Brugada Electrocardiogram Pattern Research Articles

How to demonstrate drug‐induced Type 1 Brugada pattern electrocardiogram

How to demonstrate drug‐induced Type 1 Brugada pattern electrocardiogram

Identification of a Novel SCN5A gene variant in a young female with atrioventricular canal defect in the absence of classical Brugada syndrome phenotype.

Brugada syndrome is generally considered a cardiac channelopathy disorder characterized by syncope or sudden cardiac death. The sodium voltage-gated channel alpha subunit 5 (SCN5A) gene is the most commonly mutated gene associated with Brugada syndrome. Recent discoveries of new variants of this gene, along with current guidance of family screening, have identified several asymptomatic carriers with potentially causative mutations. We present the case of a 25-year-old female patient without any family history of Brugada syndrome nor related congenital cardiovascular disorders, with an extensive atrioventricular canal defect, who tested positive for a novel heterozygous variant NM_198056.3: c.3169G>C (p. Asp1057 His) in the SCN5A gene. She had no history of syncope or aborted sudden cardiac death except for recurrent chest infections since her early childhood. Intriguingly, she did not show a type I Brugada electrocardiogram pattern. This report provides a novel heterozygous variant NM_198056.3: c.3169G>C (p. Asp1057 His) in the SCN5A gene, which may have a potential detrimental effect.

Syncope and Brugada-like ECG pattern in a patient with syndrome of inappropriate antidiuretic hormone secretion (SIADH)

Syncope is a brief loss of consciousness caused by reduced blood flow to the brain, characterised by sudden onset, short duration and full recovery without intervention. Anamnesis, physical examination and other diagnostic tests such as laboratory analysis and electrocardiogram (ECG) can be conducted to identify the underlying cause of syncope. A Brugada pattern on an ECG in individuals with syndrome of inappropriate antidiuretic hormone secretion (SIADH) who have syncope symptoms may indicate cardiac issues. A 69-year-old man with hypertension and a history of smoking presented with syncope. His vital signs were within normal limits, with no signs of a neurological deficit. The patient met the diagnostic criteria for SIADH, as evidenced by the presence of hyponatraemia (Na 118 mmol/l), a hyperosmolar condition and euvolemia. Upon arrival, a twelve-lead ECG showed ST-segment anomalies that reflected a Brugada ECG pattern. No ventricular arrhythmias were detected during the 24-hour Holter monitoring. Coronary angiography revealed no abnormalities in the coronary arteries. The ECG demonstrated the normalisation of ST elevations and the disappearance of the Brugada ECG pattern after the correction of hyponatraemia. After three months of follow-up the patient, with a normal sodium level, had no episodes of syncope.

Molecular Autopsy With Banked Cord Blood Reveals Brugada Syndrome in Past Sudden Death Case.

Molecular autopsy has recently been gaining attention as a means of postmortem diagnosis; however, it is usually performed using the victim's blood sample at the time of death. Here, we report the first case of a deceased infant with Brugada syndrome whose diagnosis was made with banked cord blood. A seemingly healthy 1-year-old male infant collapsed while having a fever; this collapse was witnessed by his mother. Despite cardiopulmonary resuscitation, he died of ventricular fibrillation. No abnormalities of cardiac structure were identified on autopsy. Genomic samples were not stored at the time because of a lack of suspicion for familial arrhythmia. Five years later, his sister showed Brugada electrocardiogram pattern while febrile from Kawasaki disease. Their father showed a spontaneous type 1 Brugada electrocardiogram pattern. A heterozygous SCN5A p.R893C variant was found by genetic testing in the proband's father and sister. Furthermore, the proband's genetic testing was performed using his banked cord blood, which identified the same variant. Family history of Brugada syndrome with an SCN5A-R893C variant and clinical evidence led to a postmortem diagnosis of Brugada syndrome in the proband. Identification of this variant in this case later contributed to verifying SCN5A-R893C as a pathogenic variant through data accumulation. Banked cord blood may prove useful for conducting molecular autopsies in previously undiagnosed cases of sudden death in which genomic samples were not stored.

