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J Anal Toxicol 2021 May 15. Epub 2021 May 15.

Institut de médecine légale, 11 rue Humann, 67000 Strasbourg, France.

A 59-year old male was found dead at home, with 2 empty vials of an oily preparation attributed to a manufacturer from East Europe. There was no label on the vial. The subject was a former weightlifter, also known as an anabolic steroids abuser. 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

Proc Natl Acad Sci U S A 2021 May;118(20)

Department of Biomedical Engineering, Washington University, St. Louis, MO 63130;

Cardiac arrhythmias are the most common cause of sudden cardiac death worldwide. Lengthening the ventricular action potential duration (APD), either congenitally or via pathologic or pharmacologic means, predisposes to a life-threatening ventricular arrhythmia, Torsade de Pointes. I (KCNQ1+KCNE1), a slowly activating K current, plays a role in action potential repolarization. Read More

Heart 2021 May 14. Epub 2021 May 14.

Cardiology, UMC Utrecht, Utrecht, The Netherlands

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by fibrofatty replacement of predominantly the right ventricle and high risk of ventricular arrhythmias and sudden cardiac death (SCD). Early diagnosis and accurate risk assessment are challenging yet essential for SCD prevention. This manuscript summarises the current state of the art on ARVC diagnosis and risk stratification. Read More

CMAJ Open 2021 Apr-Jun;9(2):E500-E509. Epub 2021 May 14.

Centre for Clinical Epidemiology (Moriello, Reynier, Aibibula, Filion), Lady Davis Institute, Jewish General Hospital, McGill University, Montréal, Que; ICES (Paterson); Institute of Health Policy, Management and Evaluation (Paterson), University of Toronto, Toronto, Ont.; Manitoba Centre for Health Policy, Department of Community Health Sciences (Dahl, Kuo), Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Man.; Department of Anesthesiology, Pharmacology and Therapeutics (Fisher), University of British Columbia, Vancouver BC; School of Pharmacy (Gamble), University of Waterloo, Kitchener, Ont.; Department of Community Health Sciences (Ronksley), Cumming School of Medicine, University of Calgary, Calgary, Alta.; Saskatchewan Health Quality Council (Winquist); Department of Community Health and Epidemiology (Winquist), College of Medicine, University of Saskatchewan, Saskatoon Sask.; Departments of Medicine and of Epidemiology, Biostatistics, and Occupational Health (Filion), McGill University, Montréal, Que.

Background: Trends in off-label postpartum use of domperidone and the impact of safety advisories on its use remain unknown. Our objectives were to describe postpartum use of domperidone in Canada, to evaluate the impact of Health Canada advisories on prescribing patterns, and to describe the association between domperidone use and a composite end point of sudden cardiac death or ventricular tachycardia (VT) among postpartum patients.

Methods: We conducted a multidatabase cohort study involving pregnant patients with live births between 2004 and 2017 using administrative health databases from 5 Canadian provinces (British Columbia, Alberta, Saskatchewan, Manitoba and Ontario). Read More

Sci Rep 2021 May 13;11(1):10228. Epub 2021 May 13.

Center for Biomedical Engineering, School of Engineering, Brown University, Providence, RI, USA.

Cardiotoxicity of pharmaceutical drugs, industrial chemicals, and environmental toxicants can be severe, even life threatening, which necessitates a thorough evaluation of the human response to chemical compounds. Predicting risks for arrhythmia and sudden cardiac death accurately is critical for defining safety profiles. Currently available approaches have limitations including a focus on single select ion channels, the use of non-human species in vitro and in vivo, and limited direct physiological translation. Read More

J Am Coll Cardiol 2021 May;77(19):2363-2365

Electrophysiology Section, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon, USA.

J Am Coll Cardiol 2021 May;77(19):2353-2362

Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, University of California-San Francisco, San Francisco, California, USA. Electronic address:

Background: In the POST SCD study, the authors autopsied all World Health Organization (WHO)-defined sudden cardiac deaths (SCDs) and found that only 56% had an arrhythmic cause; resuscitated sudden cardiac arrests (SCAs) were excluded because they did not die suddenly. They hypothesized that causes underlying resuscitated SCAs would be similarly heterogeneous.

