P6586Brugada syndrome risk stratification - Evaluation of clinical and ECG risk markers in a multicenter international primary prevention cohort

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Honarbakhsh ◽  
R Providencia ◽  
J Garcia-Hernandez ◽  
C A Martin ◽  
R J Hunter ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Risk Score ◽  
Cardiac Death ◽  
International Study ◽  
Risk Markers ◽  
History Of ◽  
Score Model

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.

Abstract 5669: Brugada Syndrome: The Absence of Symptoms is a Poor Predictor of Identifying Individuals at Risk of Sudden Death

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Michael Papadakis ◽  
Carey Edwards ◽  
John C Rawlins ◽  
Sanjay Sharma
Keyword(s):  
Sudden Cardiac Death ◽  
Sudden Death ◽  
Risk Stratification ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Polymorphic Ventricular Tachycardia ◽  
Screening Programs ◽  
First Degree Relatives ◽  
History Of

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.

scholarly journals Long term prognosis of out-of-hospital cardiac arrest due to idiopathic ventricular fibrillation - a tertiary center experience

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
R Alves Pinto ◽  
T Proenca ◽  
M Martins Carvalho ◽  
PD Grilo ◽  
CX Resende ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Fibrillation ◽  
Clinical Outcomes ◽  
Cardiac Death ◽  
Previous History ◽  
Provocative Test ◽  
Tertiary Center ◽  
History Of

Abstract Funding Acknowledgements Type of funding sources: None. Background  Sudden cardiac death (SCD) is an uncommon event in the absence of structural heart disease. However, ventricular fibrillation (VF) may occur in patients with unknown cardiac disease and a comprehensive work-up is needed to further improve diagnostic. Still, a significant and heterogenous group of patients remains labelled of Idiopathic VF and limited data is available regarding their natural history. Purpose The aim of this study was to evaluate the clinical outcomes of survivors of an aborted sudden cardiac death due to idiopathic VF or pulseless ventricular tachycardia (VT) and to assess possible predictors of recurrence. Methods  Patients who survived an idiopathic VF or pulseless VT between 2005 and 2019 referred to a cardiac defibrillator (ICD) implantation were included. Patients were followed for 1 to 15 years (median follow-up of 7 years). Clinical and device data were collected. Results A population of 29 patients, 59% male, with a median age of 50 years (age ranging from 18 to 76) at the time of the aborted SCD was studied. All patients implanted an ICD (69% single chamber, 24% dual chamber and 3% subcutaneous) at the index hospitalization. The initial rhythm was VF in 76% and pulseless VT in 24%. In relation to the context of the arrhythmic event, 48.3% occurred during daily life activities, 13.8% after an emotional stress, 6.9% during efforts and a similar percentage occurred either in rest or asleep. Of note, 12.5% of patients had previous history of syncope. Normal ECG was present in 83% of patients. Family history of SCD was present in 12% of the cases. As for the cardiovascular risk factors, 61.5% had hypertension, 19% dyslipidemia, 17% diabetes, 31% were smokers or previous smokers. Paroxysmal atrial fibrillation was present in 15% of patients. To exclude possible causes of VF, all patients were submitted to coronary angiogram and echocardiogram, 64% to genetic testing, 68% to cardiac magnetic resonance, 20% to electrophysiologic study, 12% to pharmacological provocative test and 4% were submitted to endomyocardial biopsy. At follow-up, an etiological diagnosis was established in 31% of patients: 3 events were attributed to coronary vasospasm, 3 to short coupled polymorphic VT, 1 patient had long QT syndrome, 1 had Brugada syndrome and in 1 patient an ANK2 mutation was identified. As for the clinical outcomes, 8% patients died (from non-arrhythmic causes), 31% patients received appropriate therapies and 19% had unappropriated shocks (of those 60% for sinus tachycardia and 40% for supraventricular tachycardia). Conclusion Etiologic diagnosis and prediction of recurrence of arrhythmic events in patients with idiopathic VF is challenging, even with a long-term follow-up and more sophisticated diagnostic evaluation.  Idiopathic VF is a rare but serious condition with recurrence in about one third of patients. Although not free of complications, ICD remains the gold standard of treatment.

scholarly journals Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers

EP Europace ◽  
2010 ◽  
Vol 12 (3) ◽  
pp. 313-321 ◽  
Author(s):  
Imke Christiaans ◽  
Klaartje van Engelen ◽  
Irene M. van Langen ◽  
Erwin Birnie ◽  
Gouke J. Bonsel ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Risk Markers ◽  
Clinical Risk

scholarly journals Outcome of Insertable Cardiac Monitors in Symptomatic Patients with Brugada Syndrome at Low Risk of Sudden Cardiac Death

Cardiology ◽  
2020 ◽  
Vol 145 (7) ◽  
pp. 413-420
Author(s):  
Rafi Sakhi ◽  
Amira Assaf ◽  
Dominic A.M.J. Theuns ◽  
Judith M.A. Verhagen ◽  
Tamas Szili-Torok ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Arrhythmia ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Electrophysiological Study ◽  
Pacemaker Implantation ◽  
Low Risk ◽  
History Of