Brugada syndrome: identification of subjects at risk and therapy.

Brugada syndrome mainly affects young subjects with structurally normal heart and can cause x syncope or sudden death due to ventricular arrhythmias, even as the first manifestation, in approximately 5-10% of cases. To date, two questions remain open: how to recognize subjects who will experience arrhythmic events and how to treat them. The guidelines suggest treating subjects with a previous history of cardiac arrest or arrhythmogenic syncope, while they are unconclusive about the management of asymptomatic patients, who represent ∼90% of Brugada patients. We recently demonstrated that in asymptomatic patients, the presence of spontaneous Brugada type 1 electrocardiogram (ECG) pattern and inducibility of ventricular arrhythmias at electrophysiological study allows us to identify a group of patients at greater risk who deserve treatment. Regarding treatment, there are three options: implantable cardioverter defibrillator, drugs, and epicardial transcatheter ablation. Recent studies have shown that the latter is effective and free from serious side effects, thus opening a new scenario in the treatment of Brugada patients at risk. Subjects who present drug-induced-only type 1 Brugada ECG pattern, in whom a spontaneous type 1 pattern has been ruled out with repeated ECGs and 12-lead 24-h Holter monitoring, represent a very low-risk group, provided they adhere to behavioural recommendations and undergo regular follow-up.

Brugada syndrome in Iran: Insights from a 12-year longitudinal study.

Brugada syndrome (BrS) is characterized by ST-segment elevation in the right precordial leads, which is not explained by ischemia, electrolyte disturbances, or obvious structural heart disease. In present study, we aim to evaluate presentation, long-term outcome, genetic findings, and therapeutic interventions in patients with BrS. Between September 2001 and June 2022, all consecutive patients with diagnosis of BrS were enrolled in the present study. All patients gave written informed consent for the procedure, and the local ethical committee approved the study. Of the 76 cases, 79% were proband and 21% were detected during screening after diagnosis of BrS in a family member. Thirty-three (43%) patients had a typical spontaneous electrocardiogram (ECG) pattern. Thirty percent of the patients were symptomatic; symptomatic patients were more likely to have spontaneous type 1 Brugada ECG pattern in their ECGs (p = .01), longer PR interval (p = .03), and SCN5A mutation (p = .01) than asymptomatic patients. The mean PR interval was considerably longer in men than women (p = .034). SCN5A mutation was found in 9 out of 50 (18%) studied patients. Fifteen percent received appropriate implantable cardioverter-defibrillator (ICD) therapy and inappropriate ICD interventions were observed in 17%. Presentation with aborted SCD or arrhythmic syncope was the only predictor of adverse outcome in follow-up (odds ratio: 3.1, 95% confidence interval: 0.7-19.6, p = .001). Symptomatic patients with BrS are more likely to present with spontaneous type 1 Brugada ECG pattern, longer PR interval, and pathogenic mutation in SCN5A gene. Appropriate ICD interventions are more likely in symptomatic patients and those with SCN5A mutation.

Clinical Profile, Treatment Strategies, and Outcomes of Filipino Adults with Brugada Pattern Electrocardiogram and Syndrome in a Tertiary Care Hospital in Manila, Philippines

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. METHODOLOGY: 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. RESULTS: 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. CONCLUSION: 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. KEYWORDS: Brugada pattern ECG, Brugada syndrome

Evaluation of a new treadmill exercise protocol to unmask type 1 Brugada electrocardiographic pattern: can we improve diagnostic yield?

