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Clin Res Cardiol 2019 Mar 13. Epub 2019 Mar 13.

Department of Clinical Radiology, University Hospital Muenster, Muenster, Germany.

Objectives: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS.

Methods: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT. Read More

Epilepsia Open 2019 Mar 6;4(1):63-72. Epub 2018 Dec 6.

Department of Paediatrics & Adolescent Medicine LKS Faculty of Medicine The University of Hong Kong Hong Kong SAR China.

Objective: Early onset drug-resistant epilepsy is a neurologic disorder in which 2 antiepileptic drugs fail to maintain the seizure-free status of the patient. Heterogeneous clinical presentations make the diagnosis challenging. We aim to identify the underlying genetic causes of a pediatric cohort with drug-resistant epilepsy and evaluate whether the findings can provide information on patient management. Read More

Rev Esp Anestesiol Reanim 2019 Mar 9. Epub 2019 Mar 9.

Serviço de Anestesiologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal.

Brugada Syndrome (BrS) is a rare and high risk condition, seldom encountered in the delivery room. Pregnant patients with BrS benefit from the lowest possible doses of arrhythmogenic drugs such as local anesthetics. Based on this premise, the following case report exposes how a subarachnoid approach might be a desirable technique in C-section procedures for BrS patients. Read More

Front Physiol 2019 19;10:103. Epub 2019 Feb 19.

Guang'anmen Hospital, Chinese Academy of Chinese Medical Sciences, Beijing, China.

with Brugada syndrome (BrS) is not commonly considered as an independent risk marker for subsequent cardiac events. However, the risk of combined with other clinical characteristics has not been fully investigated. The aim of this study is to investigate and evaluate risk stratification and related risk factors of in BrS. Read More

Front Genet 2019 15;10:50. Epub 2019 Feb 15.

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

In this case series, we report for the first time a family in which the inherited nonsense mutation [c. 3946C > T (p.Arg1316*)] in the gene segregates in association with Brugada syndrome (BrS). Read More

Hum Mutat 2019 Feb 28. Epub 2019 Feb 28.

Cardiovascular Genetics Center, Institut d'Investigació Biomèdica Girona, University of Girona, Girona, Spain.

Brugada syndrome is an inherited arrhythmogenic disease associated with sudden cardiac death. The main gene is SCN5A. Additional variants in 42 other genes have been reported as deleterious, although these variants have not yet received comprehensive pathogenic analysis. Read More

Europace 2019 Feb 28. Epub 2019 Feb 28.

National Heart & Lung Institute, Imperial College London, London, UK.

Aims: Abnormal rate adaptation of the action potential is proarrhythmic but is difficult to measure with current electro-anatomical mapping techniques. We developed a method to rapidly quantify spatial discordance in whole heart activation in response to rate cycle length changes. We test the hypothesis that patients with underlying channelopathies or history of aborted sudden cardiac death (SCD) have a reduced capacity to maintain uniform activation following exercise. Read More

Cureus 2018 Dec 18;10(12):e3751. Epub 2018 Dec 18.

Cardiology, Aga Khan University Hospital, Karachi, PAK.

Fainting is a common clinical presentation, with vagally mediated (neurocardiogenic) causes being the most common for syncope presentation to the emergency room, and for hospital admissions. Classic teaching is that upright posture is a prerequisite for vagally mediated syncope (VMS) and that syncope in the supine position has more sinister causes. We present five patients, three males and two females, with a mean age of 44. Read More

Ned Tijdschr Geneeskd 2019 Feb 27;163. Epub 2019 Feb 27.

Haaglanden Medisch Centrum, afd. Cardiologie, Den Haag.

We present a patient with fever-induced Brugada syndrome, a rare cardiac disease caused by an inherited ion channelopathy and associated with malignant arrhythmias. ECG findings normalized after the fever resolved. As Brugada syndrome can be confused with anterior myocardial ischemia, right bundle branch block and intoxication with tricyclic antidepressants, ECG recognition is important. Read More

J Arrhythm 2019 Feb 7;35(1):133-135. Epub 2018 Dec 7.

Division of Cardiovascular Medicine Saitama Medical Center Jichi Medical University Saitama Japan.

A 62-year-old man with Brugada syndrome underwent subcutaneous implantable cardioverter defibrillator implantation. The lead was positioned along the left sternal border and defibrillation threshold (DFT) testing was performed. However, ventricular fibrillation (VF) was not terminated with 65 J and 80 J shocks. Read More

J Arrhythm 2019 Feb 3;35(1):18-24. Epub 2018 Sep 3.

