scholarly journals Brugada Phenocopy Induced by a Lethal Methanol Intoxication

Author(s):  
Monterrubio-Villar Jesús ◽  
Llinares-Moya David

Brugada phenocopies (BrP) are clinical entities that present with an ECG pattern identical to either the type 1 or type 2 Brugada pattern without true congenital Brugada syndrome. This ECG pattern is associated with an identifiable condition and normalizes upon resolution or treatment of the underlying cause. We present a case of a 54-year-old man with extreme metabolic acidosis, hyperkalaemia and a Brugada type 1 ECG pattern in the setting of a suicidal methanol (MeOH) poisoning. Upon correction of these metabolic derangements with bicarbonate infusions and continuous veno-venous haemodiafiltration (CVVH), the Brugada type 1 ECG pattern normalized. Unfortunately, the patient developed signs of cerebral herniation followed by brain death and died on the first day of ICU admission.

Author(s):  
R. Singla ◽  
A. Udyavar ◽  
A. Gupta ◽  
A. Bade ◽  
K. Munde ◽  
...  

The present case series discuss three patients who had brugada type 2/ type 3 like ECG pattern that was converted to type 1 pattern with oral flecanide challenge test. Brugada syndrome is associated with a high incidence of sudden cardiac death,   typical ECG pattern being ST-segment elevation in the right precordial leads with T wave inversion. Pharmacological provocation should only be performed when the baseline ECG is not diagnostic of Brugada Syndrome. PR prolongation in the baseline ECG is also a contraindication because of the risk of inducing AV block. Drug challenge is performed under strict monitoring of BP and 12-lead ECG and facilities for cardio version and resuscitation are available. Atypical RBBB pattern/type 2/3 Brugada pattern on ECG in patients of syncope or family history of sudden cardiac arrest is commonly encountered by a cardiologist. This can be performed to provoke type 1 brugada pattern on ECG. Diagnosed cases of Brugada may be treated with ICD with proper indication if needed and thus prevent sudden cardiac death.


EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
J Brito ◽  
N Cortez-Dias ◽  
N Nunes-Ferreira ◽  
I Aguiar-Ricardo ◽  
G Silva ◽  
...  

Abstract Introduction The sudden cardiac death risk in Brugada Syndrome (BrS) is higher in patients with spontaneous type 1 pattern. Brugada diagnosis is also established in patients with induced type 1 morphology after provocative test with intravenous administration with a sodium blocker channel. Nevertheless, this group of patients is known to be at a lower risk of SCD, and their risk stratification is still a matter of discussion.  Late potentials (LP) detected on signal-averaged ECG (SAECG) on the RVOT have been previously proposed as a predictor factor for BrS, even though data is lacking on its value. Purpose To evaluate the association between positive LP (LMS40> 38ms) on SAECG with modified Brugada leads and a positive flecainide test in patients with non-type 1 BrS. Methods Retrospective single-center study of non-type 1 BrS patients referred for the performance of a flecainide provocative test. Patients presenting with spontaneous type 1 morphology were excluded from the study. Study of LP on SAECG with modified leads for Brugada were evaluated before administration of flecainide [2mg/kg (maximum150mg), for 10minutes] with determination of filtered QRS duration (fQRS), root mean square voltage of the last 40ms of the QRS complex (RMS40) and duration of low amplitude signals <40μV of the terminal QRS complex (LMS40). Results 126 patients (47.3 ± 14.1 years, 61.9% males) underwent study with LP SAECG and flecainide test. Among these patients, 7.9% were symptomatic and 16.7% had familiar history of BrS. Flecainide test was positive in 46.8% of patients. In patients with a positive flecainide test, 64.4% presented LMS40 > 38ms whereas LMS40 > 38ms was present in only 46% of those with a negative flecainide test (p = 0.031). The presence of positive LMS40 was a positive predictor for a positive flecainide test, associated with a two-fold increase likelihood in the induction of a Brugada pattern (OR: 2,12; IC95% 1,025-4,392; P = 0,043). There was no association between fQRS or RMS40 and a positive flecainide test (p = NS). fQRS > 114ms and RMS40 <20uV was present in 22% and 61% of patients with a positive flecainide test, respectively. Conclusion In patient with non-type 1 Brugada syndrome, LMS40 > 38ms in SAECG was a predictor for a positive flecainide test, suggesting that this finding could be helpful on the risk stratification of patients undergoing diagnostic study for Brugada syndrome. Abstract Figure. Effect of LMS 40 in flecainide test


Author(s):  
Anil Sarica ◽  
Serhat Bor ◽  
Mehmet Orman ◽  
Hector Barajas-Martinez ◽  
Jimmy Juang ◽  
...  

