BACKGROUND

Brugada syndrome is an inheritable syndrome that carries an increased risk of sudden cardiac death. This study aims to delineate the natural history, clinical and electrophysiologic profile, treatment strategies, and outcomes of patients with Brugada pattern electrocardiogram (ECG) and Brugada syndrome in a tertiary care hospital in the Philippines.

This is a retrospective observational study of patients diagnosed with Brugada pattern ECG and Brugada syndrome admitted in a single center from 2004 to 2019.

Twenty-three patients were included in the study. All patients were male. In both groups, the majority were nonsmokers (n = 13) and nonalcoholic beverage drinker (n = 14). In patients with Brugada pattern ECG, all presented as an incidental finding (n = 10; P < 0.001), whereas in Brugada syndrome, six patients presented with out-of-hospital resuscitated cardiac arrest (46.15%, P = 0.019). There were only three patients with a family history of sudden cardiac death. All patients had normal ejection fraction and sinus rhythm. Twenty-one patients had ST elevation (91.30%). Fourteen patients had spontaneous type 1 Brugada pattern ECG, whereas the rest were inducible to type 1 after flecainide test. Seven patients underwent implantable cardioverter defibrillator implantation before discharge; one patient died at the time of diagnosis, and one had recurrent admission for arrhythmia. No single patient received medical management.

Clinical profile, ECG findings, and outcomes were not significantly different between the two groups of patients except for the clinical presentation upon diagnosis. Treatment strategies were guideline-directed.

No abstract available.
Anesthesia, Intravenous* ; Brugada Syndrome* ; Humans

No abstract available.
Brugada Syndrome* ; Bundle-Branch Block* ; Death, Sudden, Cardiac*

The concept of J wave syndromes was first proposed in 2004 by Yan et al for a spectrum of electrocardiographic (ECG) manifestations of prominent J waves that are associated with a potential to predispose affected individuals to ventricular fibrillation (VF). Although the concept of J wave syndromes is widely used and accepted, there has been tremendous debate over the definition of J wave, its ionic and cellular basis and arrhythmogenic mechanism. In this review article, we attempted to discuss the history from which the concept of J wave syndromes (JWS) is evolved and current controversies in JWS.
Brugada Syndrome ; Death, Sudden, Cardiac ; Electrocardiography ; Ventricular Fibrillation

Adult ; Brugada Syndrome ; Defibrillators, Implantable ; adverse effects ; Electrocardiography ; Humans ; Male

OBJECTIVEThe objective was to provide a brief history of J wave syndromes and to summarize our current understanding of their molecular, ionic, cellular mechanisms, and clinical features. We will also discuss the existing debates and further direction in basic and clinical research for J wave syndromes.

DATA SOURCESThe publications on key words of "J wave syndromes", "early repolarization syndrome (ERS)", "Brugada syndrome (BrS)" and "ST-segment elevation myocardial infarction (STEMI)" were comprehensively reviewed through search of the PubMed literatures without restriction on the publication date.

STUDY SELECTIONOriginal articles, reviews and other literatures concerning J wave syndromes, ERS, BrS and STEMI were selected.

RESULTSJ wave syndromes were firstly defined by Yan et al. in a Chinese journal a decade ago, which represent a spectrum of variable phenotypes characterized by appearance of prominent electrocardiographic J wave including ERS, BrS and ventricular fibrillation (VF) associated with hypothermia and acute STEMI. J wave syndromes can be inherited or acquired and are mechanistically linked to amplification of the transient outward current (I to )-mediated J waves that can lead to phase 2 reentry capable of initiating VF.