Introduction: There is limited data on the experience with insertable cardiac monitors (ICMs) in patients with Brugada syndrome. Objective: To evaluate the outcome of ICM in symptomatic patients with Brugada syndrome who are at suspected low risk of sudden cardiac death (SCD). Methods: We conducted a prospective single-center cohort study including all symptomatic patients with Brugada syndrome who received an ICM (Reveal LINQ) between July 2014 and October 2019. The main indication for monitoring was to exclude ventricular arrhythmias as the cause of symptoms and to establish a symptom-rhythm relationship. Results: A total of 20 patients (mean age, 39 ± 12 years; 55% male) received an ICM during the study period. Nine patients (45%) had a history of syncope (presumed nonarrhythmogenic), and 5 patients had a recent syncope (<6 months). During a median follow-up of 32 months (interquartile range, 11–36 months), 3 patients (15%) experienced an episode of nonsustained ventricular arrhythmia. No patient died suddenly or experienced a sustained ventricular arrhythmia, and no patient had a recurrence of syncope. Overall, 17 patients (85%) experienced symptoms during follow-up, of whom 10 patients had an ICM-detected arrhythmia. In 4 patients (20%), the ICM-detected arrhythmia was an actionable event. ICM-guided management included antiarrhythmic drug therapy for symptomatic ectopic beats (n = 3), pulmonary vein isolation, and oral anticoagulation for atrial fibrillation (n = 1), electrophysiological study for risk stratification (n = 1), and pacemaker implantation for atrioventricular block (n = 1). Conclusions: An ICM can be used to exclude ventricular arrhythmias in symptomatic patients with Brugada syndrome at low risk of SCD. Furthermore, an ICM-detected arrhythmia changed clinical management in 20% of patients.

scholarly journals The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Gabrielle Norrish ◽  
Cristian Topriceanu ◽  
Chen Qu ◽  
Ella Field ◽  
Helen Walsh ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Risk Score ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Predictive Values ◽  
Arrhythmic Event ◽  
Ecg Abnormalities

Abstract Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0–7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93–2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of &gt;5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484–0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

scholarly journals Transient Type 1 Brugada Pattern without Ongoing Fever during COVID-19 Pneumonia

2020 ◽  
Author(s):  
Maria Cristina Pasqualetto ◽  
Andrea Corrado ◽  
Eleonora Secco ◽  
Fabio Graceffa ◽  
Fausto Rigo
Keyword(s):  
Family History ◽  
Sudden Cardiac Death ◽  
Adverse Effects ◽  
Cardiac Death ◽  
Antiviral Drugs ◽  
Electrolyte Disturbance ◽  
History Of ◽  
Brugada Pattern

Patients with COVID-19 pneumonia can develop heart problems and may also may be susceptible to proarrhythmia, virus-related issues such as fever, stress, electrolyte disturbance and adverse effects from the use of antiviral drugs. We report a transient Brugada-like ECG pattern without ongoing fever in a 57-year-old man, admitted with a diagnosis of COVID-19 pneumonia, who did not have syncope or a family history of sudden cardiac death.

scholarly journals Unique clinical features and long term follow up of survivors of sudden cardiac death in an Asian multicenter study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pang-Shuo Huang ◽  
Jen-Fang Cheng ◽  
Wen-Chin Ko ◽  
Shu-Hsuan Chang ◽  
Tin-Tse Lin ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Asia Pacific ◽  
Left Ventricular Ejection ◽  
Arrhythmic Death ◽  
Long Term Follow Up ◽  
History Of

AbstractThere has been no long-term clinical follow-up data of survivors or victims of sudden cardiac death (SCD). The Taiwan multi-center sudden arrhythmia death syndrome follow-up and clinical study (TFS-SADS) is a collaborative multi-center study with median follow-up time 43 months. In this cohort, the clinical characteristics of these SADS patients were compared with those with ischemic heart disease (IHD). In this SCD cohort, around half (42%) were patients with IHD, which was different from Caucasian SCD cohorts. Among those with normal heart, most had Brugada syndrome (BrS). Compared to those with SADS, patients with IHD were older, more males and more comorbidities, more arrhythmic death, and lower left ventricular ejection fraction. In the long-term follow-up, patients with SADS had a better survival than those with IHD (p < 0.001). In the Cox regression analysis to identify the independent predictors of mortality, older age, lower LVEF, prior myocardial infarction and history of out-of-hospital cardiac arrest were associated with higher mortality and beta blocker use and idiopathic ventricular fibrillation or tachycardia (IVF/IVT) with a better survival during follow-up. History of prior MI was associated with more arrhythmic death. Several distinct features of SCD were found in the Asia–Pacific region, such as higher proportion of SADS, poorer prognosis of LQTS and better prognosis of IVF/IVT. Patients with SADS had a better survival than those with IHD. For those with SADS, patients with channelopathy had a better survival than those with cardiomyopathy.

