Abstract 13486: Risk Stratification of Ventricular Fibrillation in Brugada Syndrome Using Non-invasive Scoring Methods

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Hiroshi Kawazoe ◽  
Yukiko Nakano ◽  
Takehito Tokuyama ◽  
Yuko Uchimura ◽  
Yoshikazu Watanabe ◽  
...  

Aim: Risk stratification of ventricular fibrillation (VF) in Brugada syndrome remains controversial and it is difficult to describe using one parameter. We investigated whether we are able to assess VF risk using our non-invasive scoring method of each Brugada syndrome patients. Methods: We enrolled 46 patients with Brugada syndrome (45 men, average age 42 ± 3 years). Thirteen of the subjects had VF history or subsequent VF events (VF+) after implantable cardioverter defibrillator implantation. The frequency of Type1 Brugada ECG and positive TWA were significantly higher in VF+ patients than those without (Type 1 ECG 50% vs 4.8%, P=0.0003 and positive TWA 73.3% vs 6.7%, P<0.0001). We allocated each one point to type 1 Brugada type ECG, family history of sudden cardiac death, history of syncope, positive late potential, and positive time domain T wave alternance as Brugada risk score and count up the score the points in an each subject. The area under the receiver-operator characteristic (ROC) curve for ≥3 points was 0.907 indicating that this Brugada risk score ≥3 points had a high degree of discrimination VF+ Brugada syndrome patients. The positive Brugada risk score was associated with VF history or events with a sensitivity of 85%, specificity of 88%, positive predictive value 73% and negative predictive value 93%. Conclusions: The new scoring method using previous reported non-invasive risk factors of VF in Brugada syndrome is useful for assessing risk stratification in daily medical practice.

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
S Iacopino ◽  
P Sorrenti ◽  
G Fabiano ◽  
G Campagna ◽  
A Petretta ◽  
...  

Abstract Introduction - No study has been performed to investigate the role of drug-induced ECG morphology modifications as potential risk factors for the development of malignant arrhythmias in patients with Brugada syndrome. Purpose - The aim of this study is to introduce a new index to improve asymptomatic patient stratification  and to report the first case of a patient with Brugada syndrome undergoing ajmaline testing that has been evaluated using a diagnostic 252-lead ECG vest. Methods - From December 2018 to April 2019, 26 consecutive patients [mean age 39.9 (30–59) years, 18 male] with no cardiovascular risk factors underwent ajmaline testing. By evaluating ECG recordings after ajmaline administration, we calculated an index that we called "dST-Tiso", that is the duration of the positive component of the ST-T wave to the isoelectric line, in V1 and/or V2. Results- Out of 26 patients, 16 (61.5%) had a positive test, with type 1 (coved-type) ECG diagnostic pattern in leads V1-V2 from the 2nd, 3rd and 4th intercostal spaces.  The mean recorded dST-Tiso value was 239 ± 76 ms. The ECG showed T-wave above the isoelectric line in 5 patients with a significantly higher dST-Tiso value (on average 360 ± 56 ms), and biphasic T-waves below the isoelectric line in 11 patients with a dST-Tiso value of 209 ± 42 ms (Mann-Whitney, p = 0.039). All patients with positive ajmaline test underwent programmed electrical stimulation (PES). Ventricular fibrillation was induced during PES in all 5 patients with stretched dST-Tiso. In the remaining 11 patients without stretched dST-Tiso, no ventricular arrhythmia was induced by PES.  Fig 1 Moreover, using non-invasive high-density electrocardiographic mapping (252-lead ECG vest), 3 patients with dST-Tiso positive pattern received a second ajmaline protocol, with assessment of both the depolarization and repolarization phases. Conclusion - The ECG pattern of prolonged dST-Tiso seems to have a significant impact on safety during PES and may have potential for stratifying risk of sudden death in patients with PES-induced ventricular tachycardia/fibrillation. Abstract Figure 1. Patients’ flowchart.


Author(s):  
Giuseppe Ciconte ◽  
Vincenzo Santinelli ◽  
Gabriele Vicedomini ◽  
Valeria Borrelli ◽  
Michelle M Monasky ◽  
...  

