

J Investig Med High Impact Case Rep 2020 Jan-Dec;8:2324709620934324

Mayo Clinic Arizona, Scottsdale, AZ, USA.

A 79-year-old man was admitted for a transcatheter aortic valve replacement due to severe aortic stenosis. A preoperative chest computed tomography with angiography revealed an apical variant hypertrophic cardiomyopathy with a prominent apical pouch. In addition, there was near-complete obliteration of the left ventricle in the mid to apical aspect during systole suggesting a midventricular gradient. Read More

Circ Cardiovasc Imaging 2020 Jun 16;13(6):e010489. Epub 2020 Jun 16.

Department of Cardiology, University of Messina, Messina, Italy (A.B., S.C., G.D.B.).

Background: Late gadolinium enhancement (LGE) is an important prognostic marker in hypertrophic cardiomyopathy and an extent >15% it is associated with high risk of sudden cardiac death. We proposed a novel method, the LGE-dispersion mapping, to assess heterogeneity of scar, and evaluated its prognostic role in patients with hypertrophic cardiomyopathy.

Methods: One hundred eighty-three patients with hypertrophic cardiomyopathy and a low- or intermediate 5-year risk of sudden cardiac death underwent cardiac magnetic resonance imaging. Read More

J Magn Reson Imaging 2020 Jun 11. Epub 2020 Jun 11.

Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China.

Background: The phenotype via conventional cardiac MRI analysis of MYH7 (β-myosin heavy chain)- and MYBPC3 (β-myosin-binding protein C)-associated hypertrophic cardiomyopathy (HCM) groups is similar. Few studies exist on the genotypic-phenotypic association as assessed by machine learning in HCM patients.

Purpose: To explore the phenotypic differences based on radiomics analysis of T mapping images between MYH7 and MYBPC3-associated HCM subgroups. Read More

Sci Rep 2020 Jun 8;10(1):9166. Epub 2020 Jun 8.

Division of Cardiology "Villa dei Fiori" Hospital, Acerra, Na, Italy.

Background: Mitral valve prolapse (MVP) is characterized by an abnormal movement of the valvular apparatus which may affect the papillary muscles (PMs) function and structure. Aim of the study was to investigate abnormal PM signal in MVP by using cardiac magnetic resonance imaging (MRI).

Methods And Results: We enrolled 47 consecutive patients with MVP evaluated by cardiac MRI. Read More

Echocardiography 2020 Jun 8. Epub 2020 Jun 8.

Heart Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijng, China.

Double-chambered left ventricle (DCLV) is a particularly rare congenital cardiovascular malformation that is difficult to diagnose. It is characterized by the subdivision of the left ventricle into two chambers by an abnormal septum or muscle band. Here, we report 12 patients with DCLV. Read More

J Cardiol 2020 Jun 2. Epub 2020 Jun 2.

Department of Cardiology, Clinical Emergency Hospital of Bucharest, Bucharest, Romania; University of Medicine and Pharmacy Carol Davila, Bucharest, Romania.

Background: Ventricular arrhythmias are the most frequent cause of sudden cardiac death in individuals with hypertrophic cardiomyopathy (HCM). In the present study we investigated if combined left ventricular (LV) and right ventricular (RV) mechanical dispersion (MD) are correlated with ventricular arrhythmias. We aimed also to analyze if MD enhances the arrhythmic risk stratification in HCM. Read More

Medicine (Baltimore) 2020 Jun;99(23):e20134

Department of Cardiology, West China Hospital, Sichuan University.

The phenomenon of high signal intensity on T2-weighted imaging of cardiac magnetic resonance in hypertrophic cardiomyopathy (HCM) has been previously studied. However, the underlying histopathologic mechanism remains unclear. Elevated cardiac troponin can be detected in some HCM patients. Read More

Clin Radiol 2020 Jun 1. Epub 2020 Jun 1.

Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Key Laboratory of Cardiovascular Imaging (Cultivation), Chinese Academy of Medical Sciences, Beijing 100037, China. Electronic address:

Aims: To investigate the clinical spectrum, cardiovascular magnetic resonance imaging (cMRI) characteristics, including T1 and extracellular volume fraction, and outcomes of Danon disease to facilitate further understanding of the phenotype of patients with Danon disease.

Materials And Methods: The study comprised six male patients 8-23 years old recruited to the study between 2014-2019. The clinical presentation, laboratory examinations, pathology/genetic analysis, electrocardiography (ECG), echocardiography, and cCMRI characteristics were summarised. Read More

J Clin Med 2020 Jun 1;9(6). Epub 2020 Jun 1.

Department of Cardiovascular Medicine, Chonnam National University Medical School/Hospital, Gwangju 61469, Korea.