High precordial leads (HPL) on the resting electrocardiogram (ECG) are widely used to improve diagnostic detection of type 1 Brugada ECG pattern (Br1ECGp). A parasympathetic activation marks the initial recovery phase of treadmill stress testing (TET), and this can be useful for detecting the typical ECG pattern. Our study aimed to evaluate the role of a new HPL-treadmill exercise testing (TET) protocol in detecting Br1ECGp fluctuation compared to resting HPL-ECG. 74 out of 163 patients of a Brugada syndrome (BrS) Brazilian cohort (GenBra Registry) underwent exercise testing with HPL-TET protocol. Precordial leads were displayed in strategic positions in the right and left parasternal spaces. The step-by-step analysis included ECG classification (as presence or absence of Br1ECGp) in standard vs. HPL leads placement in the following sequences: resting phase, maximal exercise, and the passive recovery phase (including 'quick lay down'). For heart rate recovery (HRR) measurements and comparisons, a Student's t-test was applied. McNemar tests compared the detection of Br1ECGp. The significance level was defined as P < 0.05. Fifty-seven patients (57/74; 77%) were male, the mean age was 49.0 ± 14, 78.4% had spontaneous BrS, and the mean Shanghai score was 4.5. The HPL-TET protocol increased Br1ECGp detection by 32.4% against resting HPL-ECG (52.7% vs. 20.3%, P = 0.001) alone. Stress testing using HPL with the passive recovery phase in the supine position offers an opportunity to unmask the type 1 Br1ECGp, which could increase the diagnostic yield in this population.

Electrocardiogram Changes in the Postictal Phase of Epileptic Seizure: Results from a Prospective Study.

The brain and heart are strictly linked and the electrical physiologies of these organs share common pathways and genes. Epilepsy patients have a higher prevalence of electrocardiogram (ECG) abnormalities compared to healthy people. Furthermore, the relationship between epilepsy, genetic arrhythmic diseases and sudden death is well known. The association between epilepsy and myocardial channelopathies, although already proposed, has not yet been fully demonstrated. The aim of this prospective observational study is to assess the role of the ECG after a seizure. From September 2018 to August 2019, all patients admitted to the emergency department of San Raffaele Hospital with a seizure were enrolled in the study; for each patient, neurological, cardiological and ECG data were collected. The ECG was performed at the time of the admission (post-ictal ECG) and 48 h later (basal ECG) and analyzed by two blinded expert cardiologists looking for abnormalities known to indicate channelopathies or arrhythmic cardiomyopathies. In all patients with abnormal post-ictal ECG, next generation sequencing (NGS) analysis was performed. One hundred and seventeen patients were enrolled (females: 45, median age: 48 ± 12 years). There were 52 abnormal post-ictal ECGs and 28 abnormal basal ECGs. All patients with an abnormal basal ECG also had an abnormal post-ictal ECG. In abnormal post-ictal ECG, a Brugada ECG pattern (BEP) was found in eight patients (of which two had BEP type I) and confirmed in two basal ECGs (of which zero had BEP type I). An abnormal QTc interval was identified in 20 patients (17%), an early repolarization pattern was found in 4 patients (3%) and right precordial abnormalities were found in 5 patients (4%). Any kind modification of post-ictal ECG was significantly more pronounced in comparison with an ECG recorded far from the seizure (p = 0.003). A 10:1 higher prevalence of a BEP of any type (particularly in post-ictal ECG, p = 0.04) was found in our population compared to general population. In three patients with post-ictal ECG alterations diagnostic for myocardial channelopathy (BrS and ERP), not confirmed at basal ECG, a pathogenic gene variant was identified (KCNJ8, PKP2 and TRMP4). The 12-lead ECG after an epileptic seizure may show disease-related alterations otherwise concealed in a population at a higher incidence of sudden death and channelopathies. Post-ictal BEP incidence was higher in cases of nocturnal seizure.