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 ◽  
...  
Keyword(s):  
Ventricular Fibrillation ◽  
Risk Stratification ◽  
Risk Score ◽  
Brugada Syndrome ◽  
Predictive Value ◽  
Scoring Method ◽  
Non Invasive ◽  
Positive Time ◽  
History Of

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.

Implantable cardiac defibrillator events in patients with arrhythmogenic right ventricular cardiomyopathy

Heart ◽  
2021 ◽  
pp. heartjnl-2020-318415
Author(s):  
Olgierd Woźniak ◽  
Karolina Borowiec ◽  
Marek Konka ◽  
Alicja Cicha-Mikołajczyk ◽  
Andrzej Przybylski ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Right Ventricle ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Icd Implantation ◽  
Right Ventricle Dysfunction ◽  
History Of

ObjectiveArrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a risk of sudden cardiac death. Optimal risk stratification is still under debate. The main purpose of this long-term, single-centre observation was to analyse predictors of appropriate and inappropriate implantable cardioverter-defibrillator (ICD) interventions in the population of patients with ARVC with a high risk of life-threatening arrhythmias.MethodsThe study comprised 65 adult patients (median age 40 years, 48 men) with a definite diagnosis of ARVC who received ICD over a time span of 20 years in primary (40%) or secondary (60%) prevention of sudden cardiac death. The study endpoints were first appropriate and inappropriate ICD interventions (shock or antitachycardia pacing) after device implantation.ResultsDuring a median follow-up of 7.75 years after ICD implantation, nine patients died and six individuals underwent heart transplantation. Appropriate ICD interventions occurred in 43 patients (66.2%) and inappropriate ICD interventions in 18 patients (27.7%). Multivariable analysis using cause-specific hazard model identified three predictors of appropriate ICD interventions: right ventricle dysfunction (cause-specific HR 2.85, 95% CI 1.56 to 5.21, p<0.001), age <40 years at ICD implantation (cause-specific HR 2.37, 95% CI 1.13 to 4.94, p=0.022) and a history of sustained ventricular tachycardia (cause-specific HR 2.55, 95% CI 1.16 to 5.63, p=0.020). Predictors of inappropriate ICD therapy were not found. Complications related to ICD implantation occurred in 12 patients.ConclusionsRight ventricle dysfunction, age <40 years and a history of sustained ventricular tachycardia were predictors of appropriate ICD interventions in patients with ARVC. The results may be used to improve risk stratification before ICD implantation.

scholarly journals Clinical risk score for individualized risk stratification of patients with clinically suspected myocardial inflammation

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
V Puntmann ◽  
G Carr-White ◽  
A Rolf ◽  
H Zainal ◽  
M Vasquez ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Risk Score ◽  
Cardiac Death ◽  
Prognostic Model ◽  
Public Institution ◽  
Myocardial Inflammation ◽  
Clinical Risk Score ◽  
Chi Square ◽  
Clinical Risk

Abstract Objective To develop a clinical risk score for individualized risk stratification of patients with clinically suspected myocardial inflammation. Background Myocardial inflammation is a prominent cause of non-ischaemic dilated cardiomyopathy, heart failure (HF) and sudden cardiac death. Methods This is a prospective multicentre longitudinal study of consecutive patients referred to cardiac magnetic resonance (CMR) with clinically suspected myocardial inflammation between October 2011 and December 2019 as a part of standard diagnostic pathway. Patients were followed up from the date of CMR. The outcome endpoints included major adverse cardiovascular event (MACE, cardiovascular mortality, sudden cardiac death, appropriate device discharge); or death or hospitalisation due to HF). A prognostic model was developed using Cox proportional hazards analysis and validated internally and externally. Results The final dataset included 722 subjects (50 years (40–61); males 422 (58%)). During a follow-up period of median 19 (15–23) months, there were 64 (9%) MACE and 130 (18%) HF events. Ten predictor variables qualified for entry into the prognostic model: age, sex, hematocrit, C-reactive protein, high-sensitive troponin-T (TNT), left and right ventricular ejection fraction, native T1 and T2, and late gadolinium enhancement (LGE). The final multivariable Cox regression model included native T2 (Figure 1A), TNT and LGE (Figure 1B) for the primary (Chi-square: 102.0, p&lt;0.001) and secondary endpoint (Chi-square: 166.9, p&lt;0.001), respectively. Cross-validation as well as external validation of the secondary models revealed good performance and no healthcare system effect. Based on the MyoRISK Score, patients were classified into three risk groups with respective event rates for MACE of 0%, 6.3% and 25.1%, and HF endpoint of 1.8%, 17.3% and 44.2%. TNT≥7 pg/ml allowed to efficiently preselect patients prior to CMR. Conclusions This is the first systematic assessment of outcomes in patients with clinically suspected myocardial inflammation, providing a non-invasive estimation of the probability of adverse events based on a score using readily available clinical parameters. FUNDunding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): DZHK

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