Background: The spectrum of genetic variants and their clinical significance of Hypertrophic cardiomyopathy (HCM) have been poorly studied in Asian patients. The objectives of this study were to assess the spectrum of genetic variants and genotype-phenotype relationships within a Korean HCM population.

Methods: Eighty-nine consecutive unrelated HCM patients were included. Read More

Cureus 2020 Apr 26;12(4):e7839. Epub 2020 Apr 26.

Internal Medicine, Citrus Memorial Hospital, Inverness, USA.

Pacemaker lead-associated cardiac perforation is a rare phenomenon. Lead perforations can be acute, subacute, or chronic following lead placement. Symptoms are typically nonspecific and depend on the location of the displaced lead. Read More

J Inherit Metab Dis 2020 May 28. Epub 2020 May 28.

Department of Radiology and Nuclear Medicine, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.

Cardiomyopathy can be a severe complication in patients with long-chain fatty acid β-oxidation disorders (LCFAOD), particularly during episodes of metabolic derangement. It is unknown whether latent cardiac abnormalities exist in adult patients. To investigate cardiac involvement in LCFAOD, we used proton magnetic resonance imaging (MRI) and spectroscopy ( H-MRS) to quantify heart function, myocardial tissue characteristics, and myocardial lipid content in 14 adult patients (two with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD); four with carnitine palmitoyltransferase II deficiency (CPT2D); and eight with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD)) and 14 gender-, age-, and BMI-matched control subjects. Read More

J Cardiovasc Imaging 2020 Jul 27;28(3):230-233. Epub 2020 May 27.

Department of Cardiology, Sultan Abdulhamid Han Training and Research Hospital, Health Science University, Istanbul, Turkey.

Int J Cardiovasc Imaging 2020 May 27. Epub 2020 May 27.

University Hospital Leipzig Department for Internal Medicine Neurology and Dermatology, Department of Cardiology, University Hospital Leipzig, Leipzig, Sachsen, Germany.

Exclusion of cardiac abnormalities should be performed at the beginning of the athlete's career. Myocarditis, right ventricular remodeling and coronary anomalies are well-known causes of life-threatening events of athletes, major cardiovascular events and sudden cardiac death. The feasibility of an extended comprehensive echocardiographic protocol for the detection of structural cardiac abnormalities in athletes should be tested. Read More

Heart 2020 Jul 25;106(14):1111-1112. Epub 2020 May 25.

Heart and Vascular Institute, Cleveland Clinic Miller Family Heart and Vascular Institute, Cleveland, Ohio, USA

Cardiovasc Pathol 2020 Sep - Oct;48:107233. Epub 2020 May 7.

Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, Texas, USA; Center for Advanced Cardiopulmonary Therapies and Transplantation, McGovern Medical School and Memorial Hermann Hospital-Texas Medical Center, Houston, Texas, USA.

This paper collates the pathological findings from initial published autopsy reports on 23 patients with coronavirus disease 2019 (COVID-19) from 5 centers in the United States of America, including 3 cases from Houston, Texas. Findings confirm that COVID-19 is a systemic disease with major involvement of the lungs and heart. Acute COVID-19 pneumonia has features of a distinctive acute interstitial pneumonia with a diffuse alveolar damage component, coupled with microvascular involvement with intra- and extravascular fibrin deposition and intravascular trapping of neutrophils, and, frequently, with formation of microthombi in arterioles. Read More

Int J Mol Sci 2020 May 15;21(10). Epub 2020 May 15.

Spectroscopy and Imaging Division, Japan Synchrotron Radiation Research Institute, SPring-8 679-5198 Hyogo, Japan.

X-ray diffraction and tension measurement experiments were conducted on rat left ventricular skinned fibers with or without "troponin-T treatment," which exchanges the endogenous troponin T/I/C complex with exogenous troponin-T. These experiments were performed to observe the structural changes in troponin-T within a fiber elicited by contractile crossbridge formation and investigate the abnormality of hypertrophic cardiomyopathy-related troponin-T mutants. The intensity of the troponin reflection at 1/38. Read More

Cardiovasc Diagn Ther 2020 Apr;10(2):262-278

Cardiovascular Genetics Laboratory, Hatter Institute for Cardiovascular Research in Africa, Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.

In sub-Saharan Africa (SSA), the burden of noncommunicable diseases (NCDs) is rising disproportionately in comparison to the rest of the world, affecting urban, semi-urban and rural dwellers alike. NCDs are predicted to surpass infections like human immunodeficiency virus, tuberculosis and malaria as the leading cause of mortality in SSA over the next decade. Heart failure (HF) is the dominant form of cardiovascular disease (CVD), and a leading cause of NCD in SSA. Read More

J Thorac Cardiovasc Surg 2020 Apr 15. Epub 2020 Apr 15.

