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Circ J 2019 Jan 12. 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 Jan 4. Epub 2019 Jan 4.

Anesthesiology, Critical Care and Pain Medicine Division, 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 pre-implantation ECG screening.

Objective: This study aimed to elucidate the additional impact of diurnal variations in the QRS complex and T-waves of 24-h Holter monitoring on the 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 (EGM) from the anterior wall of the right ventricular outflow tract (RVOT) epicardium has become the ablation target in Brugada Syndrome (BrS).

Objetive: The study's aim was to analyse the safety, feasibility and efficacy of a novel hybrid thoracoscopic approach to perform epicardial RVOT radio-frequency (RF) ablation in BrS.

Methods: Thirty-six patients affected by BrS (26 males; 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 2018 Dec 19. Epub 2018 Dec 19.

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

Pacing Clin Electrophysiol 2018 Dec 20. Epub 2018 Dec 20.

Imperial College Healthcare NHS Trust, London, UK.

Introduction: A spontaneous type I electrocardiogram (ECG) pattern and/or unheralded syncope are conventionally used as risk markers for primary prevention of sudden cardiac arrest/death (SCA/SCD) in Brugada syndrome (BrS). In this study, we determine the prevalence of conventional and newer markers of risk in those with and without previous aborted SCA events.

Methods: All patients with BrS were identified at our institute. Read More

J Cardiovasc Med (Hagerstown) 2019 Feb;20(2):59-65

Heart Rhythm Management Center, Postgraduate program in Cardiac Electrophysiology and Pacing.

Background: The present study sought to evaluate the incidence of cerebrovascular events in a large cohort of patients with Brugada syndrome (BrS) analysing possible predictors, clinical characteristics and prognosis of cardioembolic events secondary to atrial fibrillation.

Methods: A total of 671 consecutive patients (age 42.1 ± 17. Read More

J Arrhythm 2018 Dec 10;34(6):587-597. Epub 2018 Sep 10.

Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University Tianjin China.

Background: Brugada syndrome is an ion channelopathy that predisposes affected subjects to ventricular tachycardia/fibrillation (VT/VF), potentially leading to sudden cardiac death (SCD). T-T intervals, (T-T)/QT ratio and T-T dispersion have been proposed for risk stratification, but their predictive values in Brugada syndrome have been challenged recently.

Methods: A systematic review and meta-analysis was conducted to examine their values in predicting arrhythmic and mortality outcomes in Brugada Syndrome. Read More

Heart Lung Circ 2019 Jan 15;28(1):123-133. Epub 2018 Oct 15.

Department of Cardiology, Westmead Hospital, Westmead Applied Research Centre, University of Sydney, Sydney, NSW, Australia. Electronic address:

Cardiac electrical storm (ES) is characterised by three or more discrete episodes of ventricular arrhythmia within 24hours, or incessant ventricular arrhythmia for more than 12hours. ES is a distinct medical emergency that portends a significant increase in mortality risk and often presages progressive heart failure. ES is also associated with psychological morbidity from multiple implanted cardioverter defibrillator (ICD) shocks and exponential health resource utilisation. Read More

Am J Med Case Rep 2018 7;6(9):173-179. Epub 2018 Oct 7.

Division of Cardiovascular Disease and Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn, New York, U.S.A- 11203.

Brugada syndrome is a rare cardiac arrhythmia which is associated with right bundle branch block pattern (RBBB) and ST-segment elevation in right precordial leads. SCNA5 mutation is the most common genetic abnormality associated with Brugada syndrome. Brugada pattern not related to genetic mutations has been previously reported in the setting of fever, metabolic conditions, lithium use, marijuana and cocaine abuse, ischemia and pulmonary embolism, myocardial and pericardial diseases. Read More

Artif Intell Med 2018 Nov 28. Epub 2018 Nov 28.

Univ Rennes, CHU Rennes, Inserm, LTSI UMR 1099, F-35000 Rennes, France.

This paper proposes the integration and analysis of a closed-loop model of the baroreflex and cardiovascular systems, focused on a time-varying estimation of the autonomic modulation of heart rate in Brugada syndrome (BS), during exercise and subsequent recovery. Patient-specific models of 44 BS patients at different levels of risk (symptomatic and asymptomatic) were identified through a recursive evolutionary algorithm. After parameter identification, a close match between experimental and simulated signals (mean error = 0. Read More

Am J Med Genet A 2018 Dec 4;176(12):2733-2739. Epub 2018 Dec 4.

