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Hellenic J Cardiol 2021 Jun 17. Epub 2021 Jun 17.

Clinic of Cardiomyopathies and Inherited Cardiac Diseases, First Cardiology Department, AHEPA University Hospital, Thessaloniki, Greece.

Background: The aim of our study was to assess the performance of the new American College of Cardiology (ACC)/American Heart Association (AHA) Guidelines, with respect to sudden cardiac death (SCD) prevention, in comparison with the established risk score of the European Society of Cardiology (HCM Risk-SCD), in a large Mediterranean cohort of HCM patients.

Methods: The clinical and imaging characteristics of 784 HCM patients (mean age at first evaluation 52±16 years, 67.2% males) were analyzed retrospectively. Read More

JACC Cardiovasc Imaging 2021 Jun 16. Epub 2021 Jun 16.

Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Objectives: The aim of this study was to define the variability of maximal wall thickness (MWT) measurements across modalities and predict its impact on care in patients with hypertrophic cardiomyopathy (HCM).

Background: Left ventricular MWT measured by echocardiography or cardiovascular magnetic resonance (CMR) contributes to the diagnosis of HCM, stratifies risk, and guides key decisions, including whether to place an implantable cardioverter-defibrillator (ICD).

Methods: A 20-center global network provided paired echocardiographic and CMR data sets from patients with HCM, from which 17 paired data sets of the highest quality were selected. Read More

Sci Rep 2021 Jun 14;11(1):12411. Epub 2021 Jun 14.

Department of Electrophysiology, Heart Center, University of Leipzig, Strümpellstr. 39, 04289, Leipzig, Germany.

Atrial fibrillation (AF) leads to remodeling characterized by changes in both size and shape of the left atrium (LA). Here we aimed to study the effect of hypertrophic cardiomyopathy (HCM) on the pattern of LA remodeling in AF-patients. HCM-patients (n = 23) undergoing AF ablation (2009-2012) were matched and compared with 125 Non-HCM patients from our prospective registry. Read More

Zhonghua Xin Xue Guan Bing Za Zhi 2021 Jun;49(6):593-600

Department of Cardiomyopathy, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.

To analyze the clinical and genetic characteristics of clinical subtypes of non-obstructive hypertrophic cardiomyopathy (HCM). It was a cohort study. Patients with non-obstructive HCM admitted to Fuwai Hospital, Chinese Academy of Medical Sciences, from January 1999 to April 2019 were enrolled. Read More

Cardiol Res Pract 2021 24;2021:9931136. Epub 2021 May 24.

Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.

Objective: In hypertrophic cardiomyopathy (HCM), myocardial fibrosis is routinely shown by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) imaging. We evaluated the efficacy of 2 novel contrast-free CMR methods, namely, diffusion-weighted imaging (DWI) and feature-tracking (FT) method, in detecting myocardial fibrosis.

Methods: This cross-sectional study was conducted on 26 patients with HCM. Read More

Radiographics 2021 Jun 11:200142. Epub 2021 Jun 11.

From the Departments of Radiology (M.D.G., R.D.M., S.D.K., R.M.B., J.D.M., D.W.G., E.A.R.) and Cardiology (J.M.R.), Madigan Army Medical Center, 9040 Jackson Ave, Tacoma, WA 98431; and the Uniformed Services University of the Health Sciences, Bethesda, Md (M.D.G., J.M.R., D.W.G., E.A.R.).

Transthoracic echocardiography (TTE) is the primary initial imaging modality in cardiac imaging. Advantages include portability, safety, availability, and ability to assess the morphology and physiology of the heart in a noninvasive manner. Because of this, many patients who undergo advanced imaging with CT or MRI will have undergone prior TTE, particularly when cardiac CT angiography or cardiac MRI is performed. Read More

Front Cardiovasc Med 2021 25;8:670734. Epub 2021 May 25.

Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain.

Distinguishing the etiology of left ventricular hypertrophy (LVH) is clinically relevant due to patient outcomes and management. Easily obtained, echocardiography-based myocardial deformation patterns may improve standard non-invasive phenotyping, however, the relationship between deformation phenotypes and etiology-related, microstructural cardiac remodeling has not been reported. Synchrotron radiation-based X-ray phase-contrast imaging (X-PCI) can provide high resolution, three-dimensional (3D) information on myocardial microstructure. Read More

Circulation 2021 Jun 9. Epub 2021 Jun 9.