Division of Cardiac Electrophysiology Cardiology Department University Hospital Santiago de Compostela Santiago de Compostela Spain.

Supraventricular arrhythmias are common in Brugada syndrome (BS), and notoriously difficult to manage with medical therapy secondary to associated risks. Pulmonary vein isolation (PVI) is often utilized instead, but its outcomes in this population are not well-known. We aim to provide a holistic evaluation of interventional treatment for Atrial fibrillation (AF) in the BS population. Read More

Eur Heart J 2019 Feb 21. Epub 2019 Feb 21.

Cardiology Department, Fondazione Cardiocentro Ticino, Via Tesserete 48, Lugano, Switzerland.

Methodist Debakey Cardiovasc J 2018 Oct-Dec;14(4):e1-e3

HOUSTON METHODIST DEBAKEY HEART AND VASCULAR CENTER, HOUSTON METHODIST HOSPITAL, HOUSTON, TEXAS.

Brugada syndrome is an inherited arrhythmia that increases a patient's risk of sudden cardiac death. Certain pharmacologic agents may induce a transient Brugada pattern on surface electrocardiogram (EKG). One of these is loperamide, an over-the-counter agent commonly used to manage diarrhea. Read More

JACC Clin Electrophysiol 2019 Feb;5(2):149-151

Division of Cardiology, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada. Electronic address:

JACC Clin Electrophysiol 2019 Feb 1;5(2):141-148. Epub 2018 Nov 1.

Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address:

Objectives: This study sought to summarize the clinical outcome of implantable cardioverter-defibrillator (ICD) therapy in patients with Brugada syndrome.

Background: Brugada syndrome is characterized by cardiac conduction abnormalities and a high risk of ventricular arrhythmias that may result in sudden cardiac death. A complete overview of clinical outcome, appropriate and inappropriate interventions, and complications after ICD therapy in patients with Brugada syndrome is lacking. Read More

Pacing Clin Electrophysiol 2019 Feb 18. Epub 2019 Feb 18.

Department of Cardiology, Health Sciences University, Ankara City Hospital, Ankara, Turkey.

Seizure 2019 Jan 28;66:26-30. Epub 2019 Jan 28.

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China. Electronic address:

Purpose: This study aimed to investigate the genetic etiology of epilepsy in a cohort of Chinese children.

Methods: Targeted next-generation sequencing (NGS) was performed for 120 patients with unexplained epilepsy, including 71 patients with early-onset epileptic encephalopathies, and 16 patients with Dravet syndrome (including three patients with a Dravet-like phenotype) but without SCN1A pathogenic variants.

Results: Pathogenic variants of 14 genes were discovered in 22 patients (18%). Read More

Front Physiol 2018 31;9:1951. Epub 2019 Jan 31.

Guang'anmen Hospital, Chinese Academy of Chinese Medical Sciences, Beijing, China.

Risk stratification of patients with Brugada syndrome (BrS) is vital for accurate prognosis and therapeutic decisions. Spontaneous Type 1 ST segment elevation is generally considered to be an independent risk factor for arrhythmic events. Other risk factors include gender, syncope, sudden cardiac arrest (SCA), and positive electrophysiological study (EPS). Read More

J Emerg Med 2019 Feb 9. Epub 2019 Feb 9.

Department of Emergency Medicine, University of Michigan, Ann Arbor, Michigan.

Background: Brugada pattern on electrocardiography (ECG) can manifest as type 1 (coved pattern) and type 2 (saddleback pattern). Brugada syndrome represents an ECG with Brugada pattern in a patient with symptoms or clinical factors, including syncope, cardiac arrest, ventricular dysrhythmias, and family history. Brugada syndrome is caused by a genetic channelopathy, but the Brugada pattern may be drug-induced. Read More

Int Heart J 2019 Feb 8. Epub 2019 Feb 8.

Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo.

A 41-year-old man developed cardiac arrest. A resting 12-lead electrocardiogram showed a delta wave, suggestive of preexcitation syndrome. An electrophysiological test revealed the existence of inducible atrial fibrillation and a fasciculoventricular accessory pathway (FVAP). Read More

BJGP Open 2018 Dec 12;2(4):bjgpopen18X101624. Epub 2018 Dec 12.

Doctor, Department of Cardiology, Shinshu University School of Medicine, Matsumoto, Japan.

