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Cardiovasc Res 2021 Jun 21. Epub 2021 Jun 21.

INSERM UMRS1166, ICAN-Institute of CardioMetabolism and Nutrition, Sorbonne University, Institute of Cardiology, Pitié-Salpêtrière Hospital, Paris, France.

Recent developments in imaging, mapping and ablation techniques have shown that the epicardial region of the heart is a key player in the occurrence of ventricular arrhythmic events in several cardiac diseases such as Brugada syndrome, arrhythmogenic cardiomyopathy or dilated cardiomyopathy. At the atrial level as well, the epicardial region has emerged as an important determinant of the substrate of atrial fibrillation, pointing to common underlying pathophysiological mechanisms. Alteration in the gradient of repolarization between myocardial layers favoring the occurrence of re-entry circuits has largely been described. Read More

Heart Rhythm 2021 Jun 17. Epub 2021 Jun 17.

Pediatric Cardiology and Cardiac Arrhythmias Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; European Reference Network for Rare and Low Prevalence Complex Disease of the Heart (ERN GUARD-Heart). Electronic address:

Background: Brugada syndrome (BrS) is an inheritable disease with an increased risk of sudden cardiac death. Although several score systems have been proposed, the management of children with BrS has been inconsistently described.

Objective: To identify the characteristics, outcome and risk factors associated with cardiovascular and arrhythmic events in children < 12 years with BrS. Read More

J Cardiothorac Vasc Anesth 2021 May 11. Epub 2021 May 11.

Department of Anesthesiology, Pain Medicine, and Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA.

The recently released American Heart Association (AHA) scientific statement on drug-induced arrhythmias discussed medications commonly associated with bradycardia, supraventricular tachycardias, and ventricular arrhythmias. The foundational data for this statement were collected from general outpatient and inpatient populations. Patients undergoing surgical and minimally invasive treatments are a unique subgroup, because they may experience hemodynamic changes associated with anesthesia and their procedure, receive multiple drug combinations not given in either inpatient or outpatient settings, or experience postprocedural inflammatory syndromes. Read More

Cureus 2021 May 16;13(5):e15066. Epub 2021 May 16.

Interventional Cardiology, Unity Health, Searcy, USA.

Brugada syndrome is a congenital cardiac channelopathy characterized by ST-segment elevation (≥2 mm) and subsequent inverted T wave in a minimum of two right precordial leads (Brugada type 1 ECG [electrocardiogram] pattern) on ECG. Brugada syndrome is estimated to be responsible for 4%-12% of all sudden cardiac deaths and up to 20% in patients with structurally normal hearts. Development of a temporary Brugada pattern, known as Brugada phenocopy, has been observed in individuals presenting with reversible underlying conditions such as hyperkalemia, hyponatremia, acidosis, ischemia, and pulmonary embolism, among others. Read More

J Arrhythm 2021 Jun 8;37(3):481-534. Epub 2021 Apr 8.

The First Affiliated Hospital of Nanjing Medical University Nanjing China.

This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members. Read More

J Emerg Med 2021 Jun 11. Epub 2021 Jun 11.

Department of Emergency Medicine, Denver Health and Hospital Authority, Denver, Colorado; Rocky Mountain Poison and Drug Safety, Denver Health and Hospital Authority, Denver, Colorado.

Background: Loperamide, commonly sold under the brand name Imodium® (Johnson & Johnson, Fort Washington, PA), is a widely available, over-the-counter antidiarrheal medication that possesses µ-opioid agonist properties and can have catastrophic cardiac events when misused or abused. Since the start of the opioid epidemic in the United States, there has been an increasing number of case reports and deaths linking loperamide abuse with cardiac events such as torsades de pointes (TdP) and Brugada syndrome.

Case Report: This case report presents a 22-year-old man who presented in cardiac arrest from polymorphic ventricular tachycardia consistent with TdP and a Type 1 Brugada pattern after intentional loperamide abuse. Read More

Europace 2021 Jun 14. Epub 2021 Jun 14.

Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam UMC, University of Amsterdam, Cardiovascular Sciences, Meibergdreef 9, Amsterdam, The Netherlands.

Aims: In patients with Brugada syndrome (BrS) but without spontaneous Type-1 electrocardiogram, several electrocardiographic characteristics have been studied, including the β-angle. Previous studies suggested that the β-angle might be useful in distinguishing BrS-patients from patients with only suggestive repolarization patterns without performing sodium channel blocker provocation testing. In this study, we aimed to determine the diagnostic value of the β-angle in patients suspected of BrS. Read More

Front Physiol 2021 28;12:661413. Epub 2021 May 28.

INSERM, UMR_S 1166 ICAN, Paris, France.

Loss-of-function mutations in the cardiac Na channel α-subunit Na1.5, encoded by , cause Brugada syndrome (BrS), a hereditary disease characterized by sudden cardiac death due to ventricular fibrillation. We previously evidenced the dominant-negative effect of the BrS Na1. Read More

J Cardiovasc Electrophysiol 2021 Jun 13. Epub 2021 Jun 13.