Introduction: Irritable bowel syndrome (IBS) is one of the most widely recognized functional bowel disorders (FBDs) with a genetic component. SCN5A gene and SCN1B loci have been identified in population-based IBS cohorts and proposed to have a mechanistic role in the pathophysiology of IBS. These same genes have been associated with Brugada syndrome (BrS). The present study examines the hypothesis that these two inherited syndromes are linked. Methods and Results: Prevalence of FBDs over a 12 months period were compared between probands with BrS/drug-induced type 1 Brugada pattern (DI-Type1 BrP) (n=148) and a control group (n=124) matched for age, female sex, presence of arrhythmia and co-morbid conditions. SCN5A/SCN1B genes were screened in 88 patients. Prevalence of IBS was 25% in patients with BrS/DI-Type1 BrP and 8.1% in the control group (p=2.34×10−4). On stepwise logistic regression analysis, presence of current and/or history of migraine (OR of 2.75; 95% CI: 1.08 to 6.98; p=0.033) was a predictor of underlying BrS/DI-Type1 BrP among patients with FBDs. We identified 8 putative SCN5A/SCN1B variants in 7 (12.3%) patients with BrS/DI-Type1 BrP and 1 (3.2%) patient in control group. Five out of 8 (62.5%) patients with SCN5A/SCN1B variants had FBDs. Conclusion: IBS is a common co-morbidity in patients with BrS/DI-Type1 BrP. Presence of current and/or history of migraine is a predictor of underlying BrS/DI-Type1 BrP among patients with FBDs. Frequent co-existence of IBS and BrS/DI-Type1 BrP necessitates cautious use of certain drugs among the therapeutic options for IBS that are known to exacerbate the Brugada phenotype.


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
L Barbonaglia ◽  
F De Vecchi ◽  
C Devecchi ◽  
M Matta ◽  
R Peraldo ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background. Pharmacological (Ajmaline) induction of a type 1 Brugada pattern is currently considered mandatory for the diagnosis of Brugada syndrome. However, performing the test requires time and healthcare resources. Some EKG markers have been proposed as predictors of positive result at Ajmaline test. Aim. To evaluate in a large population the predictive value of multiple EKG markers for Ajmaline test results. Methods. We retrospectively analysed consecutive patients (pts) referred to our Centre to perform Ajmaline test. All pts had type 2 Brugada pattern detected at a conventional EKG or were relatives of pts with positive Ajmaline test, with or without type 2 Brugada pattern at EKG. All pts performed the Ajmaline pharmacological test (1 mg/Kg iv) with EKG "superior" right precordial unipolar derivations monitoring. To determine whether clinical parameters (age, gender, cardiomyopathy, history of arrhythmias, symptoms, familiarity) and EKG markers (heart rate (HR), PR duration, R1V1 and SV6 duration and amplitude, QRSV1/QRSV6 duration, V1 and V2 ST amplitude (coved or saddle back pattern) were independently associated to positivity at Ajmaline test, a logistic regression model was applied. Results. From January 2010 to December 2019 we evaluated 442 consecutive pts: mean age 40.1 ± 14.5 years; 273 (65%) male; 352 (80%) pts were included because of type 2 Brugada pattern at EKG and 90 (20%) for familial screening. The Ajmaline test was positive in 150 (34%) pts. At multivariate logistic regression analysis adjusted for baseline confounders, age > 45 years (OR= 1.64, 95%CI: 1.03 to 2.54; p = 0.0385), female gender (OR = 1.79, 95%CI: 1.12 to 2.85; p = 0.0141), HR > 60 bpm (OR = 2.44, 95%CI: 1.48 to 4.03; p = 0.0005), QRSV1/QRSV6 duration (msec) >1 (OR = 5.34, 95%CI: 3.28 to 8.69; p < 0.0001) and non isoelectric pattern (coved/saddle back) in V2 (OR = 1.93, 95%CI: 1.03 to 3.63, p = 0.0416) remained associated with a positive Ajmaline test. The percentage of pts with positive Ajmaline test increased according to the presence of significant EKG markers in their risk profile: 11.3% (8 out 71, absence of both QRSV1/QRSV6 duration (msec) >1 and V2 non isoelectric pattern), 24.3% (50 out 206, presence of only V2 non isoelectric pattern), 48.5% (16 out 33, presence of only QRSV1/QRSV6 duration (msec) >1), 57.6% (76 out 132, presence of both factors). Conclusions. In our large population: 1) we confirmed the positive predictive power of QRSV1/QRSV6 duration (msec) >1 and of a non isoelectric pattern (coved/saddle back) in V2 for a pharmacologically induced type 1 Brugada pattern; 2) we observed a non-negligible percentage of pts who would not be correctly diagnosed for type 1 Brugada pattern, if selected according to an EKG parameters-based prescreening.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Sabatini