Heart Rhythm 2019 Feb 2. Epub 2019 Feb 2.

Department of Pharmacology, Columbia University Medical Center, New York, New York. Electronic address:

Much has been written about arrhythmias in structurally normal hearts. In this review, we focus on rapid ventricular arrhythmias that occur in hearts having a pathogenic genetic variant that has been found in families in which arrhythmias occur. We discuss these mutations in terms of their effect on cardiac cell electrical function and initiation of arrhythmias. Read More

J Cardiovasc Electrophysiol 2019 Jan 30. Epub 2019 Jan 30.

Arrhythmia and EP Research Center, IRCCS Humanitas Research & University Hospital, Milan, Italy.

Circulation 2019 Jan 30. Epub 2019 Jan 30.

Cardiology Department, Sourasky Tel-Aviv Medical Center, Israel.

Background: Polymorphic ventricular tachycardia (VT) without QT prolongation is well described in patients without structural heart disease [mainly idiopathic ventricular fibrillation (VF) and Brugada syndrome] and in patients with acute ST-elevation myocardial infarction.

Methods: Retrospective study of patients with polymorphic VT related to coronary artery disease but without evidence of acute myocardial ischemia.

Results: We identified 43 patients who developed polymorphic VT within days of an otherwise uncomplicated myocardial infarction or coronary revascularization procedure. Read More

Coron Artery Dis 2019 Jan 28. Epub 2019 Jan 28.

Cardiology, Albert Einstein Medical Center, Philadelphia, Pennsylvania, USA.

Background: Rapid diagnosis of ST-segment elevation myocardial infarction (STEMI) is crucial for appropriate management. Catheterization for a false STEMI activation has risks including exposure to contrast agent and radiation, increased healthcare costs and delay in treatment of the primary medical condition.

Patients And Methods: This was a single center retrospective study including all 'cath alerts' between January 2012 and December 2015. Read More

Acta Cardiol 2019 Jan 29:1-3. Epub 2019 Jan 29.

a Service de Pathologie Cardiovasculaire , Cliniques Universitaires Saint-Luc , Bruxelles , Belgique.

QJM 2019 Jan 25. Epub 2019 Jan 25.

Department of Cardiovascular Medicine, Shiga University of Medical Sciences, Shiga, Japan.

Background: Brugada syndrome (BrS) is a heritable sudden cardiac death (SCD) disease with male predominance. Information on gender difference of BrS remains scarce.

Aim: We aimed to investigate the gender difference of BrS in Han Chinese. Read More

Exp Ther Med 2019 Feb 30;17(2):1143-1148. Epub 2018 Nov 30.

Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, 11527 Athens, Greece.

Athletic pre-participation screening is essential for minimizing the risk for sudden cardiac death (SCD) in athletes participating in either competitive or leisure sporting activities. The primary causes of SCD in young athletes (<35 years of age) include hypertrophic cardiomyopathy, congenital anomalies of the coronary artery and arrhythmogenic right ventricular cardiomyopathy. Other abnormalities, such as malignant arrhythmia due to blunt trauma to the chest (commotio cordis), myocarditis, valvular disease, aortic rupture (in Marfan syndrome) and ion channelopathies (catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, long or short QT syndrome), also contribute to a lesser degree to SCD. Read More

J Am Heart Assoc 2019 Feb;8(3):e010115

1 Department of Cardiology Hospital General Universitario Gregorio Marañón Instituto de Investigación Sanitaria Gregorio Marañón Facultad de Medicina Universidad Complutense Madrid Spain.

Background Several metabolic conditions can cause the Brugada ECG pattern, also called Brugada phenotype (BrPh). We aimed to define the clinical characteristics and outcome of BrPh patients and elucidate the mechanisms underlying BrPh attributed to hyperkalemia. Methods and Results We prospectively identified patients hospitalized with severe hyperkalemia and ECG diagnosis of BrPh and compared their clinical characteristics and outcome with patients with hyperkalemia but no BrPh ECG. Read More

Case Rep Cardiol 2018 18;2018:9837840. Epub 2018 Dec 18.

Department of Internal Medicine, University of Texas Health Science Center at the Heart and Vascular Institute, Houston, Texas, USA.

[This corrects the article DOI: 10.1155/2018/1465867.]. Read More

Front Genet 2018 4;9:680. Epub 2019 Jan 4.

Lankenau Institute for Medical Research, Wynnewood, PA, United States.

