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.

OBJECTIVE: To explore the correlation between DSG2, TTN and GATA4 genes and Brugada syndrome in Henan Province of China. METHODS: From February 2017 to February 2019, 100 patients with Brugada syndrome and 100 healthy individuals were selected as the study and the control groups, respectively. Electrocardiogram and echocardiography were carried out, and peripheral blood samples was collected. Coding regions of DSG2, TTN and GATA4 genes were amplified by PCR and sequenced. The results were compared with standard sequences from GenBank. RESULTS: Electrocardiogram showed that all patients from the study group had ventricular arrhythmia, 87 cases (87%) presented ventricular tachycardia (VT), 84 cases (84%) presented T wave inversion, and 51 cases (51%) presented Epsilon wave. Echocardiography showed that the right ventricle in the study group was enlarged with the inner diameter of the right ventricle being (40.0±13.3) mm, and the right ventricle showed various degree of abnormal systolic function. The enlargement of right atrium accounted for 64%, and the involvement of the left ventricle accounted for 27%. The right ventricular diameter and left ventricular diastolic diameter of the study group were significantly greater than those of the control group (P< 0.05). DNA sequencing showed that 60 patients carried DSG2 gene variants, among which 18 had missense variant of exon 8. Fifty patients carried TTN gene variants, including 8 in the A-band domain and 3 in the I-band domain. Twenty patients carried 3 variants of the GATA4 gene. CONCLUSION Variants of the DSG2, TTN and GATA4 genes in Henan region are correlated with the onset of Brugada syndrome.
Arrhythmogenic Right Ventricular Dysplasia ; Brugada Syndrome/genetics* ; China ; Connectin ; Desmoglein 2/genetics* ; GATA4 Transcription Factor ; Humans ; Pedigree ; Sequence Analysis, DNA

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

Brugada syndrome (BrS) is an arrhythmogenic disease associated with an increased risk of ventricular fibrillation (VF) and sudden cardiac death (SCD). To date, the standard therapy for the prevention of SCD in BrS is the use of an implantable cardioverter-defibrillator (ICD) especially in patients who have experienced a prior cardiac arrest or syncopal events secondary to VF. However, ICDs do not prevent the occurrence of VF but react to defibrillate the VF episode, thereby preventing SCD. Often patients with recurrent VF have to be maintained on antiarrhythmic drugs that are effective but have remarkable adverse effects. An alternative therapy for BrS with recurrent VF is catheter ablation which emerged as an effective therapy in eliminating VF-triggering premature ventricular complexes in limited case series; however, there has been a remarkable progress in effectiveness of catheter ablation since epicardial substrate ablation was first applied in 2011 and such approach is now widely applicable.
Anti-Arrhythmia Agents ; Brugada Syndrome ; Catheter Ablation ; Catheters ; Death, Sudden, Cardiac ; Defibrillators, Implantable ; Heart Arrest ; Humans ; Ventricular Fibrillation ; Ventricular Premature Complexes

Anti-Arrhythmia Agents ; Brugada Syndrome/complications* ; Bundle-Branch Block/complications* ; Electrocardiography ; Humans ; Quinidine ; Ventricular Fibrillation

A 34-year-old man presented to the outpatient clinic with syncope for 1 minute when he was working. He had no past medical and family history of sudden cardiac death. Electrocardiography (ECG) showed no remarkable findings. Follow-up ECG different from initial ECG was changed to type 1 Brugada ECG pattern. As time goes by, follow-up ECG was changed to type 2 Brugada ECG pattern. Although cause of syncope was assessed as Brugada syndrome, we performed coronary angiography to rule out ischemic heart disease. Coronary angiogram showed significant stenosis in the left main coronary artery (LMCA). Percutaneous coronary intervention with sirolimus-eluting stent was done at LMCA. He has been followed up for 1 year without any events. This is the first case of LMCA as syncope with Brugada type ECG in a young patient and it gives us the lesson that ischemic heart disease should be considered as the cause of syncope with Brugada type ECG.
Adult ; Ambulatory Care Facilities ; Brugada Syndrome ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease ; Coronary Stenosis ; Coronary Vessels ; Death, Sudden, Cardiac ; Electrocardiography ; Follow-Up Studies ; Humans ; Myocardial Ischemia ; Percutaneous Coronary Intervention ; Stents ; Syncope