Abstract Aims Brugada syndrome (BrS) represents a major cause of sudden cardiac death in young individuals. The risk stratification to forecast future life-threatening events is still controversial. Non-invasive assessment of late potentials (LPs) has been proposed as a risk stratification tool. However, their nature in BrS is still undetermined. The purpose of this study is to assess the electrophysiological determinants of non-invasive LPs. Methods and Results Two hundred and fifty consecutive patients with (Group 1, n = 96) and without (Group 2, n = 154) BrS-related symptoms were prospectively enrolled in the registry. Signal-averaged electrocardiogram (SAECG) was performed in all subjects before undergoing epicardial mapping. Group 1 patients exhibited larger arrhythmogenic substrates (AS; 5.8 ± 2.8 vs. 2.6 ± 2.1 cm2, P < 0.001) with more delayed potentials (220.4 ± 46.0 vs. 186.7 ± 42.3 ms, P < 0.001). Late potentials were present in 82/96 (85.4%) Group 1 and in 31/154 (20.1%) Group 2 individuals (P < 0.001). Patients exhibiting LPs had more frequently a spontaneous Type 1 pattern (30.1% vs. 10.9%, P < 0.001), SCN5A mutation (34.5% vs. 21.2%, P = 0.02), and exhibited a larger AS with longer potentials (5.8 ± 2.7 vs. 2.2 ± 1.7 cm2; 231.2 ± 37.3 vs. 213.8 ± 39.0 ms; P < 0.001, respectively). Arrhythmogenic substrate dimension was the strongest predictor of the presence of LPs (odds ratio 1.9; P < 0.001). An AS area of at least 3.5 cm2 identified patients with LPs (area under the curve 0.88, 95% confidence interval 0.843–0.931; P < 0.001) with a sensitivity of 86%, specificity 88%, positive predictive value 85%, and negative predictive value 89%. Conclusion The results of this study support the role of the epicardial AS as an electrophysiological determinant of non-invasive LPs, which may serve as a tool in the non-invasive assessment of the BrS substrate, as SAECG-LPs could be considered an expression of the abnormal epicardial electrical activity. ClinicalTrials.gov number (NCT02641431; NCT03106701).


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Michael Papadakis ◽  
Carey Edwards ◽  
John C Rawlins ◽  
Sanjay Sharma

Risk stratification for sudden death in Brugada syndrome and hence indications for implantation of an internal cardioverter defibrillator are based on the presence of a spontaneous type 1 electrocardiogram (ECG) pattern, in association with unheralded syncope or documented polymorphic ventricular tachycardia. Increasingly, awareness of sudden death in the young and implementation of pre-participation screening programs in sport will identify young, asymptomatic patients with the Brugada phenotype. We evaluated the predictive accuracy of symptoms, particularly unheralded syncope in victims of sudden death from Brugada syndrome. Over the past 3 years we identified 22 victims of sudden cardiac death secondary to Brugada syndrome. The diagnosis was based on sudden death with normal findings at post mortem and the identification of the type 1 Brugada ECG pattern in first-degree relatives (spontaneous or following ajmaline provocation test). All relatives underwent 12-lead ECG, echocardiography, exercise testing, 24-hour Holter monitor and biochemical tests. Cardiac magnetic resonance imaging and coronary angiography were performed when appropriate. Of the victims dying from Brugada syndrome, 15 (68%) were male. The mean age of sudden death was 30 years (range 8 –56 years) and 95% died suddenly at rest or during sleep. We interviewed first-degree relatives and partners regarding prodromal symptoms or a history of epilepsy. Only 3 out of 22 victims (14%) had significant symptoms. Specifically, 2 individuals experienced unheralded syncope and 1 suffered nocturnal seizures. Only 14% had a family history of premature sudden cardiac death. Indeed we obtained 12-lead ECGs in 3 victims taken less than 6 months prior to sudden death, which failed to reveal the typical Brugada ECG phenotype. Our results indicate that the vast majority of individuals who die from Brugada syndrome in the UK are asymptomatic and raise concerns that the absence of symptoms does not necessarily mean low risk. Better understanding of the disorder and risk stratification protocols is necessary to permit genuine reassurance in asymptomatic individuals exhibiting the Brugada phenotype.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Honarbakhsh ◽  
R Providencia ◽  
J Garcia-Hernandez ◽  
C A Martin ◽  
R J Hunter ◽  
...  