CONCLUSIONSJ wave syndromes are a group of newly highlighted clinical entities that share similar molecular, ionic and cellular mechanism and marked by amplified J wave on the electrocardiogram and a risk of VF. The clinical challenge ahead is to identify the patients with J wave syndromes who are at risk for sudden cardiac death and determine the alternative therapeutic strategies to reduce mortality.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) is the therapy of choice in the prevention of SCD. The ICD has been proven to improve survival among survivors of cardiac arrest, patients who are at risk of having one because of myocardial scarring and low left ventricular ejection fraction, and those with primary inherited arrhythmia syndromes. The insertion of an ICD is indicated for survivors of cardiac arrest due to ventricular fibrillation or hemodynamically unstable sustained ventricular tachycardia after the exclusion of any reversible cause. At present, there are no nationwide data regarding the prevalence of ventricular tachyarrhythmias requiring an ICD. OBJECTIVES: The aim of this study was to determine the (1) total hospitalization claims per year (2017 and 2018) for arrhythmias requiring an ICD, (2) total number of cardioverter-defibrillator insertions done for patients mentioned in (1), (3) prevalence of hospitalization for cardiac arrhythmias requiring insertion of ICD among patients admitted for medical conditions for 2017 and 2018, (4) the demographic profile of patients who were hospitalized for cardiac arrhythmias requiring insertion of ICD, (5) type of facilities (primary, secondary, or tertiary; government or private) where the patients were confined, and (6) duration of hospitalization and mortality rate of patients admitted for the above conditions. METHODS: This is a descriptive study using the database of Philippine Health Insurance Corporation (PhilHealth) on hospital claims of admitted patients 19 years or older in PhilHealth-accredited hospitals from January 1, 2017, to December 31, 2018. Anonymized records were reviewed using the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) of the World Health Organization. The codes relevant to the study question were chosen and used. The PhilHealth Relative Value Scale (RVS) codes were used to determine the procedures that the patients underwent for their cardiac arrhythmias. Patients’ demographic profile, diagnosis, region, hospitalization stay and status on discharge, and type of facility of admission were collected. Descriptive statistics using median value and interquartile range for quantitative data and frequency and percentage for categorical data were reported. RESULTS: In 2017 and 2018, among patients 19 years or older, there were 1282 and 1480 claims of cases of cardiac arrhythmias requiring an ICD (based on ICD-10 codes), whereas those who underwent placement of an ICD (based on RVS codes) numbered 50 and 45, respectively. The prevalence of hospitalization for cardiac arrhythmias related to ICD among patients who were admitted for medical conditions was 0.05% and 0.06%, for the same years. The overall in-hospital mortality rates were 10.84% and 9.46% in each year for those who were admitted for cardiac arrhythmias that required an ICD; however, there were no recorded mortalities for those who underwent implantation of a cardioverter-defibrillator. For patients with arrhythmias requiring an ICD (based on ICD-10 and RVS codes), the median (first quartile [Q1], third quartile [Q3]) age was 59 (43, 72) years. In 2018, the median (Q1, Q3) age was 57 (38, 71) years. There were similar proportions of males (53%) and females (47%) in both years. The majority of cases based on ICD-10 codes of cardiac arrhythmias requiring an ICD were confined in government hospitals, whereas most of the claims for procedures based on RVS codes were in private hospitals. Most of the procedures were done in the National Capital Region. The duration of hospitalization was 3 to 4 days. CONCLUSION Based on PhilHealth claims, the admission due to cardiac arrhythmias requiring ICD is common in both private and government hospitals. Cardioverter-defibrillator implantation is being done mostly in the National Capital Region and private hospitals. There is a wide gap between the prevalence of cardiac arrhythmias requiring an ICD and the frequency of implantation of the lifesaving device.
implantable cardioverter-defibrillator ; Defibrillators, Implantable ; Death, Sudden, Cardiac ; Brugada Syndrome

Implantable cardioverter defibrillator (ICD) can be a crucial therapeutic modality for pediatric patients with congenital heart disease, Brugada syndrome, long QT syndrome and cardiomyopathy. Because transvenous implantation of ICD is mostly unfeasible for pediatric patients due to anatomical and technical limitations, epicardial patch type or subcutaneous type ICD have been used. Implantation of these alternative ICDs, however, was reported to be frequently associated with significant complications. We report a case of successful intrapericardial implantation of a single coil-type ICD through the transverse sinus in a 27 month-old child weighing lesser than 10 kg, and it was inferred from this experience that this alternative technique may decrease complications and morbidities after ICD implantation in children.
Arrhythmias, Cardiac ; Brugada Syndrome ; Cardiomyopathies ; Child ; Defibrillators ; Defibrillators, Implantable ; Heart Diseases ; Humans ; Long QT Syndrome

Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.
Adult ; Brugada Syndrome ; Death, Sudden, Cardiac ; Electrocardiography ; Humans ; Korea ; Sodium Channels ; Tachycardia, Ventricular

BACKGROUND AND OBJECTIVES: Implantable cardioverter–defibrillators (ICDs) are indicated in patients with Brugada syndrome (BS), early repolarization syndrome (ERS), or idiopathic ventricular fibrillation (IVF) who are at high risk for sudden cardiac death. The optimal ICD programming for reducing inappropriate shocks in these patients remains to be determined. We investigated the difference in the mean cycle length of tachyarrhythmias that activated either appropriate or inappropriate ICD shocks in these three patient groups to determine the optimal ventricular fibrillation (VF) zone for minimizing inappropriate ICD shocks. SUBJECTS AND METHODS: We selected 41 patients (35 men) (mean age±standard deviation=42.6±13.0 year) who received ICD shocks between April 1996 and April 2014 to treat BS (n=24), ERS (n=9), or IVF (n=8). Clinical and ICD interrogation data were retrospectively collected and analyzed for all events with ICD shocks. RESULTS: Of the 244 episodes, 180 (73.8%) shocks were appropriate and 64 (26.2%) were inappropriate. The mean cycle lengths of the tachyarrhythmias that activated appropriate and inappropriate shocks were 178.9±28.7 ms and 284.8±24.4 ms, respectively (p<0.001). The cutoff value with the highest sensitivity and specificity for discriminating between appropriate and inappropriate shocks was 235 ms (sensitivity, 98.4%; specificity, 95.6%). When we programmed a single VF zone of ≤270 ms, inappropriate ICD shocks were reduced by 70.5% and appropriate shocks were missed in 1.7% of these patients. CONCLUSION: Programming of a single VF zone of ≤270 ms in patients with BS, ERS, or IVF could reduce inappropriate ICD shocks, with a low risk of missing appropriate shocks.
Brugada Syndrome* ; Death, Sudden, Cardiac ; Defibrillators* ; Humans ; Retrospective Studies ; Sensitivity and Specificity ; Shock* ; Tachycardia* ; Ventricular Fibrillation*