Objectives: The aim of this study was to determine the causes and outcomes of resuscitated SCAs. Read More

J Med Cases 2021 May 5;12(5):173-176. Epub 2021 Mar 5.

Hospital Santa Maria Maior, Barcelos, Portugal.

We report the case of a 41-year-old patient with no family history of sudden cardiac death. The patient presented with high fever and vomiting and was diagnosed with acute pyelonephritis. Screening for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection was positive. Read More

Circ Res 2021 May 13;128(10):1533-1553. Epub 2021 May 13.

Center for Cardiovascular Genetics, Institute of Molecular Medicine and Department of Medicine, University of Texas Health Sciences Center at Houston.

Hypertrophic cardiomyopathy (HCM) is a genetic disease of the myocardium characterized by a hypertrophic left ventricle with a preserved or increased ejection fraction. Cardiac hypertrophy is often asymmetrical, which is associated with left ventricular outflow tract obstruction. Myocyte hypertrophy, disarray, and myocardial fibrosis constitute the histological features of HCM. Read More

Epilepsia 2021 May 13. Epub 2021 May 13.

Department of Biological Sciences, Hunter College of City University of New York, New York, New York, USA.

Objective: Fibroblast growth factor homologous factors (FHFs) are brain and cardiac sodium channel-binding proteins that modulate channel density and inactivation gating. A recurrent de novo gain-of-function missense mutation in the FHF1(FGF12) gene (p.Arg52His) is associated with early infantile epileptic encephalopathy 47 (EIEE47; Online Mendelian Inheritance in Man database 617166). Read More

Front Physiol 2021 26;12:642083. Epub 2021 Apr 26.

Department of Medical Physiology, Universitair Medisch Centrum Utrecht, Utrecht, Netherlands.

Introduction: Torsade de pointes arrhythmias (TdP) in the chronic atrioventricular block (CAVB) dog model result from proarrhythmic factors, which trigger TdP and/or reinforce the arrhythmic substrate. This study investigated electrophysiological and arrhythmogenic consequences of severe bradycardia for TdP.

Methods: Dofetilide (25 μg/kg per 5 min) was administered to eight anesthetized, idioventricular rhythm (IVR) remodeled CAVB dogs in two serial experiments: once under 60 beats per minute (bpm), right ventricular apex paced (RVA60) conditions, once under more bradycardic IVR conditions. Read More

Circ J 2021 May 12. Epub 2021 May 12.

Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine.

Background: Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.Methods and Results:Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019. Read More

JAMA Cardiol 2021 May 12. Epub 2021 May 12.

Heart Failure and Heart Transplantation Unit, Virgen del Rocio University Hospital, Sevilla, Spain.

Importance: Truncating variants in the gene encoding filamin C (FLNCtv) are associated with arrhythmogenic and dilated cardiomyopathies with a reportedly high risk of ventricular arrhythmia.

Objective: To determine the frequency of and risk factors associated with adverse events among FLNCtv carriers compared with individuals carrying TTN truncating variants (TTNtv).

Design, Setting, And Participants: This cohort study recruited 167 consecutive FLNCtv carriers and a control cohort of 244 patients with TTNtv matched for left ventricular ejection fraction (LVEF) from 19 European cardiomyopathy referral units between 1990 and 2018. Read More

JAMA Cardiol 2021 May 12. Epub 2021 May 12.

Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Clin Chem Lab Med 2021 May 11. Epub 2021 May 11.

University Institute of Sports Medicine, Prevention and Rehabilitation and Research Institute of Molecular Sports Medicine and Rehabilitation, Paracelsus Medical University, Salzburg, Austria.

Objectives: Micro ribonucleic acids (miRNAs) are small non-coding RNA molecules that control gene expression by translational inhibition. Exercise has been shown to affect several miRNAs' expression in healthy subjects, but this has not yet been studied in patients with coronary artery disease (CAD). Since exercise training confers beneficial long-term effects and may also trigger acute coronary events, it is of utmost interest to be able to identify those who are risk for untoward effects. Read More

Integr Org Biol 2021 27;3(1):obaa047. Epub 2021 Jan 27.