Division of Cardiology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX.

Metabolic remodeling precedes most alterations during cardiac hypertrophic growth under hemodynamic stress. The elevation of glucose utilization has been recognized as a hallmark of metabolic remodeling. However, its role in cardiac hypertrophic growth and heart failure in response to pressure overload remains to be fully illustrated. Read More

Mayo Clin Proc 2021 06;96(6):1684-1686

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

J Cardiovasc Magn Reson 2021 Jun 3;23(1):67. Epub 2021 Jun 3.

Department of Magnetic Resonance Imaging, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, Xicheng District, Beijing, 100037, China.

Background: Patients who have unexplained giant T-wave inversions but do not meet criteria for hypertrophic cardiomyopathy (HCM) (left ventricular (LV) wall thickness < 1.5 cm) demonstrate LV apical morphological features that differ from healthy subjects. Currently, it remains unknown how the abnormal LV apical morphology in this patient population changes over time. Read More

Diagnostics (Basel) 2021 May 26;11(6). Epub 2021 May 26.

Klinik für Innere Medizin II, Universitätsklinikum Ulm, Albert-Einstein Allee 23, 89081 Ulm, Germany.

Right ventricular (RV) systolic function represents an important independent predictor of adverse outcomes in many cardiovascular (CV) diseases. However, conventional parameters of RV systolic function (tricuspid annular plane excursion (TAPSE), RV myocardial performance index (MPI), and fractional area change (FAC)) are not always able to detect subtle changes in RV function. New evidence indicates a significantly higher predictive value of RV longitudinal strain (LS) over conventional parameters. Read More

J Clin Med 2021 May 17;10(10). Epub 2021 May 17.

Laboratory of Cardiac Imaging and 3D Printing, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China.

Left ventricular hypertrophy (LVH) caused by cardiac variant Fabry disease (FD) is typically late-onset and may mimic LVH caused by abnormal loading conditions. We aimed to determine the prevalence of FD in a non-selective patient population of everyday practice presenting with LVH, including those with hypertension and valve disease. We measured plasma alpha-galactosidase A activity using dried blood spot tests in 499 (age = 66 ± 13 years; 336 men) Hong Kong Chinese patients with LVH defined as maximal LV septal/posterior wall thickness ≥13 mm on echocardiography. Read More

Medicina (Kaunas) 2021 May 7;57(5). Epub 2021 May 7.

Department of Clinical, Special and Dental Sciences, University Hospital "Ospedali Riuniti Umberto I-Lancisi-Salesi", 60126 Ancona, Italy.

Athlete's heart (AH) is the result of morphological and functional cardiac modifications due to long-lasting athletic training. Athletes can develop very marked structural myocardial changes, which may simulate or cover unknown cardiomyopathies. The differential diagnosis between AH and cardiomyopathy is necessary to prevent the risk of catastrophic events, such as sudden cardiac death, but it can be a challenging task. Read More

J Clin Med 2021 May 6;10(9). Epub 2021 May 6.

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy.

Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Read More

Front Cardiovasc Med 2021 13;8:647857. Epub 2021 May 13.

Cardiovascular Division, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, United States.

Development of advanced heart failure (HF) symptoms is the most common adverse pathway in hypertrophic cardiomyopathy (HCM) patients. Currently, there is a limited ability to identify HCM patients at risk of HF. In this study, we present a machine learning (ML)-based model to identify individual HCM patients who are at high risk of developing advanced HF symptoms. Read More

Neuromuscul Disord 2021 Apr 26. Epub 2021 Apr 26.

Department of Medical Chemistry and Biochemistry, Medical University Sofia, Sofia, Bulgaria; Genetic Medico-Diagnostic Laboratory "Genica", Sofia, Bulgaria.