Division of Pediatric Neurology, Department of Neurology, University of Washington, Seattle, Washington.

CACNA1C (NM_000719.6) encodes an L-type calcium voltage-gated calcium channel (Ca 1.2), and pathogenic variants have been associated with two distinct clinical entities: Timothy syndrome and Brugada syndrome. Read More

J Electrocardiol 2018 Nov - Dec;51(6):963-965. Epub 2018 Aug 23.

Division of Cardiology, Kingston Health Sciences Centre, Queen's University, Kingston, ON, Canada.

Brugada phenocopies (BrP) are clinical entities that are characterized by ECG patterns identical to those of Brugada syndrome, but are the result of various clinical conditions. We describe the case of a 41-year-old male who exhibited BrP due to a left ventricular aneurysm in the context of chronic coronary artery disease. Read More

J Electrocardiol 2018 Nov - Dec;51(6):1121-1123. Epub 2018 Oct 9.

Department of Cardiovasology, Changhai Hospital, Second Military Medical University, Shanghai, China. Electronic address:

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Read More

J Am Coll Cardiol 2018 Dec;72(22):2758-2760

Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua, Padua, Italy.

J Am Coll Cardiol 2018 Dec;72(22):2747-2757

Cardiovascular Department, San Donato Hospital, Arezzo, Italy.

Background: The prevalence and significance of structural abnormalities in Brugada syndrome (BrS) are still largely debated.

Objectives: The authors investigated the relationship between genetic background, electroanatomic abnormalities, and pathologic substrate in BrS.

Methods: They performed 3-dimensional electroanatomic unipolar and bipolar mapping in 30 patients with BrS. Read More

Toxins (Basel) 2018 Nov 23;10(12). Epub 2018 Nov 23.

Institute for Drug Research, School of Pharmacy, The Hebrew University of Jerusalem, Jerusalem 91120, Israel.

This article describes the discovery and use of the South American cinchona bark and its main therapeutic (and toxic) alkaloids, quinine and quinidine. Since the introduction of cinchona to Europe in the 17th century, it played a role in treating emperors and peasants and was central to colonialism and wars. Over those 400 years, the medical use of cinchona alkaloids has evolved from bark extracts to chemical synthesis and controlled clinical trials. Read More

J Electrocardiol 2018 Nov 14;52:82-87. Epub 2018 Nov 14.

Department of Cardiovascular Medicine, First Affiliated Hospital of Xi'an Jiaotong University, PR China; Key Laboratory of Molecular Cardiology, Xi'an Jiaotong University, Shaanxi Province, PR China; Key Laboratory of Environment and Genes Related to Diseases, Xi'an Jiaotong University, PR China. Electronic address:

Brugada syndrome (BS) is an autosomal dominant disease. The most common causes of BS are loss-of-function mutations occur in the SCN5A gene which encodes the sodium channel protein Nav1.5. Read More

J Electrocardiol 2018 Nov 6;52:75-78. Epub 2018 Nov 6.

Cardiology Department, ASST Nord Milano, Ospedale E. Bassini, Cinisello Balsamo, Milano, Italy.

Zhonghua Xin Xue Guan Bing Za Zhi 2018 Nov;46(11):901-903

Zhonghua Xin Xue Guan Bing Za Zhi 2018 Nov;46(11):862-867

Department of Cardiology, First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.

To investigate the risk factors of ventricular arrhythmias in patients with Brugada syndrome. Clinical data of 60 Brugada syndrome patients admitted in the department of cardiology of the First Affiliated Hospital of Nanjing Medical University from March 2003 to December 2016 were collected and retrospectively analyzed. The age at diagnosis was (43. Read More

Front Cardiovasc Med 2018 6;5:161. Epub 2018 Nov 6.

Heart Rhythm Management Centre, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.