The recommendations outlined constitute the first clinical practice guidelines of the Korean Heart Rhythm Society regarding catheter ablation of ventricular arrhythmias (VA). This is a guideline PART 2, which includes VA in the structurally normal heart, inherited primary arrhythmia syndromes, VA related to congenital heart disease, as well as VA and sudden cardiac death observed in specific populations. In the structurally normal heart, treatment is guided by the occurrence of symptoms or the frequency of arrhythmias that cause ventricular dysfunction over time. Catheter ablation can be recommended in patients in whom anti-arrhythmic medications are ineffective. The sites of origin of arrhythmic activity are known to be the outflow tract, fascicles, papillary muscle, or the annulus. Specific cardiac channelopathies include congenital long QT and Brugada syndrome. This guideline discusses the diagnostic criteria, risk stratification, and treatment of these syndromes. We have included recommendations for adult congenital heart disease. Moreover, we have discussed the management of VA occurring in specific populations such as in patients with psychiatric and neurological disorders, pregnant patients, those with obstructive sleep apnea or drug-related pro-arrhythmias, athletes, and elderly patients.
Adult ; Aged ; Arrhythmias, Cardiac ; Athletes ; Brugada Syndrome ; Catheter Ablation ; Catheters ; Channelopathies ; Death, Sudden, Cardiac ; Heart ; Heart Defects, Congenital ; Humans ; Nervous System Diseases ; Papillary Muscles ; Sleep Apnea, Obstructive ; Ventricular Dysfunction

Brugada syndrome is an arrhythmic syndrome characterized by right bundle branch block, ST segment elevation in the precordial lead (V1-V3), and sudden death caused by ventricular fibrillation, which is not effectively prevented by anti-arrhythmic drug therapy. We are reporting a 30-year-old male patient with Brugada syndrome who got an exploratory laparotomy and a tenorrhaphy due to stab wound which was managed with general anesthesia and brachial plexus block without any complications.
Adult ; Anesthesia, General ; Brachial Plexus Block* ; Brachial Plexus* ; Brugada Syndrome* ; Bundle-Branch Block ; Death, Sudden ; Drug Therapy ; Humans ; Laparotomy ; Male ; Ventricular Fibrillation ; Wounds, Stab

The ST segment corresponds to the plateau phase of ventricular repolarization, i.e., phase 2 of the action potential. Heightened awareness of the characteristic patterns of ST segment changes is vital to quickly identifying life-threatening disorders. The differential diagnosis of ST segment elevation includes four major processes: ST segment elevation myocardial infarction (STEMI); early repolarization; pericarditis; and ST elevation secondary to an abnormality of the QRS complex (left bundle branch block, left ventricular hypertrophy, or preexcitation). Other processes that may be associated with ST elevation include hyperkalemia, pulmonary embolism, and Brugada syndrome. Two particular patterns of ST segment depression reflect STEMI rather than non-ST-segment elevation acute coronary syndrome: ST segment depression that is reciprocal to a subtle and sometimes overlooked ST-segment elevation, and ST segment depression that is maximal in leads V₁-V₃, suggesting true posterior infarction. The clinical setting and specific electrocardiographic criteria often allow identification of the cause.
Action Potentials ; Acute Coronary Syndrome ; Brugada Syndrome ; Bundle-Branch Block ; Depression ; Diagnosis, Differential ; Electrocardiography ; Hyperkalemia ; Hypertrophy, Left Ventricular ; Infarction ; Myocardial Infarction ; Pericarditis ; Pulmonary Embolism

Sudden unexplained nocturnal death syndrome （SUNDS） is always a difficulty in forensic medicine researches. Although the development of molecular genetics promotes the etiologic study of SUNDS, the pathogenesis of most such cases is still unclear. Sleep apnea syndrome （SAS） is one of the common forms of sleep disorders, and obstructive sleep apnea hypopnea syndrome （OSAHS） is the most common. In recent years, some domestic and international researches show that OSAHS is related to the development of cardiovascular disease, which may cause cardiac arrhythmia, even sudden death. This article reviews the relationship between SUNDS and OSAHS and aims to provide new ideas for the pathogenesis of SUNDS.
Arrhythmias, Cardiac ; Brugada Syndrome/pathology* ; Death, Sudden/etiology* ; Humans ; Male ; Sleep Apnea, Obstructive/pathology*