Asymptomatic Patients with Brugada Syndrome: Does the Risk of Sudden Death Justify ICD Implantation?

Brugada syndrome (BrS) is a clinical entity characterised by an incomplete right bundle branch block associated with an ST segment elevation in the right precordial leads and a risk of ventricular arrhythmia and sudden death in the absence of structural abnormalities. Patients with a personal history of sudden death have an annual arrhythmia risk of recurrence as high as 10 %. Similarly, the presence of syncope is consistently associated with an increased arrhythmic risk. This risk can be estimated at about 1.5 % per year. The risk is lower in asymptomatic patients. Regarding the relatively high rate of complication of Implantable cardioverter defibrillator (ICD) implantation, in most of the cases, asymptomatic patients with a Brugada syndrome revealed during ajmaline challenge do not need to be implanted. The situation is more complex in patients with a spontaneous type 1 aspect since the risk could be estimated to be around 0.8 % per year. For these patients, a careful evaluation of the arrhythmic risk using all the different tools available is mandatory.

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Abstract 2640: Clinical Characteristics and Risk Stratification in Symptomatic and Asymptomatic Patients with Brugada Syndrome - Multi-center Study in Japan-

Circulation ◽

10.1161/circ.116.suppl_16.ii_585-a ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Masahiko Takagi ◽

Yasuhiro Yokoyama ◽

Kazutaka Aonuma ◽

Naohiko Aihara ◽

Masayasu Hiraoka

Keyword(s):

Sudden Death ◽

Risk Stratification ◽

Brugada Syndrome ◽

Clinical Characteristics ◽

Low Risk ◽

Cardiac Events ◽

Asymptomatic Patients ◽

Risk Patients ◽

Qrs Duration

Background Neither the clinical characteristics nor risk stratification of symptomatic and asymptomatic patients with Brugada syndrome have been clearly determined. We compared clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for distinguishing high- from low-risk patients. Methods A total of 216 consecutive individuals with Brugada syndrome (mean age 52±14 years, 197 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were compared among 3 groups of patients: VF group; patients with aborted sudden death and documented VF (N=34), Syncope (Sy) group; patients with syncope without documented VF (N=70), and Asymptomatic (As) group; subjects without symptoms (N=112). Comparisons were made among the 3 groups as well as between the symptomatic (VF/Sy) and asymptomatic (As) groups. Short-term prognosis was also compared among the 3 groups, and between the VF/Sy and As groups. Results 1) Clinical characteristics: incidence of past history of AF was significantly higher in the VF and Sy groups than in the AS group (26, 26, and 12 %, respectively; [p=0.04]), though no other clinical parameters differed among the groups. 2) On resting 12-lead ECG, r-J interval (interval from QRS onset to J point) in lead V2 and QRS duration in lead V6 were highest in the VF group (104, 98, and 92 msec in V2 [p<0.001]; 106, 103, and 94 msec in V6 [p<0.0001], respectively, VF vs. Sy vs. As). 3) Positive late potential and inducibility of VF by EPS did not differ in incidence among the 3 groups. 4) Clinical follow-up: during a mean follow-up of 36±16 months, incidence of cardiac events (sudden death and/or VF) was higher in the VF/Sy groups than in the As group (29, 8, and 0 %, respectively [p<0.001]). Multivariate analysis showed that the frequencies of r-J interval ≥ 90 msec in lead V2 and QRS duration ≥ 90 msec in lead V6 were significantly higher in patients with cardiac events (p=0.02, 0.02, respectively). Conclusions In symptomatic patients, prolonged ventricular depolarization in precordial leads of the ECG was prominent in the VF group, and this sign can be used to distinguish high- from low-risk patients with Brugada syndrome.

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Identification of a SCN5A Founder Mutation Causing Sudden Death, Brugada Syndrome and Conduction Blocks in Southern Italy

Heart Rhythm ◽

10.1016/j.hrthm.2021.07.003 ◽

2021 ◽

Author(s):

Antonio Curcio ◽

Alberto Malovini ◽

Andrea Mazzanti ◽

Mirella Memmi ◽

Patrick Gambelli ◽

...

Keyword(s):

Sudden Death ◽

Brugada Syndrome ◽

Southern Italy ◽

Founder Mutation

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Incidence of patients at high risk of sudden death among patients referred for the suspicion of a brugada-syndrome: a prospective study in 35 consecutive patients with Brugada-like ECG changes

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(02)80489-2 ◽

2002 ◽

Vol 39 ◽

pp. 112

Author(s):

Constanze M. Echternach ◽

Martin Borggrefe ◽

Susanne Spehl ◽

Ayse Bilbal ◽

Michael Junker ◽

...

Keyword(s):

High Risk ◽

Sudden Death ◽

Prospective Study ◽

Brugada Syndrome ◽

Ecg Changes ◽

A Prospective Study

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Brugada Syndrome Inducted Ventricular Fibrillation Successfully Supported With Veno-arterial Extracorporeal Membrane Oxygenation and Continuous Renal Replacement Therapy:A Case Report

10.21203/rs.3.rs-77349/v1 ◽

2020 ◽

Author(s):

Chengfeng Huang ◽

Shifang Huang ◽

Jiawen Huang ◽

Xiaoshen Zhang ◽

Fanna Liu ◽

...