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

Objectives: To categorize and assess the functional significance of anomalous papillary muscles in patients undergoing surgical management of obstructive hypertrophic cardiomyopathy.

Methods: We reviewed the records of operations for obstructive hypertrophic cardiomyopathy and identified 73 patients with an anomalous papillary muscle. Anomalous papillary muscles inserting directly into the body of the anterior mitral valve leaflet were classified as type I, those with both direct insertion into the body of the leaflet and attachment to the free edge of the anterior leaflet were categorized as type II, and anomalous papillary muscles inserting into the free edge of the anterior leaflet were grouped as type III. Read More

J Cardiovasc Transl Res 2020 May 14. Epub 2020 May 14.

Instituto Cardiovascular, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares (CIBERCV), Barcelona, Spain.

Imaging techniques have allowed knowing the structural adaptative changes observed in the hearts of highly trained athletes. Athletes can develop very marked structural changes and the need may rise for a differential diagnosis with real cardiomyopathy. In this chapter, authors review the physiologic and morphologic features associated with athletic training and the keys to differentiate normal adaptive athlete's heart from mild or initial expression forms of left-heart side cardiomyopathies such as hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and left ventricle non-compaction (LVNC). Read More

Circ Cardiovasc Imaging 2020 May 15;13(5):e009901. Epub 2020 May 15.

Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital Sydney Street, London, United Kingdom (Z.K., P.F.F., A.D.S., S.N.-V., D.N.F., D.J.P.).

Background Cardiac amyloidosis (CA) is a disease of interstitial myocardial infiltration, usually by light chains or transthyretin. We used diffusion tensor cardiovascular magnetic resonance (DT-CMR) to noninvasively assess the effects of amyloid infiltration on the cardiac microstructure. Methods DT-CMR was performed at diastole and systole in 20 CA, 11 hypertrophic cardiomyopathy, and 10 control subjects with calculation of mean diffusivity, fractional anisotropy, and sheetlet orientation (secondary eigenvector angle). Read More

Int J Cardiovasc Imaging 2020 May 13. Epub 2020 May 13.

Cardiology Department, Hospital Universitario de la Princesa, IIS-IP, Universidad Autónoma de Madrid, Calle Diego de León, 62, 28006, Madrid, Spain.

Whether sex differences exist in cardiac magnetic resonance (CMR) findings in patients with hypertrophic cardiomyopathy (HCM) remain unknown. We sought to assess and compare CMR characteristics in male and female patients with HCM. From January-2006 to October-2017, 165 consecutive HCM patients evaluated with CMR were included. Read More

J Peripher Nerv Syst 2020 May 12. Epub 2020 May 12.

Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

V122I is one of more than 130 mutations in transthyretin gene associated with hereditary TTR (ATTRv) amyloidosis. Main clinical expression is an infiltrative pseudohypertrophic cardiomyopathy with mild or no neurological symptoms. It is particularly common among African-Americans (prevalence: 3%-4%). Read More

Kardiologiia 2020 Mar 18;60(4):137-145. Epub 2020 Mar 18.

Institute of Genetics and Cytology of Belarus National Academy of Sciences.

Aim To study the clinical course of the mixed phenotype (hypertrophic cardiomyopathy, HCMP, and left ventricular noncompaction, LVNC); to determine its genetic causes; and to evaluate incidence of cardiovascular complications (CVC) during the follow-up period.Material and methods In screening of 286 patients with HCMP, 8 of them (2.8 %; median age, 41. Read More

J Cardiovasc Magn Reson 2020 05 11;22(1):34. Epub 2020 May 11.

Department of Radiology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands.

Background: The clinical application of cardiovascular magnetic resonance (CMR) T and T mapping is currently limited as ranges for healthy and cardiac diseases are poorly defined. In this meta-analysis we aimed to determine the weighted mean of T and T mapping values in patients with myocardial infarction (MI), heart transplantation, non-ischemic cardiomyopathies (NICM) and hypertension, and the standardized mean difference (SMD) of each population with healthy controls. Additionally, the variation of mapping outcomes between studies was investigated. Read More

Front Physiol 2020 24;11:344. Epub 2020 Apr 24.

Department of Medicine, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States.

Background: Mechanisms of arrhythmogenicity in hypertrophic cardiomyopathy (HCM) are not well understood.

Objective: To characterize an electrophysiological substrate of HCM in comparison to ischemic cardiomyopathy (ICM), or healthy individuals.

Methods: We conducted a prospective case-control study. Read More

Cardiovasc Pathol 2020 Sep - Oct;48:107218. Epub 2020 Mar 7.

Hypertrophic Cardiomyopathy Program, Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, NY.

Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Read More

Kardiologiia 2020 Jan 20;60(3):44-50. Epub 2020 Jan 20.

Almazov National Medical Research Centre.