Brugada Phenocopy That Lurks After Hemodialysis: A Case Report

Brugada phenocopies (BrP) are clinical commodities that are etiologically distinguishable from genuine congenital Brugada syndrome. BrP is marked by type-1 or type-2 Brugada electrocardiogram (ECG) patterns in precordial V1 to V3, which is provoked by various underlying clinical disorders. Hemodialysis may improve the BrP ECG image. This case report describes the occurrence of a transient Brugada pattern that was seen on the ECG of an asymptomatic 64-years-old woman after administering hemodialysis due to renal failure. Diagnosis of Brugada phenocopy and BrS is necessary to achieve appropriate further treatment. Hemodialysis may improve the BrP ECG image.

COVID-19 associated Brugada pattern electrocardiogram: systematic review of case reports

Abstract Objective To summarize published case reports of patients diagnosed with COVID-19 infection and Brugada pattern electrocardiogram (ECG). Background Fever is a common clinical manifestation of COVID-19 infection. Fever has also been associated with unmasking Brugada pattern ECG in patients and may result in life-threatening arrhythmia. Little is known regarding COVID-19 associated Brugada pattern ECG. There is paucity of data and guidance in how to manage these patients. Methods To identify all published case reports, the latest Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist was followed. A literature search was conducted using PubMed, EMBASE, and Scopus through September 2021. A systematic review was performed to identify the incidence, clinical characteristics, and management outcomes of COVID-19 patients with a Brugada pattern ECG. Results A total of 18 cases were collected. The mean age was 47.1 years and 11.1% were women. No patient had prior confirmed diagnosis of Brugada syndrome. The most common presenting clinical symptoms were fever (83.3%), chest pain (38.8%), shortness of breath (38.8%), and syncope (16.6%). All 18 patients presented with type 1 Brugada pattern ECG. Four patients (22.2%) underwent left heart catheterization, and none demonstrated the presence of obstructive coronary disease. The most common reported therapies included antipyretics (55.5%), hydroxychloroquine (27.7%), and antibiotics (16.6%). One patient (5.5%) died during hospitalization. Three patients (16.6%) who presented with syncope received either an implantable cardioverter defibrillator or wearable cardioverter defibrillator at discharge. At follow up, thirteen patients (72.2%) had resolution of type 1 Brugada pattern ECG. Conclusion COVID-19 associated Brugada pattern ECG is rare. Most patients may see resolution of the ECG pattern once their symptoms have improved. Increased awareness and timely use of antipyretics is warranted in this population. Funding Acknowledgement Type of funding sources: None.

Fever following Covid-19 vaccination in subjects with Brugada syndrome: Incidence and management.

BackgroundFever is a potential side effect of the Covid‐19 vaccination. Patients with Brugada syndrome (BrS) have an increased risk of life‐threatening arrhythmias when experiencing fever. Prompt treatment with antipyretic drugs is suggested in these patients.Aim of the studyTo evaluate the incidence and management of fever within 48 h from Covid‐19 vaccination among BrS patients.MethodsOne hundred sixty‐three consecutive patients were enrolled in a prospective registry involving five European hospitals with a dedicated inherited disease ambulatory.ResultsThe mean age was 50 ± 14 years and 121 (75%) patients were male. Prevalence of Brugada electrocardiogram (ECG) pattern type‐1, ‐2, and ‐3 was 32%, 44%, and 24%, respectively. Twenty‐eight (17%) patients had an implantable cardioverter‐defibrillator (ICD). Fever occurred in 32 (19%) BrS patients after 16 ± 10 h from vaccination, with a peak of body temperature of 37.9° ± 0.5°. Patients with fever were younger (39 ± 13 vs. 48 ± 13 years, p = .04). No additional differences in terms of sex and cardiovascular risk factors were found between patients with fever and not. Twenty‐seven (84%) out of 32 patients experienced mild fever and five (16%) moderate fever. Pharmacological treatment with antipyretic drugs was required in 18 (56%) out of 32 patients and was associated with the resolution of symptoms. No patient required hospital admission and no arrhythmic episode was recorded in patients with ICD within 48 h after vaccination. No induced type 1 BrS ECG pattern and new ECG features were found among patients with moderate fever.ConclusionFever is a common side effect in BrS patients after the Covid‐19 vaccination. Careful evaluation of body temperature and prompt treatment with antipyretic drugs may be needed.