Keyword(s):

Cardiac Arrest ◽

Case Report ◽

Renal Replacement Therapy ◽

Brugada Syndrome ◽

Sudden Cardiac Arrest ◽

The Internet ◽

Asymptomatic Patients ◽

Rescue Treatment ◽

Malignant Arrhythmia ◽

Va Ecmo

Abstract BackgroundThe brugada syndrome（BrS）is an inherited disorder that can lead to sudden death. A young man who experienced sudden cardiac arrest caused by Brs was successfully rescued after 2hour cardiopulmonary resuscitation (CPR). This is a case that has raised concerns in China (more than 200 million people read this news via the internet).Case presentationA 28 years old young man was successfully managed with Veno-arterial ECMO (VA-ECMO) and continuous renal replacement therapy (CRRT) after more than 2 hours CPR. The diagnosis of BrS was identified during ECMO supported, and further ECG screening found two asymptomatic patients. ConclusionBrS can lead to malignant arrhythmia. VA-ECMO is the rescue treatment for patients with cardiac arrest who cannot be cured by conventional resuscitation. VA-ECMO combined with CRRT may help to improve the prognosis of critically ill patients.

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Abstract 4876: LAMP2 Cardiomyopathy is a Particularly Adverse Phenocopy of Sarcomeric Hypertrophic Cardiomyopathy

Circulation ◽

10.1161/circ.118.suppl_18.s_955 ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Barry Maron ◽

William C Roberts ◽

Michael Arad ◽

Carolyn Y Ho ◽

Tammy S Haas ◽

...

Keyword(s):

Heart Failure ◽

Hypertrophic Cardiomyopathy ◽

Sudden Death ◽

Heart Transplantation ◽

Systolic Dysfunction ◽

Ventricular Tachyarrhythmia ◽

Young Patients ◽

Disease Process ◽

Left Ventricular ◽

Asymptomatic Patients

Mutations in the X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a morphologic phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM) in young patients, characterized by extreme left ventricular (LV) hypertrophy and pre-excitation. However, the natural history of this newly recognized cardiomyopathy is incompletely resolved. Seven young asymptomatic patients with LAMP2 cardiomyopathy were identified at ages 8 to 15 years; 6 were male. LV hypertrophy was particularly marked (septal thickness 25– 65 mm; mean 42±17) in the presence of nondilated LV cavity. On each ECG, Wolff-Parkinson-White pre-excitation pattern was associated with markedly increased voltages (74±38mm for R- or S-wave). Over the 7±3 year follow-up from initial cardiac diagnosis, all 7 patients experienced particularly adverse disease consequences associated with progressive LV wall thinning and cavity dilatation and systolic dysfunction (ejection fraction, 29±7%) by the ages of 12 – 24 years (mean 20). Of the 7 patients, 5 either died of progressive heart failure, had heart transplantation or were considered for a donor heart; 2 others had sudden death events, including one fatal ventricular tachyarrhythmia refractory to defibrillator therapy and one appropriate defibrillator shock in an asymptomatic female survivor. Pathologic examination of hearts at autopsy showed histopathologic findings compatible with both HCM due to sarcomere protein mutations (i.e., extensive myocyte disarray, intramural small vessel disease, myocardial replacement scarring), and also evidence of a storage disease process (i.e., clusters of myocytes with vacuolated sarcoplasm within fibrotic areas). Heart weights, 1266 and 1425 grams, are the most substantial recorded for hypertrophic cardiomyopathies. LAMP2 cardiomyopathy is a uniformly profound, and particularly deleterious disease entity, causing refractory heart failure with systolic dysfunction as well as sudden death in young patients < 25 years of age. This novel phenocopy of sarcomeric HCM underscores the power of molecular diagnosis for predicting prognosis, and should also raise consideration for intervention with early heart transplantation.

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Abstract 1158: Sudden Death in Children with Cardiomyopathy: Long Term Follow-Up from a National Population-Based Study of Childhood Cardiomyopathy

Circulation ◽

10.1161/circ.118.suppl_18.s_651-b ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Tara Bharucha ◽

Andrew M Davis ◽

Christian Turner ◽

Robert Justo ◽

Terry Robertson ◽

...

Keyword(s):

Risk Factors ◽

Primary Prevention ◽

Sudden Death ◽

Systolic Dysfunction ◽

Population Based ◽

Z Score ◽

Icd Implantation ◽

Population Based Study ◽

Increased Risk

Introduction Better data regarding the incidence and risk factors for sudden cardiac death (SCD) in children with cardiomyopathy (CM) is critical in defining appropriate primary prevention strategies. Methods The National Australian Childhood Cardiomyopathy Study is a prospective cohort study, including all children in Australia with primary CM diagnosed at 0 – 10 years of age, between 1987–1997. SCD was defined as sudden and unexpected death in children who were not hospitalized and not in congestive heart failure at the time of death. Nine subjects with sudden death as presenting symptom were excluded. Indexed echocardiographic measurements at latest follow-up were compared between subjects with SCD and survivors. Results Study criteria were met by 291 children. Mean duration of follow-up was 9.2 years. The incidence of sudden death relative to each CM type, for all cases and as a proportion of deaths, is shown in the Table : Incidence of SCD by CM type. SCD incidence was significantly associated with CM type, for all cases ( p = 0.006) and when only those subjects who died were considered ( p = 0.005), with LVNC and RCM having up to 4 times the risk of other CM types. Children with familial DCM had a significantly higher rate of SCD than subjects with non-familial CM (12% vs 3%; p = 0.028), however, familial CM was not a risk factor in other CM types. DCM SCD subjects had larger LVEDd Z score than survivors (median 5.53 vs 1.16; p <0.0001) and lower FS Z score (median −9.23 vs −0.51; p = 0.0025). HCM SCD subjects had thicker LVPW dimension Z scores than survivors (median 4.63 vs 1.18; p = 0.007). Twelve subjects (2 DCM, 8 HCM and 2 LVNC) underwent ICD implantation (8/12 for primary prevention). Conclusions: This population based study defines new risk factors for sudden death in children with CM. RCM is well known to have a high incidence of SCD. In addition, children with LVNC and those with DCM who have severe dilatation, systolic dysfunction or familial DCM are at increased risk of sudden death.

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