Abstract Background Risk stratification for sudden cardiac death (SCD) in Brugada syndrome (BrS) is a significant challenge. Purpose To evaluate the importance of clinical and ECG factors in the likelihood of developing significant ventricular arrhythmias (VAs)/SCD in BrS patients. Methods VA occurrence during follow-up were assessed and the role of 16 proposed clinical or ECG risk markers evaluated in a multicenter international study of BrS patients and no history of cardiac arrest. Markers with predictive power were identified and incorporated into a risk score model. Results Across 15 international centers, 1084 patients were included. During a follow-up of 5.3 years (IQR 2.7–9.0 years)- 110 patients had VA occurrence (10.1%) with an annual event rate of 1.7% (95% CI 1.4–2.0). Of the 16 proposed risk factors, diagnosis by family screening of sudden cardiac death (HR 4.65; p<0.001), probable arrhythmia related syncope (HR 3.88, p<0.001), type 1 spontaneous ECG (HR 3.56; p<0.001), Early Repolarisation (HR 3.15; p<0.001) and type 1 Brugada pattern in peripheral leads (HR 2.42; p<0.001) were associated with a higher VA occurrence risk during follow-up. These 5 variables were incorporated into a risk score model whereby each variable was allocated a point score based on the variable's predictive strength. The total points obtained from the model for a patient could then be translated into the predicted VA occurrence risk during follow-up (Figure 1). The model showed a sensitivity of 63.5% (95% CI 50.0–76.9) and specificity of 84.2% (95% CI 81.1–87.1) in predicting VA occurrence at 5-years follow-up. The model showed a greater discriminative power compared to an existing model (AUC 0.83 vs. 0.71; p<0.001). Figure 1 Conclusions This multicenter study with longest reported follow-up to date identified 5 risk markers for VA occurrence. Utilizing these markers in a risk score model can aid BrS risk stratification to enable individualized risk prediction and ICD prescription.


2019 ◽  
Vol 40 (2) ◽  
Author(s):  
Ardian Rizal ◽  
Sunu Budhi Raharjo ◽  
Dicky Armein Hanafy ◽  
Yoga Yuniadi

Background : Brugada syndrome is an inherited disease characterized by an increased risk of sudden cardiac death owing to ventricular arrhythmias in the absence of structural heart disease. It has been reported that this syndrome is more prevalent in South-East Asia than in Western countries. Furthermore, genetic studies showed important contributions of several gene mutations to the phenotype of BrS. These suggest that ethnic difference play significant roles in the pathogenesis of BrS. In addition, ICD implantation remains the cornerstone management with a low rate of appropriate shocked. Therefore, it is important to investigate patients’ characteristics for risk stratification. Our objective to investigate the clinical, electrocardiography (ECG) and electrophysiological characteristics that can be used as predictor of appropriate shock due to ventricular arrhythmia (VA) in Indonesian patients with BrS. Methods : We analyse data from Brugada syndrome registry at National Cardiovascular Centre Harapan Kita since January 2013. Total 22 patients were included. Characteristics of BrS that we analysed were baseline characteristics (age and sex), Clinical finding (syncope, cardiac arrest), ECG finding (spontaneous type 1 or drug induced) and Electrophysiology study result (inducible VA and RV ERP). We also added some new ECG characteristic (S wave in lead 1, S wave duration in V1, Fragmented QRS, Junction ST elevation and early repolarization pattern in infero-lateral) to  be analysed. Our end point are appropriate shock during ICD interrogation for those who have been implanted an ICD, and documented VA for those who didn’t receive ICD. Result : We found high incidence of  appropriate ICD’s shock in our population (50% in our study vs 5-11.5% in real world). Predictors of appropriate shock and documented VA are history of syncope (p = 0.045; OR 2.57 [1.44-4.59]), spontaneous type-1 ECG (p = 0.005) and right ventricular effective refractory period (RV ERP) of <200 ms (p=0.018). Other parameters that have been reported to correlate with the occurrence of VA (S Wave in lead 1 (p = 0.530), early repolarization pattern (p = 0.578), fragmented QRS (p = 0.601), S Wave duration (p = 0.365) and J Point STE (p = 0.800) were found to be not correlated to appropriate shock in our populations. Conclusion : History of syncope, spontaneous type-1 Brugada ECG and RV ERP of <200 ms have predictive values for risk stratification of Indonesian patients with Brugada syndrome.    Keywords : Brugada Syndrome, Ventricular arrhythmia, ICD shock       