School of Physics, Georgia Institute of Technology, Atlanta, GA, USA.

Effective cardiac contraction during each heartbeat relies on the coordination of an electrical wave of excitation propagating across the heart. Dynamically induced heterogeneous wave propagation may fracture and initiate reentry-based cardiac arrhythmias, during which fast-rotating electrical waves lead to repeated self-excitation that compromises cardiac function and potentially results in sudden cardiac death. Species which function effectively over a large range of heart temperatures must balance the many interacting, temperature-sensitive biochemical processes to maintain normal wave propagation at all temperatures. Read More

Cureus 2021 Apr 9;13(4):e14381. Epub 2021 Apr 9.

Emergency Medicine, Hamad Medical Corporation, Doha, QAT.

Brugada syndrome (BS) is a hereditary cardiac disease leading to sudden cardiac death. It does not display any structural cardiac abnormalities. It was first described in 1992, as the syndrome of 'right bundle branch block, persistent ST segment elevation, and sudden death. Read More

Acta Cardiol Sin 2021 May;37(3):296-304

Graduate Institute of Clinical Medicine, National Taiwan University Hospital and Medical College, National Taiwan University, Taiwan e Administration of National Health Insurance, Taipei, Taiwan.

Background: The epidemiology of pediatric potentially sudden death (SD) events and the rescue rate remain unclear.

Methods: We established a birth cohort (2000-2014) from a national database 2000-2015.

Results: Of 3,097,277 live births, we identified 3126 children (56. Read More

Ir J Med Sci 2021 May 10. Epub 2021 May 10.

Department of Cardiovascular Surgery, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training And Research Hospital, University Of Health Sciences, Istanbul, Turkey.

Background: The vast majority of sudden and unexpected natural deaths are related to cardiovascular diseases, especially coronary artery diseases.

Aims: In this study, we aimed to reveal the epidemiological differences between men and women and to investigate the most common pathologies that cause cardiac deaths.

Methods: Five thousand seven hundred sixty-eight autopsy cases that were done in 2016 were reviewed for the autopsy information and histopathological findings. Read More

Int J Legal Med 2021 May 11. Epub 2021 May 11.

Section of Forensic Genetics, Department of Forensic Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.

Cardiac diseases and sudden cardiac death (SCD) are more prevalent in individuals diagnosed with schizophrenia compared to the general population, with especially coronary artery disease (CAD) as the major cardiovascular cause of death. Antipsychotic medications, genetics, and lifestyle factors may contribute to the increased SCD in individuals with schizophrenia. The role of antipsychotic medications and lifestyle factors have been widely investigated, while the genetic predisposition to inherited cardiac diseases in schizophrenia is poorly understood. Read More

Ann Cardiol Angeiol (Paris) 2021 May 7. Epub 2021 May 7.

Centre d'Expertise Mort Subite de Paris (Paris-CEMS), Inserm U970, 56, rue Leblanc, 75015 Paris, France; Université de Paris, 85, boulevard Saint Germain, 75006 Paris, France; Service de cardiologie, Unité de rythmologie, Hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France. Electronic address:

Sudden cardiac death, mostly related to ventricular arrhythmia, is a major public health issue, with still very poor survival at hospital discharge. Although coronary artery disease remains the leading cause, other etiologies should be systematically investigated. Exhaustive and standardized exploration is required to eventually offer specific therapeutics and management to the patient as well as his/her family members in case of inherited cardiac disease. Read More

Trials 2021 May 10;22(1):336. Epub 2021 May 10.

The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, 510006, Guangdong Province, China.

Background: Cardiovascular disease is the leading cause of mortality and morbidity worldwide, Chronic stable angina (CSA) is the main symptom of myocardial ischemia, causes increased risk of major cardiovascular events such as sudden cardiac death and myocardial infarction. Naoxintong (NXT) capsule is a classical traditional Chinese medication used to treat CSA, however, few evidence to support the wide utility of NXT capsule for the treatment of CSA. We design this study to evaluate the efficacy and safety of NXT capsule versus placebo in patients with CSA. Read More

Resuscitation 2021 May 7. Epub 2021 May 7.