Recording of Brugada electrocardiogram pattern by an implantable cardiac monitor

A 51-year-old man with Brugada syndrome (BrS) electrocardiogram (ECG) type I pattern underwent implantable cardiac monitor (ICM) insertion. After pre-insertion potential mapping, we could observe the patient-specific repolarization abnormalities on the subcutaneous ECG provided by the ICM. A few weeks later, we received remotely a device recording with a higher ST-segment elevation and a longer duration of the interval between the onset of the coved elevation and its termination at the isoelectric line.Our observation supports the conceptual premise that ICM could add information on quantifying the amount of time with abnormal ECG patterns rather than only for the diagnosis of cardiac arrhythmias.

Contribution of the vectorcardiogram in the differential diagnosis of Brugada electrocardiographic pattern

BackgroundThe electrocardiogram (ECG) is a powerful tool for differential diagnosis among a group of pathologies with different therapeutic approaches/prognoses, the so-called J-wave syndrome. The vectorcardiogram (VCG) can be used as a complementary method to the ECG in several dubious electrocardiographic alterations. ObjectiveWe carried out a VCG analysis after conceiving and measuring a novel parameter (JT-distance) that allows diagnosis of the Brugada ECG pattern. MethodsA retrospective cohort study selected ninety-six ECGs with J-point elevation in V1/V2, ECG superior leads and VCGs, all performed on the same day. A new VCG measurement by Frank method (JT-distance) was conceived and designed in transverse and right sagittal planes by 3 lines drawn 1) at the final third of the QRS loop, comprehending the J-point; 2) at the initial portion of the T loop; 3) a parallel of the J-point line at the beginning of the T loop. JT measure was determined by the distance between parallels. A validation cohort was established in a new sample of thirty-five patients. ResultsJT-distance ≥1.5 mm (tranverse plane) and JT-distance >1.25 mm, in the sagittal plane, differentiated Brugada type-1 from Brugada type-2, early repolarization and others, with 95% sensitivity and 68% specificity. JT-distance <1.5 mm (transverse plane) and JT >1.25 mm (sagittal plane) had 100% sensitivity and 85% specificity for Brugada type-1 diagnosis. A validation cohort showed very similar Cohen's kappa levels (0.65 and 0.77, test and validation cohorts, respectively), with overlapping 95% confidence intervals. ConclusionsThe novel vectorcardiogram measurement (JT-distance) presented a new diagnostic criterion to identify Brugada pattern. Nevertheless, prospective studies should be performed by other centers to confirm these findings.

Long-Term Prognosis of Febrile Individuals with Right Precordial Coved-Type ST-Segment Elevation Brugada Pattern: A 10-Year Prospective Follow-Up Study.

A febrile state may provoke a Brugada electrocardiogram (ECG) pattern and trigger ventricular tachyarrhythmias in susceptible individuals. However, the prognostic value of fever-induced Brugada ECG pattern remains unclear. We analyzed the clinical and extended long-term follow-up data of consecutive febrile patients with a type 1 Brugada ECG presented to the emergency department. A total of 21 individuals (18 males; mean age, 43.7 ± 18.6 years at diagnosis) were divided into symptomatic (resuscitated cardiac arrest in one, syncope in two) and asymptomatic (18, 86%) groups. Sustained polymorphic ventricular tachycardias were inducible in two patients with previous syncope. All 18 asymptomatic patients had no spontaneous type 1 Brugada ECG recorded at second intercostal space and no family history of sudden death. Among asymptomatic individuals, 4 had a total 12 of repeated non-arrhythmogenic febrile episodes all with recurrent type 1 Brugada ECGs, and none had a ventricular arrhythmic event during 116 ± 19 months of follow-up. In the symptomatic group, two had defibrillator shocks for a new arrhythmic event at 31- and 49 months follow-up, respectively, and one without defibrillator therapy died suddenly at 8 months follow-up. A previous history of aborted sudden death or syncope was significantly associated with adverse outcomes in symptomatic compared with asymptomatic individuals (log-rank p < 0.0001). In conclusion, clinical presentation or history of syncope is the most important parameter in the risk stratification of febrile patients with type 1 Brugada ECG. Asymptomatic individuals with a negative family history of sudden death and without spontaneous type 1 Brugada ECG, have an exceptionally low future risk of arrhythmic events. Careful follow-up with timely and aggressive control of fever is an appropriate management option.