During last centuries, Early Repolarization pattern has been interpreted as an ECG manifestation not linked to serious cardiovascular events. This view has been challenged on the basis of sporadic clinical observations that linked the J-wave with ventricular arrhythmias and sudden cardiac death. The particular role of this characteristic pattern in initiating ventricular fibrillation has been sustained by clinical descriptions of a marked and consistent J-wave elevation preceding the onset of the ventricular arrhythmia. Read More

Front Cardiovasc Med 2018 29;5:149. Epub 2018 Oct 29.

Medical Science Department, University of Girona, Girona, Spain.

Short QT syndrome is a highly malignant inherited cardiac disease characterized by ventricular tachyarrhythmias leading to syncope and sudden cardiac death. It is responsible of lethal episodes in young people, mainly infants. International guidelines establish diagnostic criteria with the presence of a QTc ≤ 340 ms in the electrocardiogram despite clinical diagnostic values remain controversial. Read More

PLoS One 2018 12;13(11):e0207437. Epub 2018 Nov 12.

Laboratory for Biological Information Processing, Graduate School of Science and Engineering, University of Toyama, Toyama City, Toyama, Japan.

SCN5A encodes the main subunit of the NaV1.5 channel, which mediates the fast Na+ current responsible for generating cardiac action potentials. The single nucleotide polymorphism SCN5A(R1193Q), which results in an amino acid replacement in the subunit, is common in East Asia. Read More

Arrhythm Electrophysiol Rev 2018 Aug;7(3):199-209

Lankenau Institute for Medical Research Wynnewood, PA, USA.

A number of antipsychotic and antidepressant drugs are known to increase the risk of ventricular arrhythmias and sudden cardiac death. Based largely on a concern over the development of life-threatening arrhythmias, a number of antipsychotic drugs have been temporarily or permanently withdrawn from the market or their use restricted. While many antidepressants and antipsychotics have been linked to QT prolongation and the development of torsade de pointes arrhythmias, some have been associated with a Brugada syndrome phenotype and the development of polymorphic ventricular arrhythmias. Read More

Am J Emerg Med 2018 Oct 23. Epub 2018 Oct 23.

Carl R. Darnall Army Medical Center, Emergency Medicine Residency, 36065 Santa Fe Ave, Fort Hood, TX 76544, United States of America. Electronic address:

Background: Brugada pattern is a well-known pathological finding on electrocardiogram (ECG) which increases the likelihood of cardiac arrest due to ventricular arrhythmia. These cases generally present in younger patients without evidence of an electrolyte abnormality, structural heart disease, or cardiac ischemia. In many instances, this pattern is either hidden on initial presentation or presents as an incidental finding on an EKG. Read More

Orv Hetil 2018 11;159(45):1848-1850

A Betegápoló Irgalmasrend Budai Irgalmasrendi Kórháza Budapest, Árpád fejedelem útja 7., 1023.

An Asian patient was admitted with palpitation. The ECG showed coved-shaped type Brugada pattern with QRS-fragmentations. Early recognition and risk stratification are the most important issues in Brugada syndrome. Read More

Pol Arch Intern Med 2018 12 7;128(12):721-730. Epub 2018 Nov 7.

INTRODUCTION Unexplained sudden cardiac arrest (SCA), occurs in up to 10% of patients and is often attributed to an inherited arrhythmia syndrome. Family screening and genetic testing may help clarify the cause of unexplained SCA. OBJECTIVES We aimed to assess the usefulness of clinical evaluation and genetic testing in patients after unexplained SCA and in their families. Read More

Minerva Anestesiol 2018 Oct 30. Epub 2018 Oct 30.

Pediatric Arrhythmia Unit, Department of Cardiology, Sant Joan de Déu Hospital, Barcelona, Spain.

Brugada syndrome is characterized by arrhythmogenic risk that may be exacerbated by different metabolic and pharmacological factors. Since its first description, knowledge of this syndrome and its detection by physicians belonging to different specialties have gradually increased. The risk of arrhythmias is well known to increase in the postoperative period, and this risk is particularly accentuated in patients with Brugada syndrome. Read More

J Pharmacol Sci 2018 Nov 17;138(3):198-202. Epub 2018 Oct 17.