Abstract Background Brugada syndrome (BrS) risk stratification in asymptomatic subjects is still currently the most important yet unresolved clinical problem to determine the subset of patients with BrS requiring ICD implantation. The underlying pathophysiological mechanisms responsible for BrS ECG patterns remain unknown, as well as the mechanisms of the sudden onset of polymorphic ventricular tachycardia which leads to ventricular fibrillation and sudden cardiac death (SCD). Purpose This study aims to analyze from a totally alternative perspective, superficial 12-lead ECG signals. It departs from the numerous and various attempts to characterize and measure single morphology of specific and individual ECG segments, intervals and waves, rather focusing on and studying the dynamics and stability of the superficial 12-lead ECG signal as a whole to determine stability parameters able to contribute to BrS ECG pattern risk stratification and differential diagnosis of BrS. Methods A quantitative stability control closed loop system has been designed to model the electrophysiology dynamics of the cardiac conduction system with a 12-lead superficial ECG signal being the input and output of the system (Fig. 1). A normal ECG signal and a type-1 coved Brugada pattern ECG-V2 portion have been scanned, digitized and quantitatively processed to obtain stability parameters (poles and zeros in the S-plane). Scanning was performed by Digitizeit – Digital River GmbH. Processing in the S-plane was performed by ©2019 Wolfram Demonstrations Project & Contributors, http://demonstrations.wolfram.com/, poles and zeros and Microsoft Excel software was also used. Results Poles and zeros of the system for type-1 coved Brugada pattern ECG-V2 and for the normal ECG-V2 are shown in Fig. 2, together with stability. Conclusions Based on our data, 1. It appears that portions of the ECG patterns, approximated by mathematical continuous time models representing, at the infinitesimal limit, every possible pattern and behaviors of an ECG signal, such as repolarization patterns, may exhibit interesting dynamics characteristics of stability and can be stratified as stable, marginally stable or unstable. 2. Such a classification may then be implemented to risk stratify repolarization patterns. When tending to instability, such patterns seem to be associated to high risk repolarization patterns such as BrS coved type-1 pattern, hence indicating higher risk of developing polymorphic VT or SCD. In conclusion, more work will be needed to further this methodology to improve the understanding of the effects of the various physiological and pathological substrates involved with malignant arrhythmias and to improve risk stratification strategies to determine the subset of patients with Brugada syndrome requiring ICD insertion. Control systems and stability theory may indeed indicate an interesting and effective procedure for future work.


2021 ◽  
pp. 1-3
Author(s):  
Tomoya Tsuchihashi ◽  
Masahiro Kamada ◽  
Yukiko Nakano

Abstract We report a 25-year-old woman who was diagnosed with atrial septal defect (ASD). An ECG showed only first-degree atrioventricular block and incomplete right bundle branch block. One day after the percutaneous ASD closure, she had a slight fever and an ECG showed a type 1 Brugada pattern. ECG characteristics of ASD are similar to those of a Brugada ECG. This case is rare combination of Brugada syndrome with ASD.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Shepard D Weiner ◽  
Kirsten O Healy ◽  
James A Reiffel ◽  
James Coromilas