Pathogenic variants in MYH7 cause a wide range of cardiac and skeletal muscle diseases with childhood or adult onset. These include dilated and/or hypertrophic cardiomyopathy, left ventricular non-compaction cardiomyopathy, congenital myopathies with multi-minicores and myofiber type disproportion, myosin storage myopathy, Laing distal myopathy and others (scapulo-peroneal or limb-girdle muscle forms). Here we report the results from molecular genetic analyses (NGS and Sanger sequencing) of 4 patients in two families with variable neuromuscular phenotypes with or without cardiac involvement. Read More

J Cardiovasc Magn Reson 2021 May 31;23(1):63. Epub 2021 May 31.

Berlin Ultrahigh Field Facility (B.U.F.F.), Max Delbrück Center for Molecular Medicine in the Helmholtz Association (MDC), Robert-Rössle Strasse 10, 13125, Berlin, Germany.

Background: Hypertrophic cardiomyopathy (HCM) related myocardial vascular remodelling may lead to the reduction of myocardial blood supply and a subsequent progressive loss of cardiac function. This process has been difficult to observe and thus their connection remains unclear. Here we used non-invasive myocardial blood flow sensitive CMR to show an impairment of resting myocardial perfusion in a mouse model of naturally occurring HCM. Read More

Clin Res Cardiol 2021 May 27. Epub 2021 May 27.

Service de Cardiologie-Hôpital Pontchaillou, Univ Rennes, CHU Rennes, Inserm, LTSI-UMR 1099, F-35000, Rennes, France.

Aims: Defining the risk of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) patients is an important clinical and prognostic challenge. The aim of this study is to determine HCM phenogroups with different risk of AF occurrence at 5 years.

Methods And Results: We applied retrospectively the Bayesian method, which can analyze a large number of variables, to differentiate phenogroups of patients with different risks of AF and prognoses across a French prospective on-going hospital-based registry of adult HCM patients (REMY). Read More

ESC Heart Fail 2021 May 27. Epub 2021 May 27.

Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, Toon, Japan.

Aims: Cardiac amyloidosis (CA) is an infiltrative myocardial disease that occasionally mimics hypertrophic cardiomyopathy (HCM). The aim of this study is to investigate the discriminatory ability of visual assessment of left atrial (LA) function between CA and HCM on echocardiography.

Methods And Results: In total, 93 patients with cardiac magnetic resonance imaging (CMR)-confirmed HCM and 34 with cardiac biopsy-confirmed CA were retrospectively assessed. Read More

Heart Lung Circ 2021 May 19. Epub 2021 May 19.

Division of Cardiology, Bluhm Cardiovascular Institute, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Background: Patients with hypertrophic cardiomyopathy (HCM) and an identified sarcomere mutation have worse outcomes than those without though the underlying mechanism is incompletely understood. The presence of replacement fibrosis measured by late gadolinium enhancement (LGE) and diffuse fibrosis measured by extracellular volume (ECV) using cardiac magnetic resonance imaging (CMR) are associated with ventricular arrhythmias and cardiac mortality. We aimed to associate these two forms of fibrosis with identified sarcomere mutations. Read More

Int J Cardiovasc Imaging 2021 May 21. Epub 2021 May 21.

Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Tübingen, Germany.

To provide clinically relevant criteria for differentiation between the athlete's heart and similar appearing hypertrophic (HCM), dilated (DCM), and arrhythmogenic right-ventricular cardiomyopathy (ARVC) in MRI. 40 top-level athletes were prospectively examined with cardiac MR (CMR) in two university centres and compared to retrospectively recruited patients diagnosed with HCM (n = 14), ARVC (n = 18), and DCM (n = 48). Analysed MR imaging parameters in the whole study cohort included morphology, functional parameters and late gadolinium enhancement (LGE). Read More

Cureus 2021 Apr 15;13(4):e14496. Epub 2021 Apr 15.

Division of Cardiovascular Medicine, University of Missouri, Columbia, USA.

Apical hypertrophic cardiomyopathy (ApHCM) is a clinical challenge when presenting with symptoms of angina plus shortness of breath. An appropriate diagnosis of concurrent coronary artery disease (CAD) is needed for proper diagnosis, risk stratification, and management. We present a case of a 64-year-old gentleman with a history of ApHCM and CAD with previous percutaneous intervention presenting with recurrent angina. Read More

J Med Case Rep 2021 May 18;15(1):269. Epub 2021 May 18.

Department of Anesthesiology and Intensive Care Medicine (CVK, CCM) Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Augustenburger Platz 1, 13357, Berlin, Germany.