Prevalence and characteristics of the Brugada electrocardiogram pattern in patients with arrhythmogenic right ventricular cardiomyopathy.

BackgroundDespite distinct pathophysiology, arrhythmogenic right ventricular cardiomyopathy (ARVC) and Brugada syndrome (BrS) exhibit overlapping phenotypes. We investigated the prevalence and characteristics of the Brugada electrocardiogram (ECG) pattern in ARVC patients.MethodsA total of 114 ARVC patients fulfilling the revised Task Force Criteria were enrolled. The Brugada ECG pattern was evaluated according to the consensus report on right precordial leads, and 1141 ECGs (median, 1; interquartile range, 1‐16 ECGs/patient) were analyzed.ResultsFive patients (4%) showed a Brugada ECG pattern, which disappeared in four patients with ECGs recorded more than 2 years afterward. ARVC patients with the Brugada ECG pattern had a longer PQ interval (220 ± 62 ms vs 180 ± 35 ms, P = .02) and longer QRS duration (138 ± 25 ms vs 102 ± 23 ms, P < .001) than patients without the pattern. During follow‐up (median, 11.4; interquartile range, 5.5‐17.1 years), 19 ARVC patients experienced cardiac death and 29 experienced heart failure (HF) hospitalization. Kaplan‐Meier analysis determined that the Brugada ECG pattern increased the risk of cardiac death and HF hospitalization (log‐rank; P < .001, P < .001 respectively). The mean J‐point and S‐wave amplitudes of the Brugada ECG pattern were 0.29 ± 0.05 mV and 0.34 ± 0.21 mV, respectively, which were significantly lower than those of 26 age‐matched BrS patients with a previous ventricular fibrillation episode (0.66 ± 0.33 mV, P < .001 and 0.67 ± 0.39 mV, P = .02 respectively).ConclusionThe Brugada ECG pattern was infrequently encountered, was transient in ARVC patients, and was associated with a longer PQ interval, longer QRS duration, and cardiac events.

B-PO02-181 THE COMPARISONS OF ARTIFICIAL INTELLIGENCE AND CARDIOLOGISTS FOR THE DIAGNOSIS OF TYPE 1 BRUGADA ELECTROCARDIOGRAM PATTERN

An artificial intelligence (AI) system had been developed for the automatic diagnosis of the type 1 Brugada electrocardiogram (ECG) pattern in our previous report. To compare the accuracy of the diagnosis for the type 1 Brugada ECG pattern between the AI model and cardiologists. The 138 type 1 Brugada and 138 non-Brugada 12 lead ECGs were randomly selected from the ECG database of Taipei Veterans General Hospital. The ECG diagnoses of type 1 Brugada ECG patterns had been confirmed by two electrophysiologists as standard diagnoses based on 2017 AHA/ACC/HRS Guideline. All ECGs were blindly assigned to eight cardiologists and the AI model, respectively. The accuracy, sensitivity and specificity for the diagnosis of type I Brugada ECG pattern between AI and cardiologists were compared. The AUC curve of the ECG diagnosis of type 1 Brugada pattern was 0.96 (sensitivity: 88.4%, specificity: 89.1%). The average sensitivity of cardiologists was 62.7±17.8% while specificity was 98.5±3.0%. The prediction probability between the ECG diagnosis by the AI model and the standard diagnosis was highly consistent (Table, Kappa coefficient 0.78 and McNemar P=0.86). The predictive probability of cardiologists was only moderately consistent with the standard diagnosis. The accuracy of type 1 Brugada ECG patterns by the AI model was superior to those by the cardiologists. The cardiologists sometimes ignored the diagnosis of type 1 Brugada ECG, reflected by low sensitivity. The AI model was superior to the cardiologists for the diagnosis of type 1 Brugada ECG pattern. This implied the potential application of the AI model for early screening of Brugada syndrome.