Heart Rhythm O2 2020 Dec 28;1(5):326-335. Epub 2020 Oct 28.

Academic Medical Center, Amsterdam, Netherlands.

Background: The subcutaneous implantable cardioverter-defibrillator (S-ICD) is an alternative to conventional transvenous ICD (TV-ICD) therapy to reduce lead complications.

Objective: To evaluate outcomes in channelopathy vs patients with structural heart disease in the EFFORTLESS-SICD Registry and with a previously reported TV-ICD meta-analysis in channnelopathies.

Methods: The EFFORTLESS registry includes 199 patients with channelopathies (Brugada syndrome 83, long QT syndrome 24, idiopathic ventricular fibrillation 78, others 14) and 786 patients with structural heart disease. Read More

BMJ Case Rep 2021 Jun 9;14(6). Epub 2021 Jun 9.

Department of Cardiology, CHUV, Lausanne, Switzerland.

Lithium is frequently used in the treatment of bipolar disorders and is known to induce ECG alterations. This case study describes various patterns of lithium-induced ECG modifications in a patient with acute-on-chronic lithium intoxication. Clinicians should be familiar with this problem as it can have life-threatening consequences and lead to important changes in patient's management. Read More

Pacing Clin Electrophysiol 2021 Jun 7. Epub 2021 Jun 7.

Elettrofisiologia, U.O.C di Cardiologia, Ospedale Civile Treviso, Treviso, Italy.

Three-dimensional (3D) reconstruction by means of electroanatomic mapping (EAM) systems, allows for the understanding of the mechanism of focal or re-entrant arrhythmic circuits, which can be identified by means of dynamic (activation and propagation) and static (voltage) color-coded maps. However, besides this conventional use, EAM may offer helpful anatomical and functional information for tissue characterisation in several clinical settings. Today, data regarding electromechanical myocardial viability, scar detection in ischaemic and nonischaemic cardiomyopathy and arrhythmogenic right ventricle dysplasia (ARVC/D) definition are mostly consolidated, while emerging results are becoming available in contexts such as Brugada syndrome and cardiac resynchronisation therapy (CRT) implant procedures. Read More

Circ Genom Precis Med 2021 Jun 7;14(3):e003229. Epub 2021 Jun 7.

Department of Medicine, Center of Excellence in Arrhythmia Research Chulalongkorn University (N.C., S.P., A.K., J.M., B.S., P.W., K.N.), Chulalongkorn University, Bangkok, Thailand.

J Biochem 2021 Jun 4. Epub 2021 Jun 4.

Division of Molecular Medical Biochemistry, Department of Biochemistry and Molecular Biology, Shiga University of Medical Science.

Transmembrane protein 168 (TMEM168) was found to be localized on the nuclear membrane. A heterozygous mutation (c.1616G>A, p. Read More

J Electrocardiol 2021 May 12;67:52-54. Epub 2021 May 12.

University of Szeged, Deparment of Anesthesiology and Intensive Care, Semmelweis utca 6, 6720 Szeged, Hungary. Electronic address:

We present a case of a patient who suffered subarachnoid haemorrhage (SAH), complicated by takotsubo syndrome, paroxysmal atrial fibrillation and ECG repolarisation abnormality, compatible with Brugada phenocopy. The early repolarisation morphology showed a paradox association with the cardiac cycle length; a relationship not yet documented in SAH. Our observation also sheds light on the genesis of the "spiked helmet" ECG sign. Read More

Hellenic J Cardiol 2021 May 31. Epub 2021 May 31.

First Department of Medicine, University Medical Centre Mannheim, Medical Faculty Mannheim, University of Heidelberg, Germany; DZHK (German Centre for Cardiovascular Research) partner site Mannheim, Germany.

Background: This study evaluated the prognostic significance of cardiac magnetic resonance myocardial feature tracking (CMR-FT) in patients with Brugada syndrome (BrS) to detect subclinical alterations and predict major adverse events (MAE).

Methods: CMR was performed in 106 patients with BrS and 25 healthy controls. Biventricular global strain analysis was assessed using CMR-FT. Read More

Diagnostics (Basel) 2021 May 17;11(5). Epub 2021 May 17.

Section of Legal Medicine, Department of Health Surveillance and Bioethics, Catholic University of the Sacred Heart, 00168 Rome, Italy.

Sudden death (SD) is defined as the unexpected natural death occurred within an hour after the onset of symptoms or from the last moment the subject has been seen in a healthy condition. Brugada syndrome (BrS) is one of the most remarkable cardiac causes of SD among young people. We report the case of a 20-year-old man who suddenly died after reportedly having smoked cannabis. Read More

J Cardiovasc Med (Hagerstown) 2021 May 28. Epub 2021 May 28.

Division of Cardiology, Magna Graecia University, Catanzaro, Italy Mediterranea Cardiocentro, Naples, Italy.

Life Sci 2021 Aug 26;278:119646. Epub 2021 May 26.