Department of Pharmacology, Toho University Graduate School of Medicine, 5-21-16 Omori-nishi, Ota-ku, Tokyo, 143-8540, Japan; Department of Pharmacology, Faculty of Medicine, Toho University, 5-21-16 Omori-nishi, Ota-ku, Tokyo, 143-8540, Japan. Electronic address:

Although azithromycin can suppress cardiac I, I, I, I and I, its onset mechanisms for cardiovascular death have not been fully investigated. We examined electropharmacological effects of azithromycin in intravenous doses of 0.3, 3 and 30 mg/kg using microminipigs under the halothane anesthesia (n = 4), which provided plasma concentrations of 3. Read More

Am J Case Rep 2018 Nov 3;19:1311-1316. Epub 2018 Nov 3.

Department of Cardiology, Camden Clark Medical Center, Parkersburg, WV, USA.

BACKGROUND Brugada syndrome is a cardiac disorder associated with sudden death due to sodium channelopathy, most commonly the SCN5a mutation. There are 3 different patterns of electrocardiogram (ECG) changes characterized as type I, II, and III. ECG patterns consist of variations of incomplete RBBB and ST elevation in anterior precordial leads only. Read More

Heart Lung Circ 2019 Jan 4;28(1):22-30. Epub 2018 Oct 4.

Heart Centre AMC, Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA.

Forty per cent (40%) of sudden unexpected natural deaths in people under 35 years of age are associated with a negative autopsy, and the cardiac ion channelopathies are the prime suspects in such cases. Long QT syndrome (LQTS), Brugada syndrome (BrS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are the most commonly identified with genetic testing. The cellular action potential driving the heart cycle is shaped by a specific series of depolarising and repolarising ion currents mediated by ion channels. Read More

J Cardiovasc Electrophysiol 2019 Jan 12;30(1):128-131. Epub 2018 Nov 12.

Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam.

J Am Heart Assoc 2018 Sep;7(18):e009387

1 Keio University Hospital Tokyo Japan.

Background Brugada syndrome ( BS ) is known to be 9 times more prevalent in males than females. However, little is known about the development of sick sinus syndrome in female members with familial BS . Methods and Results Familial BS patients and family members, both from our institutions and collaborating sites that specialize in clinical care of BS , participated in this study. Read More

J Interv Card Electrophysiol 2018 Oct 26. Epub 2018 Oct 26.

Department of Cardiology, Graduate School of Medicine, Gifu University, Gifu, Japan.

Front Physiol 2018 9;9:1372. Epub 2018 Oct 9.

Department of Cardiology, Shanghai Institute of Cardiovascular Disease, Zhongshan Hospital, Fudan University, Shanghai, China.

The gene encodes the alpha subunit of the main cardiac sodium channel Na1.5. This channel predominates inward sodium current (INa) and plays a critical role in regulation of cardiac electrophysiological function. Read More

Front Cardiovasc Med 2018 9;5:139. Epub 2018 Oct 9.

CERVO Research Centre, Institut Universitaire en Santé Mentale de Québec, Quebec City, QC, Canada.

Voltage gated sodium channels (Na) are broadly expressed in the human body. They are responsible for the initiation of action potentials in excitable cells. They also underlie several physiological processes such as cognitive, sensitive, motor, and cardiac functions. Read More

Circ Arrhythm Electrophysiol 2018 Aug;11(8):e006675

Hopital Cardiologique du Haut-Leveque, Center Hospitalier Universitaire Bordeaux, University Hospital Institutions L'Institut de Rhythmologie et Modelisation Cardiaque France (M.H.).

Circ Arrhythm Electrophysiol 2018 Aug;11(8):e005631

Cardiovascular Division, Faculty of Medicine, Tsukuba University, Ibaraki, Japan (A.K.T., S.A., E.S., Y.K., K.K., T.K., X.D., K.T., M.I., H.Y., N.M., Y.S., A.N., K.A.).

Background Both endocardial trigger elimination and epicardial substrate modification are effective in treating ventricular fibrillation (VF) in Brugada syndrome. However, the primary approach and the characteristics of patients who respond to endocardial ablation remain unknown. Methods Among 123 symptomatic Brugada syndrome patients (VF, 63%; syncope, 37%), ablation was performed in 21 VF/electrical storm patients, the majority of whom were resistant to antiarrhythmic drugs. Read More