2021 ◽  
Vol 22 (9) ◽  
pp. 4700
Author(s):  
Michelle M. Monasky ◽  
Emanuele Micaglio ◽  
Giuseppe Ciconte ◽  
Ilaria Rivolta ◽  
Valeria Borrelli ◽  
...  

Genetic testing in Brugada syndrome (BrS) is still not considered to be useful for clinical management of patients in the majority of cases, due to the current lack of understanding about the effect of specific variants. Additionally, family history of sudden death is generally not considered useful for arrhythmic risk stratification. We sought to demonstrate the usefulness of genetic testing and family history in diagnosis and risk stratification. The family history was collected for a proband who presented with a personal history of aborted cardiac arrest and in whom a novel variant in the SCN5A gene was found. Living family members underwent ajmaline testing, electrophysiological study, and genetic testing to determine genotype-phenotype segregation, if any. Patch-clamp experiments on transfected human embryonic kidney 293 cells enabled the functional characterization of the SCN5A novel variant in vitro. In this study, we provide crucial human data on the novel heterozygous variant NM_198056.2:c.5000T>A (p.Val1667Asp) in the SCN5A gene, and demonstrate its segregation with a severe form of BrS and multiple sudden deaths. Functional data revealed a loss of function of the protein affected by the variant. These results provide the first disease association with this variant and demonstrate the usefulness of genetic testing for diagnosis and risk stratification in certain patients. This study also demonstrates the usefulness of collecting the family history, which can assist in understanding the severity of the disease in certain situations and confirm the importance of the functional studies to distinguish between pathogenic mutations and harmless genetic variants.


2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Huiying Wu ◽  
Ning Zhou ◽  
Lianwei Lu ◽  
Xiwen Chen ◽  
Tao Liu ◽  
...  

Abstract Background Extrahepatic portal vein obstruction (EHPVO) is the most important cause of hematemesis in children. Intrahepatic left portal vein and superior mesenteric vein anastomosis, also known as meso-Rex bypass (MRB), is becoming the gold standard treatment for EHPVO. We analyzed the value of preoperative computed tomography (CT) in determining whether MRB is feasible in children with EHPVO. Results We retrieved data on 76 children with EHPVO (50 male, 26 female; median age, 5.9 years) who underwent MRB (n = 68) or the Warren procedure (n = 8) from 2013 to 2019 and retrospectively analyzed their clinical and CT characteristics. The Rex recess was categorized into four subtypes (types 1–4) depending on its diameter in CT images. Of all 76 children, 7.9% had a history of umbilical catheterization and 1.3% had leukemia. Sixteen patients (20 lesions) had associated malformations. A total of 72.4% of Rex recesses could be measured by CT, and their mean diameter was 3.5 ± 1.8 mm (range 0.6–10.5 mm). A type 1, 2, 3, and 4 Rex recess was present in 9.2%, 53.9%, 11.8%, and 25.0% of patients, respectively. MRB could be performed in patients with types 1, 2, and 3, but those with type 4 required further evaluation. The sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of CT were 100%, 83.8%, 42.1%, 100%, and 85.5%, respectively. Conclusions Among the four types of Rex recesses on CT angiography, types 1–3 allow for the performance of MRB.


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&gt; 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 &lt;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 &gt; 38ms whereas LMS40 &gt; 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 &gt; 114ms and RMS40 &lt;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 &gt; 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):  
Gary Tse ◽  
Jiandong Zhou ◽  
Sharen Lee ◽  
Tong Liu ◽  
George Bazoukis ◽  
...  