Brugada syndrome (BrS) is a heritable disease that results in sudden cardiac death. In the exome/genomic era, certain reported pathogenic variants in some genetic diseases have been reclassified as benign owing to their high frequency in some ancestries. In the present study, we comprehensively reassessed all previously reported pathogenic variants of BrS. Read More

J Cardiovasc Electrophysiol 2019 Jan 19. Epub 2019 Jan 19.

Department of Cardiovascular Biology and Medicine, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Introduction: Early repolarization syndrome is a recently proposed condition characterized by an early repolarization pattern in the electrocardiogram (ECG) and ventricular fibrillation in the absence of structural heart abnormalities. Although some studies have suggested that early repolarization is associated with frequency of atrial fibrillation, the association of early repolarization with atrial fibrillation is not well known.

Hypothesis: Early repolarization indicates the substrate for atrial fibrillation in addition to that for ventricular fibrillation. Read More

Epilepsy Res 2019 02 14;150:66-69. Epub 2019 Jan 14.

Research Institute of the McGill University Health Center, 2155 Guy Street, Montreal, PQ, H3H 2L9, Canada; Department of Pediatrics, Division of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, 1001 Boulevard Décarie, Montreal, PQ, H4A 3J1, Canada.

We aimed to describe families with genetic epilepsy with febrile seizures plus (GEFS+) in which individuals suffered sudden unexpected death. The Epilepsy Pharmacogenomics Research Database was reviewed for GEFS + families in which at least one individual had suffered sudden death, and two families were identified. In Family A, five males had febrile seizures and one girl had febrile seizures plus. Read More

Circ Res 2019 Jan;124(2):205-207

Department of Experimental Cardiology (T.O., R.C.), Amsterdam Cardiovascular Sciences, University of Amsterdam, Amsterdam UMC, the Netherlands.

Circ J 2019 Feb 12;83(3):532-539. Epub 2019 Jan 12.

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center.

Background: Spontaneous type 1 electrocardiogram (ECG) in the right precordial lead is a dominant predictor of ventricular fibrillation (VF) in Brugada syndrome (BrS). In some BrS patients with VF, however, spontaneous type 1 ECG is undetectable, even in repeated ECG and immediately after VF. This study investigated differences between BrS patients with spontaneous or drug-induced type 1 ECG. Read More

Kardiologiia 2018 Nov 24;58(11):41-52. Epub 2018 Nov 24.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University).

This review includes main positions of the revision of diagnostic criteria of "J-wave syndromes in the J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge" (2016). The article, systematized according to the sections of the above-mentioned document, outlines the questions of terminology, new criteria for diagnosis of the Brugada syndrome (BrS) and early repolarization syndrome (ERS). The section devoted to ERS on the issues of new terminology and standardization of measurements, is supplemented with material from the Consensus Paper - The Early Repolarization Pattern (2015). Read More

Minerva Anestesiol 2019 Feb 4;85(2):121-123. Epub 2019 Jan 4.

Unit of Anesthesiology, Division of Critical Care and Pain Medicine, Department of Medicine and Surgery, University of Parma, Parma, Italy.

Front Cardiovasc Med 2018 18;5:184. Epub 2018 Dec 18.

Leon H. Charney Division of Cardiology, NYU School of Medicine, New York, NY, United States.

Plakophilin-2 (PKP2) is a component of the desmosome complex and known for its role in cell-cell adhesion. Recently, alterations in the gene have been associated with different inherited cardiac conditions including Arrythmogenic Cardiomyopathy (ACM or ARVC), Brugada syndrome (BrS), and idiopathic ventricular fibrillation to name the most relevant. However, the assessment of pathogenicity regarding the genetic variations associated with is still a challenging task: the gene has a positive Residual Variation Intolerance Score and the potential deleterious role of several of its variants has been disputed. Read More

Heart Rhythm 2019 Jan 4. Epub 2019 Jan 4.

Department of Cardiovascular Medicine, Tokyo Medical and Dental University, Tokyo, Japan.

Background: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are an established therapy for preventing sudden cardiac death. However, a considerable number of patients still undergo inappropriate shocks even after conventional preimplantation electrocardiographic (ECG) screening.

Objective: This study aimed to elucidate the additional effect of diurnal variations in the QRS complex and T waves of 24-hour Holter screening on S-ICD eligibility. Read More

J Electrocardiol 2019 Jan 3;53:52-56. Epub 2019 Jan 3.