Laboratory of CardioBiology, Department of Biophysics, Federal University of São Paulo, Brazil. Electronic address:

Aims: SCN5A gene encodes the α-subunit of Na1.5, mainly found in the human heart. SCN5A variants are the most common genetic alterations associated with Brugada syndrome (BrS). Read More

Ann Clin Transl Neurol 2021 May 28. Epub 2021 May 28.

IRCCS Istituto Giannina Gaslini, Genova, Italy.

The influence of the central nervous system and autonomic system on cardiac activity is being intensively studied, as it contributes to the high rate of cardiologic comorbidities observed in people with epilepsy. Indeed, neuroanatomic connections between the brain and the heart provide links that allow cardiac arrhythmias to occur in response to brain activation, have been shown to produce arrhythmia both experimentally and clinically. Moreover, seizures may induce a variety of transient cardiac effects, which include changes in heart rate, heart rate variability, arrhythmias, asystole, and other ECG abnormalities, and can trigger the development of Takotsubo syndrome. Read More

Front Cardiovasc Med 2021 11;8:688875. Epub 2021 May 11.

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

Hum Hered 2021 May 26:1-9. Epub 2021 May 26.

Department of Biochemistry and Molecular Biology, Jiamusi University School of Basic Medical Sciences, Jiamusi, China.

Background: Voltage-gated sodium channels are protein complexes composed of 2 subunits, namely, pore-forming α- and regulatory β-subunits. A β-subunit consists of 5 proteins encoded by 4 genes (i.e. Read More

Glob Cardiol Sci Pract 2021 Apr 30;2021(1):e202104. Epub 2021 Apr 30.

Hospital Ramon y Cajal, Madrid, Spain.

Brugada syndrome is an autosomal dominant arrhythmogenic disease associated with an increased risk of ventricular fibrillation and sudden cardiac death. The mainstay of treatment in high-risk patients is an implantable cardioverter-defibrillator (ICD), however radiofrequency ablation has been proposed over the past decade as an additional therapy in patients with recurrent ICD firing. We report a case of Brugada syndrome with an electrical storm which was successfully managed by radiofrequency ablation. Read More

J Innov Card Rhythm Manag 2021 May 15;12(5):4520-4524. Epub 2021 May 15.

Cardiac Arrhythmia Unit, Heart Center, Massachusetts General Hospital, Boston, MA, USA.

We report a case of catheter ablation of Brugada syndrome in a patient with refractory ventricular fibrillation despite quinidine therapy. We performed epicardial substrate mapping, which identified an area of abnormal fractionated, prolonged electrogram in the anterior right ventricular outflow tract. Warm saline infusion into the pericardial space induced further delay of the local electrogram, consistent with Brugada syndrome physiology. Read More

Am J Cardiol 2021 May 22. Epub 2021 May 22.

Department of Cardiology, Ege University School of Medicine, Izmir, Turkey. Electronic address:

Irritable bowel syndrome (IBS) is one of the most widely recognized functional bowel disorders (FBDs) with a genetic component. SCN5A gene and SCN1B loci have been identified in population-based IBS cohorts and proposed to have a mechanistic role in the pathophysiology of IBS. These same genes have been associated with Brugada syndrome (BrS). Read More

HeartRhythm Case Rep 2021 May 2;7(5):273-277. Epub 2021 Feb 2.

IRCCS Ospedale Sacro Cuore Don Calabria, Negrar di Valpolicella, Italy.

Proc Natl Acad Sci U S A 2021 May;118(21)

Department of Biomedical Engineering, Johns Hopkins University, Baltimore, MD 21218;

In cardiomyocytes, Na1.5 channels mediate initiation and fast propagation of action potentials. The Ca-binding protein calmodulin (CaM) serves as a de facto subunit of Na1. Read More

JACC Clin Electrophysiol 2021 05;7(5):673-675

J Am Coll Cardiol 2021 May;77(20):2573-2591

Victor Chang Cardiac Research Institute, Darlinghurst, New South Wales, Australia; St. Vincent's Clinical School, University of New South Wales, Sydney, Kensington, New South Wales, Australia; Cardiology Department, St Vincent's Hospital, Sydney, New South Wales, Australia; Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, New York, New York, USA. Electronic address:

In the initial 3 papers in this Focus Seminar series, the fundamentals and key concepts of precision medicine were reviewed, followed by a focus on precision medicine in the context of vascular disease and cardiomyopathy. For the remaining 2 papers, we focus on precision medicine in the context of arrhythmias. Specifically, in this fourth paper we focus on long QT syndrome, Brugada syndrome, and atrial fibrillation. Read More

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211014060

Saint Michael's Medical Center, Newark, NJ, USA.

Brugada syndrome is a rare arrhythmogenic syndrome that is associated with an increased risk of ventricular fibrillation and sudden cardiac death. Electrocardiographic findings include patterns similar to a right bundle branch block (RBBB) and persistent ST-segment elevation in precordial leads (V1 and V2). There are numerous reports of Brugada syndrome mimicking ST-segment elevation myocardial infraction (STEMI); however, we describe a case of 47-year-old male who presented with STEMI mimics Brugada syndrome with preexisting RBBB. Read More