INTRODUCTION: Many precipitants of the Brugada pattern ECG have been identified, including fever. However, the association of fever with Brugada pattern type is unknown. We assessed the hypothesis that the Brugada pattern ECGs that occur in the setting of a febrile episode are related to the Brugada pattern type. METHODS: This retrospective study examined all ECGs performed at Columbia University Medical Center from October 2004 to March 2007. The ECGs identified as showing possible Brugada pattern were reviewed and classified according to the ECG criteria endorsed by the Heart Rhythm Society and European Heart Rhythm Association. The medical record was then investigated for documentation of fever, defined as a temperature greater than or equal to 38.0 degrees Celsius. The primary outcome was the presence of fever within 48 hours of the Brugada pattern ECG. RESULTS: Forty Brugada pattern ECGs were identified (21 type 1 and 19 type 2). Background characteristics of the patients according to Brugada pattern type are shown in the Table . Eleven patients (27.5%) had fever (median temperature 38.4; temperature range 38.0 – 40.1) and Brugada pattern ECGs. There was a statistically significant association of Brugada pattern type 1 (nine patients) and fever compared to Brugada pattern type 2 (two patients) and fever (p=0.031; OR 6.96, 95% CI 1.26 – 38.44). Seven of nine patients (77.7%) with fever and Brugada pattern type 1 had a normal ECG when afebrile and one of two patients (50.0%) with fever and Brugada pattern type 2 had a normal ECG when afebrile. CONCLUSIONS: A significant number of patients with Brugada pattern type 1 had this ECG finding in the context of fever. The majority of these patients had a normal ECG when afebrile. Further investigation is needed to assess the biologic plausibility of this finding as well as the relationship of Brugada pattern type 1 unmasked by fever in asymptomatic patients to the clinical features of Brugada syndrome. Background Characteristics by Brugada Pattern Type


2020 ◽  
Vol 31 (12) ◽  
pp. 3311-3317
Author(s):  
Can Hasdemir ◽  
Figen Gokcay ◽  
Mehmet N. Orman ◽  
Umut Kocabas ◽  
Serdar Payzin ◽  
...  

2019 ◽  
Vol 76 (23) ◽  
pp. 1930-1933 ◽  
Author(s):  
Naseer Khan ◽  
Katie Tso ◽  
Joan Broussard ◽  
Monica Dziuba

Abstract Purpose Canagliflozin is a sodium-glucose cotransporter-2 (SGLT2) inhibitor which received U.S. Food and Drug Administration approval in 2013 for the treatment of type 2 diabetes mellitus. Fanconi syndrome is a rare acquired disorder which typically occurs in adults as an adverse effect of medications. The literature includes few case reports of Fanconi syndrome caused by the use of canagliflozin. Here, we present a case of Fanconi syndrome in a patient with type 1 diabetes previously miscategorized as type 2 diabetes. Summary A 32-year-old woman with a 6-year history of type 2 diabetes was started on canagliflozin. Within 2 months of therapy initiation, she began to develop symptoms of high anion gap metabolic acidosis. Further laboratory test results showed severe life-threatening hypophosphatemia. Further investigation by nephrology revealed the presence of Fanconi syndrome. During the admission, she was found to have clinical and laboratory features of type 1 (insulin-dependent) diabetes. After discontinuation of canagliflozin, she was treated with intravenous (i.v.) fluids for hydration, subcutaneous insulin, and i.v. potassium phosphate. She recovered from all metabolic acidosis and electrolyte abnormalities. Conclusion Fanconi syndrome is a rare, exogenously acquired disorder in adults that often develops as an adverse effect of medication therapy. Our patient presented with Fanconi syndrome as a complication of canagliflozin use for the treatment of presumed type 2 diabetes. She was then started on subcutaneous insulin monotherapy for the treatment of type 1 diabetes mellitus.


2019 ◽  
Vol 2019 ◽  
pp. 1-5 ◽  
Author(s):  
Michel Ibrahim ◽  
Garly Saint-Croix ◽  
Rosario Colombo

Only one case report has previously described a patient with multiple sclerosis and a type 1 Brugada pattern on the electrocardiogram. Patients with multiple sclerosis have several neurological deficits including sensory symptoms, acute or subacute motor weakness, gait disturbance, and balance problems that may lead to an increased risk of falls. Concurrent autonomic dysfunction and neurologic consequences of multiple sclerosis may precipitate both mechanical falls and falls with loss of consciousness. While mechanistically different, the type 1 Brugada pattern presents similarly with syncope due to an insufficient cardiac output during dysrhythmia. In such patients, intracardiac defibrillators have shown to prevent sudden cardiac death in patients with the Brugada syndrome. In light of these similarly presenting but unique clinical entities, MS patients who develop a syncopal event in the setting of a spontaneous type I Brugada pattern pose a diagnostic and therapeutic dilemma. This case illustrates an approach to the risks and benefits of an ICD placement in an MS patient with the type 1 Brugada pattern.


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