Department of Cardiology, People's Hospital of Zhengzhou University (Henan provincial people's hospital), Fuwai Central China Cardiovascular Hospital, Zhengzhou, Henan Province, China.

Background: ST-segment elevation (STE) and an increased Tpeak-Tend interval (Tp-e) have prognostic value for malignant arrhythmia events (MAEs) in patients with ST-segment elevation myocardial infarction (STEMI) and Brugada syndrome. Whether STE could predict MAEs and has an electrophysiological relationship with Tp-e in electrocardiogram (ECG) of vasospastic angina (VA) patients needs to be elucidated.

Methods: Sixty-five patients with VA and 23 patients with VA complicated by MAEs were enrolled. Read More

J Electrocardiol 2018 Dec 19;53:31-35. Epub 2018 Dec 19.

Department of Clinical and Experimental Medicine, University Hospital of Messina, Italy.

Since its first description, Brugada Syndrome is characterized by definite ECG abnormalities (J wave, elevated ST segment) confined in right precordial leads. Brugada Pattern (BP) could be found in one or more right precordial leads, at conventional or higher intercostal spaces. A recent study, conducted by our group, reported that slightly less than one half of patients with type 1 BP show a definite ST segment depression (≥0. Read More

Heart Rhythm 2018 Dec 27. Epub 2018 Dec 27.

Cardiac Surgery Department, Universitair Ziekenhuis Brussel - Vrije Universiteit Brussel, Brussels, Belgium.

Background: Abnormal delayed electrograms (EGMs) from the anterior wall of the right ventricular outflow tract (RVOT) epicardium have become the ablation target in Brugada syndrome (BrS).

Objective: The aim of this study was to analyze the safety, feasibility, and efficacy of a novel hybrid thoracoscopic approach to perform epicardial RVOT radiofrequency ablation in BrS.

Methods: Thirty-six patients affected by BrS (26 men; mean age 36. Read More

Europace 2018 Dec 24. Epub 2018 Dec 24.

Department of Cardiology, Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

Aims: Data on predictors of time-to-first appropriate implantable cardioverter-defibrillator (ICD) therapy in patients with Brugada Syndrome (BrS) and prophylactically implanted ICD's are scarce.

Methods And Results: SABRUS (Survey on Arrhythmic Events in BRUgada Syndrome) is an international survey on 678 BrS patients who experienced arrhythmic event (AE) including 252 patients in whom AE occurred after prophylactic ICD implantation. Analysis was performed on time-to-first appropriate ICD discharge regarding patients' characteristics. Read More

J Cardiovasc Electrophysiol 2018 Dec 21. Epub 2018 Dec 21.

Electrophysiology and Arrhythmia Service, Cardiology Division, Department of Medicine, Boston Medical Center, Boston, Massachusetts.

Subcutaneous implantable cardioverter defibrillators (S-ICDs) provide reliable defibrillation and have enhanced supraventricular tachycardia discrimination and fewer infection rates compared with traditional transvenous systems. However, inappropriate shocks remain a frequent problem. Herein, we review the various mechanisms of these inappropriate therapies, some of which are unique to S-ICDs, and propose an algorithm for preventing recurrences. Read More

J Electrocardiol 2018 Dec 12. Epub 2018 Dec 12.

Cardiologia Ospedale San Vincenzo Taormina, ASP Messina, Contrada Sirina, 98039 Taormina, Italy. Electronic address:

J Am Heart Assoc 2018 Nov;7(21):e009713

1 Division of Arrhythmia and Electrophysiology Department of Cardiovascular Medicine National Cerebral and Cardiovascular Center Suita Japan.

Background Epicardial mapping can reveal low-voltage areas on the right ventricular outflow tract in patients with Brugada syndrome with several ventricular fibrillation ( VF ) episodes. A type 1 ECG is associated with an abnormal electrogram on right ventricular outflow tract epicardium. This study investigated the clinical significance of the amplitude of type 1 ECGs in patients with Brugada syndrome. Read More

Circulation 2018 Nov;138(22):2582-2583

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

Circulation 2018 Nov;138(22):2584-2585

Tel Aviv Medical Center and Sackler School of Medicine, Tel Aviv University, Israel.

Circ Genom Precis Med 2018 Nov;11(11):e002263

Department of Cardiology, University Hospital Heidelberg (C.S., F.W., A.R., H.A.K., D.T.).

Pacing Clin Electrophysiol 2019 Feb 4;42(2):265-266. Epub 2019 Jan 4.

Division of Cardiology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.