Paris Fire Brigade Medical Emergency Department, Paris, France.

Front Cardiovasc Med 2021 21;8:652027. Epub 2021 Apr 21.

Arrhythmology Department, IRCCS Policlinico San Donato, Milan, Italy.

The evolution of the current dogma surrounding Brugada syndrome (BrS) has led to a significant debate about the real usefulness of genetic testing in this syndrome. Since BrS is defined by a particular electrocardiogram (ECG) pattern, after ruling out certain possible causes, this disease has come to be defined more for what it is than for what it . Extensive research is required to understand the effects of specific individual variants, including modifiers, rather than necessarily grouping together, for example, "all variants" when trying to determine genotype-phenotype relationships, because not all variants within a particular gene act similarly. Read More

Cardiovasc Diagn Ther 2021 Apr;11(2):683-695

Department of Internal Medicine I, Fabry Center for Interdisciplinary Therapy (FAZIT) and Comprehensive Heart Failure Center (CHFC), University Hospital Würzburg, Würzburg, Germany.

Fabry disease (OMIM 301500) is an X-linked (Xq22.1) lysosomal storage disorder leading to a progressive multisystem disease with high variability in both genotype and phenotype expression. The pathophysiological origin is found in an enzyme deficiency of the α-galactosidase A (enzyme commission no. Read More

Cardiovasc Diagn Ther 2021 Apr;11(2):650-660

2nd Department of Internal Cardiovascular Medicine, General University Hospital, Prague, Czech Republic.

Fabry disease (FD) is an X-linked lysosomal storage disorder due to reduced or undetectable α-galactosidase A (AGAL-A) enzyme activity caused by pathogenic variants in the AGAL-A gene (). Tissue and organ changes are caused by widespread progressive accumulation of globotriaosylceramide (Gb) and globotriaosylsphingosine (lysoGb). The classical form of FD is multisystemic with cutaneous (angiokeratomas), neurological (peripheral neuropathy, premature stroke), renal (proteinuria and renal insufficiency), and cardiac involvement. Read More

Cardiovasc Diagn Ther 2021 Apr;11(2):637-649

Medical Genetics Center, Munich, Germany.

Background: Comprehensive genetic analysis yields in a higher diagnostic rate but also in a higher number of secondary findings (SF). American College of Medical Genetics and Genomics (ACMG) published a list of 59 actionable genes for which disease causing sequence variants are recommended to be reported as SF including 27 genes linked to inherited cardiovascular disease (CVD) such as arrhythmia syndromes, cardiomyopathies and vascular and connective tissue disorders. One of the selected conditions represented in the actionable gene list is the arrhythmogenic right ventricle cardiomyopathy (ARVC), an inherited heart muscle disease with a particularly high risk of sudden cardiac death (SCD). Read More

Cardiovasc Diagn Ther 2021 Apr;11(2):538-549

Department of Cardiology II - Electrophysiology, University Hospital Muenster, Muenster, Germany.

Fortunately, the population of adults with congenital heart disease (ACHD) is growing due to improved operation techniques. Life expectancy is continuously rising, nevertheless, sudden cardiac death is one of the leading causes of mortality in ACHD late after initial diagnosis. Risk stratification in ACHD remains challenging as large study results are missing, congenital defects and operation methods differ considerably between individual patients and results from acquired heart diseases are often not conferrable. Read More

Cureus 2021 Apr 5;13(4):e14305. Epub 2021 Apr 5.

Cardiology, Sanger Heart & Vascular Institute, Charlotte, USA.

Introduction: An underlying cardiomyopathy should be suspected in young patients presenting with ventricular arrhythmias and sudden cardiac arrest. Electrocardiograms revealing epsilon waves are associated with many serious conditions such as arrhythmogenic right ventricular cardiomyopathy, posterior myocardial infarction, right ventricular infarction, infiltration disease, sarcoidosis, Brugada Syndrome, Tetralogy of Fallot, and hypothermia. This case report features epsilon waves in a young cardiac arrest patient suspected of having an unrecognized cardiomyopathy that resulted in a fatal arrhythmia in the setting of exogenous bovine thyroid hormone and steroid use. Read More