Background A combination of clinical and electrocardiographic risk factors is used for risk stratification in Brugada syndrome. In this study, we tested the hypothesis that the incorporation of latent variables between variables using nonnegative matrix factorization can improve risk stratification compared with logistic regression. Methods and Results This was a retrospective cohort study of patients presented with Brugada electrocardiographic patterns between 2000 and 2016 from Hong Kong, China. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation. The external validation cohort included patients from 3 countries. A total of 149 patients with Brugada syndrome (84% males, median age of presentation 50 [38–61] years) were included. Compared with the nonarrhythmic group (n=117, 79%), the spontaneous ventricular tachycardia/ ventricular fibrillation group (n=32, 21%) were more likely to suffer from syncope (69% versus 37%, P =0.001) and atrial fibrillation (16% versus 4%, P =0.023) as well as displayed longer QTc intervals (424 [399–449] versus 408 [386–425]; P =0.020). No difference in QRS interval was observed (108 [98–114] versus 102 [95–110], P =0.104). Logistic regression found that syncope (odds ratio, 3.79; 95% CI, 1.64–8.74; P =0.002), atrial fibrillation (odds ratio, 4.15; 95% CI, 1.12–15.36; P =0.033), QRS duration (odds ratio, 1.03; 95% CI, 1.002–1.06; P =0.037) and QTc interval (odds ratio, 1.02; 95% CI, 1.01–1.03; P =0.009) were significant predictors of spontaneous ventricular tachycardia/ventricular fibrillation. Increasing the number of latent variables of these electrocardiographic indices incorporated from n=0 (logistic regression) to n=6 by nonnegative matrix factorization improved the area under the curve of the receiving operating characteristics curve from 0.71 to 0.80. The model improves area under the curve of external validation cohort (n=227) from 0.64 to 0.71. Conclusions Nonnegative matrix factorization improves the predictive performance of arrhythmic outcomes by extracting latent features between different variables.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Mazzanti ◽  
S Marelli ◽  
T Chargeishvili ◽  
A Trancuccio ◽  
M Marino ◽  
...  

Abstract Background A conclusive estimate of the eligibility rate for the use of subcutaneous implantable cardioverter defibrillators (S-ICD) in patients with Brugada Syndrome (BrS) is lacking. Objective We aimed to: 1) evaluate the eligibility for S-ICD in patients with BrS using a novel automated tool; 2) investigate predictors of ineligibility for S-ICD, based on baseline 12-lead electrocardiogram. Methods Automated screening for S-ICD was performed in 118 consecutive BrS patients using the programmer provided by the S-ICD producer. The system automatically assessed the acceptability of each of the three sense vectors used by the S-ICD for the detection of cardiac rhythm. Eligibility was defined when at least one vector was acceptable both in supine and standing position. Results The clinical characteristics of 118 BrS patients were as follow: age 43±11 years; 89% males; 2% with aborted cardiac arrest; 14% with a history of syncope; 81% with spontaneous type 1 ECG pattern; 21% with a familial history of sudden cardiac death; 24% with an SCN5A mutation. No patients had an indication for pacing. Only 8/118 (7%) patients were ineligible for S-ICD. Of note, 5/8 (63%) patients who failed the screening exhibited a slurred S wave of ≥80 ms duration in the peripheral lead aVF on the 12-lead baseline electrocardiogram, vs. 4/110 (4%) of those who passed the screening (sensitivity of S wave in aVF for screening failure 63%, specificity 97%; p<0.001). Remarkably, the presence of a slurred S wave of ≥80 ms duration in lead aVF remained significantly associated to the failure of eligibility for S-ICD (odds ratio 50, p<0.001) in a multivariable analysis that included the previous history of symptoms, the presence of a spontaneous type 1 ECG pattern, the gender and the presence of SCN5A mutations. ECG predictor of S-ICD screening Conclusion Up to 93% of BrS patients are eligible for S-ICD when the automated screening tool is used. The presence of a slurred S wave in lead aVF on the 12-lead electrocardiogram is a powerful predictor of screening failure.


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