Background: In cases of hypertrophic obstructive cardiomyopathy (HOCM), the systolic anterior motion of the mitral valve apparatus results in an obstruction of the left ventricular outflow tract (LVOT), which is known as the SAM [systolic anterior motion] phenomenon. Hypothetically, a pathological obstruction of the LVOT of a different etiology would result in a comparable hemodynamic instability, which would be refractory to inotrope therapy, and may be detectable through echocardiography.

Case Presentation: We observed a severely impaired left ventricular function due to a combination of a thrombotic LVOT obstruction and distinctive mitral regurgitation in a 56-year-old Caucasian, female patient after massive transfusion with aggressive procoagulant therapy. Read More

Heart Fail Rev 2021 May 15. Epub 2021 May 15.

Cardiomyopathy & Neuromuscular Disease Unit, Third Cardiology Department, Hippokrateion University Hospital, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Hypertrophic cardiomyopathy (HCM) represents one of the primary cardiomyopathies and may lead to heart failure and sudden cardiac death. Among various histologic features of the disease examined, assessment of myocardial fibrosis may offer valuable information, since it may be considered the common nominator for all HCM connected complications. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) has emerged as the reference noninvasive method for visualizing and quantifying myocardial fibrosis in patients with HCM. Read More

ASAIO J 2021 May 12. Epub 2021 May 12.

From the Department of Cardiac Surgery, Medical University of Vienna, Vienna, Austria Division of Cardiovascular and Interventional Radiology, Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria Department of Internal Medicine 3, University Hospital St. Pölten Division of Cardiac Thoracic Vascular Anaesthesia and Intensive Care Medicine, Department of Anaesthesia, Intensive Care Medicine and Pain Medicine, Medical University of Vienna, Vienna, Austria.

Fixed pulmonary hypertension (FPH) is a contraindication for heart transplantation (HTX). However, this condition might be reversed by continuous left-ventricular unloading with a left-ventricular assist device. We present a case of apical hypertrophic cardiomyopathy with extensive left-ventricular endocardial calcification and severe FPH (systolic pulmonary artery pressure, 102 mm Hg). Read More

J Cardiovasc Magn Reson 2021 May 13;23(1):54. Epub 2021 May 13.

Division of Cardiology, Department of Cardiovascular Diseases, Cliniques Universitaires St. Luc UCL, Av Hippocrate 10/2806, 1200, Woluwe St. Lambert, Belgium.

Background: Cardiovascular magnetic resonance (CMR) 2D feature tracking (FT) left ventricular (LV) myocardial strain has seen widespread use to characterize myocardial deformation. Yet, validation of CMR FT measurements remains scarce, particularly for regional strain. Therefore, we aimed to perform intervendor comparison of 3 different FT software against tagging. Read More

Cardiovasc Diagn Ther 2021 Apr;11(2):373-382

Sydney Medical School, Faculty of Medicine and Health, The University of Sydney, Camperdown, NSW, Australia.

Background: Post-mortem cardiac magnetic resonance (CMR) is a non-invasive alternative to conventional autopsy. At present, diagnostic guidelines for cardiovascular conditions such as hypertrophic cardiomyopathy have not been established. We correlated post-mortem CMR images to definite conventional autopsy findings and hypothesed that elevated T2-weighted signal intensity and RV to LV area ratios can identify myocardial infarction and pulmonary emboli respectively. Read More

Eur J Clin Invest 2021 May 4:e13593. Epub 2021 May 4.

Liverpool Centre for Cardiovascular Science, University of Liverpool and Liverpool Heart & Chest Hospital, Liverpool, UK.

Introduction: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac condition and the most common cause of sudden cardiac death (SCD) in patients below the age of 35. Genetic testing is a vital part of HCM diagnostics, yet correlation with clinical phenotypes remains complex. Identifying clinical predictors of informative genetic testing may prevent unnecessary investigations and improve cost-effectiveness of services. Read More

Circ Cardiovasc Imaging 2021 May 4;14(5):e012656. Epub 2021 May 4.

Division of Cardiology, Department of Medicine (J.D.M., R.G.B., S.C.M., S.C.), Washington University, St Louis, MO.