Brugada syndrome and syncope: a practical approach for diagnosis and treatment.

Syncope in patients with Brugada electrocardiogram pattern may represent a conundrum in the decision algorithm because incidental benign forms, especially neurally mediated syncope, are very frequent in this syndrome similarly to the general population. Arrhythmic syncope in Brugada syndrome typically results from a self-terminating sustained ventricular tachycardia or paroxysmal ventricular fibrillation, potentially leading to sudden cardiac death. Distinguishing syncope due to malignant arrhythmias from a benign form is often difficult unless an electrocardiogram is recorded during the episode. We performed a review of the existing literature and propose a practical approach for diagnosis and treatment of the patients with Brugada syndrome and syncope.

Predictors of inappropriate shock in Brugada syndrome patients with a subcutaneous implantable cardiac defibrillator.

Subcutaneous implantable cardioverter defibrillators (S-ICDs) avoid complications secondary to transvenous leads, but inappropriate shocks (ISs) are frequent. Furthermore, IS data from patients with Brugada syndrome (BrS) with an S-ICD are scarce. We aimed to establish the frequency and predictors of IS in this population. We analyzed the clinical and electrocardiographic characteristics, automated screening test data, device programming, and IS occurrence in adult patients with BrS with an S-ICD. Thirty-nine patients were enrolled (69% male, mean age at diagnosis 46 ± 13 years, mean age at implantation 48 ± 13 years). During a mean follow-up of 26 ± 21 months, 18% patients experienced IS. Patients with IS were younger at the time of diagnosis (36 ± 8 vs. 48 ± 13 years, p = .018) and S-ICD implantation (38 ± 9 vs. 50 ± 23 years, p = .019) and presented with spontaneous type 1 Brugada electrocardiogram pattern more frequently at diagnosis or during follow-up (71% vs. 25%, p = .018). During automated screening tests, patients with IS showed lower QRS voltage in the primary vector in the supine position (0.58 ± 0.26 vs. 1.10 ± 0.35 mV, p = .011) and lower defibrillator automated screening score in the primary vector in the supine (123 ± 165 vs. 554 ± 390 mV, p = .005) and standing (162 ± 179 vs. 486 ± 388 mV, p = .038) positions. Age at diagnosis was the only independent predictor of IS (hazard ratio = 0.873, 95% confidence interval: 0.767-0.992, p = .037). IS was a frequent complication in patients with BrS with an S-ICD. Younger age was independently associated with IS. A more thorough screening process might help prevent IS in this population.

A case of anomalous aortic origin of coronary artery associated with a coved‐type electrocardiogram

Brugada syndrome (BrS) is a sudden cardiac death syndrome characterized by a coved-type electrocardiogram (ECG). Different disorders, such as ischemia, can emulate a Brugada-pattern ECG (Brugada phenocopy). We report herein, the first case of surgical epicardial electrophysiological mapping in a successfully resuscitated patient with an anomalous aortic origin of the coronary artery (AAOCA) associated with a coved-type ECG. It was debatable whether the coved-type ECG and the abnormal arrhythmogenic substrate in the epicardial right ventricular outflow tract were derived from BrS or from repetitive ischemia due to AAOCA; however, the epicardial electrophysiological mapping helped in deciding the treatment strategy.