Objective To evaluate prospects for clinical use of circulating biomarkers for characterizing fibrotic changes in the myocardium of patients with hypertrophic cardiomyopathy (HCMP) with left ventricular (LV) outflow tract obstruction.Materials and Methods This was a prospective study with a 12-month follow-up period. The study included 47 patients (29 females and 18 males) with obstructive HCMP who were selected for septal reduction. Read More

Clin Res Cardiol 2020 May 5. Epub 2020 May 5.

Department of Cardiology I, University Hospital Münster, Albert-Schweitzer-Campus 1, Building A1, 48149, Münster, Germany.

Background: Coronary microvascular dysfunction (CMD) is present in various non-ischemic cardiomyopathies and in particular in those with left-ventricular hypertrophy. This study evaluated the diagnostic value of the novel cardiovascular magnetic resonance (CMR) parameter "myocardial transit-time" (MyoTT) in distinguishing cardiac amyloidosis from other hypertrophic cardiomyopathies.

Methods: N = 20 patients with biopsy-proven cardiac amyloidosis (CA), N = 20 patients with known hypertrophic cardiomyopathy (HCM), and N = 20 control patients without relevant cardiac disease underwent dedicated CMR studies on a 1. Read More

Zhonghua Xin Xue Guan Bing Za Zhi 2020 Apr;48(4):287-293

Department of Ultrasound, Xijing Hospital, Air Force Medical University, Xi'an 710032, China.

To evaluate the cardiac functional changes in hypertrophic cardiomyopathy(HCM) patients with β-myosin heavy chain gene (MYH7) mutations by three-dimensional (3D) speckle tracking imaging(3D-STI) and conventional echocardiography modalities, and then to explore the potential predictors of adverse cardiovascular events in these patients. A consecutive series of 192 HCM patients admitted in our center from October 2014 to October 2016 were genetically screened to identify MYH7 mutations in this retrospective study. A total of 43 HCM patients with MYH7 mutations were enrolled. Read More

J Cardiovasc Magn Reson 2020 05 4;22(1):30. Epub 2020 May 4.

Department of Radiology and Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.

Background: Hypertrophic cardiomyopathy (HCM) is thought to be associated with microvascular dysfunction. Adenosine stress-perfusion cardiovascular magnetic resonance imaging (CMR) is a sensitive method for assessing microvascular perfusion abnormalities. We evaluated the prevalence and clinical characteristics of HCM patients with adenosine-induced perfusion defects on CMR. Read More

Cardiovasc Res 2020 Jul;116(9):1585-1599

Department of Neurosciences, Psychiatry, Drug Research and Child Health (NeuroFarBa), University of Florence, Florence, Italy.

Hypertrophic cardiomyopathy (HCM) is a common inherited monogenic disease with a prevalence of 1/500 in the general population, representing an important cause of arrhythmic sudden cardiac death (SCD), heart failure, and atrial fibrillation in the young. HCM is a global condition, diagnosed in >50 countries and in all continents. HCM affects people of both sexes and various ethnic and racial origins, with similar clinical course and phenotypic expression. Read More

J Comput Assist Tomogr 2020 May/Jun;44(3):341-345

Cardiology.

Objective: The aim of this study was to evaluate the usefulness of texture analysis of T2-weighted short inversion time inversion recovery (T2-STIR) for detecting myocardial tissue alterations in hypertrophic cardiomyopathy (HCM).

Methods: Twenty patients with HCM and 11 controls were examined. Texture analysis was performed for the hypertrophied regions with and without and abnormal hyperintensity (AHI) and for the interventricular septum of the controls on T2-STIR. Read More

Ann Noninvasive Electrocardiol 2020 Apr 23:e12763. Epub 2020 Apr 23.

Heart and Vascular Center, Semmelweis University, Budapest, Hungary.

Background: Structural myocardial changes in hypertrophic cardiomyopathy (HCM) are associated with different abnormalities on electrocardiographs (ECGs). The diagnostic value of the ECG voltage criteria used to screen for left ventricular hypertrophy (LVH) may depend on the presence and degree of myocardial fibrosis. Fibrosis can cause other changes in ECG parameters, such as pathological Q waves, fragmented QRS (fQRS), or repolarization abnormalities. Read More

Int J Cardiovasc Imaging 2020 Apr 20. Epub 2020 Apr 20.

Department of Radiology, The First Affiliated Hospital of Nanjing Medical University, No. 300, Guangzhou Rd., Nanjing, China.

PRKAG2 syndrome (PS) is a rare autosomal dominant syndrome that mainly presents with hypertrophic cardiomyopathy, ventricular preexcitation, and conduction abnormalities. Few reports have demonstrated the morphologic manifestation of patients with PRKAG2 gene defect. This case report demonstrates the cardiac magnetic resonance characteristics of the PS patients from a Chinese family. Read More