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1.
Inn Med (Heidelb) ; 65(8): 787-797, 2024 Aug.
Artículo en Alemán | MEDLINE | ID: mdl-38977442

RESUMEN

Genetic arrhythmia disorders are rare diseases; however, they are a common cause of sudden cardiac death in children, adolescents, and young adults. In principle, a distinction can be made between channelopathies and cardiomyopathies in the context of genetic diseases. This paper focuses on the channelopathies long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Early diagnosis of these diseases is essential, as drug therapy, behavioral measures, and if necessary, implantation of a cardioverter defibrillator can significantly improve the prognosis and quality of life of patients. This paper highlights the pathophysiological and genetic basis of these channelopathies, describes their clinical manifestations, and comments on the principles of diagnosis, risk stratification and therapy.


Asunto(s)
Arritmias Cardíacas , Síndrome de Brugada , Canalopatías , Humanos , Arritmias Cardíacas/genética , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/terapia , Arritmias Cardíacas/fisiopatología , Canalopatías/genética , Canalopatías/diagnóstico , Canalopatías/terapia , Síndrome de Brugada/genética , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/fisiopatología , Síndrome de Brugada/terapia , Taquicardia Ventricular/genética , Taquicardia Ventricular/terapia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Adolescente , Niño , Síndrome de QT Prolongado/genética , Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/terapia , Síndrome de QT Prolongado/fisiopatología , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología , Adulto , Desfibriladores Implantables , Electrocardiografía
3.
Semin Nephrol ; 44(2): 151518, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38772780

RESUMEN

The risk of arrhythmia and its management become increasingly complex as kidney disease progresses. This presents a multifaceted clinical challenge. Our discussion addresses these specific challenges relevant to patients as their kidney disease advances. We highlight numerous opportunities for enhancing the current standard of care within this realm. Additionally, this review delves into research concerning early detection, prevention, diagnosis, and treatment of various arrhythmias spanning the spectrum of kidney disease.


Asunto(s)
Arritmias Cardíacas , Humanos , Arritmias Cardíacas/etiología , Arritmias Cardíacas/fisiopatología , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/fisiopatología , Fibrilación Atrial/complicaciones , Síndrome de Brugada/complicaciones , Síndrome de Brugada/fisiopatología , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Síndrome de QT Prolongado/complicaciones , Síndrome de QT Prolongado/diagnóstico , Fibrilación Ventricular/etiología , Fibrilación Ventricular/terapia , Fibrilación Ventricular/fisiopatología
4.
Medicine (Baltimore) ; 103(18): e37990, 2024 May 03.
Artículo en Inglés | MEDLINE | ID: mdl-38701276

RESUMEN

Brugada syndrome (BS) is characterized by ST segment elevation in right precordial leads (V1-V3), ventricular tachycardia (VT), ventricular fibrillation (VF), and sudden cardiac death (SCD) in individuals without structural heart disease. The aim of this study is to contribute to the controversial issue of finding the most valuable marker that can predict poor prognosis during follow-up in patients with a diagnosis of BS. A total of 68 patients diagnosed with BS or had Brugada-type ECG change between January 1997 and July 2012 at the Department of Cardiology of Baskent University Faculty of Medicine, Ankara, Turkey, were included in this cohort study. Patients were screened every 6 months for arrhythmia-related syncope, SCD, appropriate and inappropriate defibrillation (shock), AF development and death; collectively defined as "arrhythmic events" and were the primary endpoints. Patients with and without arrhythmic events were compared. The mean age was 34.9 ±â€…12.2 years (9-71 years), and 52 (76.5%) patients were male. Mean follow-up was 49.6 ±â€…37.6 months (4-188 months). Univariate analysis showed that male sex (P = .004), type 1 electrocardiographic pattern (P = .008), SCD (P = .036), VT/VF history (P = .046), requirement for electrophysiological studies (P = .034), implantable cardioverter-defibrillator placement (P = .014) were found to demonstrate significant differences in patients with and without arrhythmic events. In multivariable analyzes, spontaneous type 1 ECG presence (HR = 8.54, 95% CI: 0.38-26.37; P = .003) and VT/VF history (HR = 9.21, 95% CI: 0.004-1.88; P = .002) were found to be independently associated with arrhythmic events. We found the presence of spontaneous type 1 ECG and a history of VT/VF to be associated with increased likelihood of overall arrhythmic events in BS. Given the higher risk of poor prognosis, we recommend additional measures in patients with BS who have these features.


Asunto(s)
Síndrome de Brugada , Muerte Súbita Cardíaca , Electrocardiografía , Humanos , Síndrome de Brugada/terapia , Síndrome de Brugada/complicaciones , Síndrome de Brugada/fisiopatología , Masculino , Femenino , Adulto , Persona de Mediana Edad , Factores de Riesgo , Estudios de Seguimiento , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/epidemiología , Adolescente , Adulto Joven , Anciano , Niño , Turquía/epidemiología , Pronóstico , Desfibriladores Implantables , Fibrilación Ventricular/terapia , Fibrilación Ventricular/etiología , Fibrilación Ventricular/complicaciones , Arritmias Cardíacas/etiología , Arritmias Cardíacas/terapia
6.
Curr Probl Cardiol ; 49(6): 102566, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38599558

RESUMEN

Brugada syndrome (BrS) is a genetic disorder known for its characteristic electrocardiogram (ECG) patterns and increased risk of sudden cardiac death. Brugada phenocopy (BrP) presents similar ECG patterns but is distinguished by its reversible nature when the underlying conditions are resolved. This article delineates the intricacies of BrP, emphasizing its etiology, clinical presentation, diagnosis, treatment, and prognosis. The article categorizes BrP based on various underlying causes, including metabolic disturbances, myocardial infarction, and mechanical compression, among others. It also underscores the critical importance of differentiating BrP from BrS to avoid misdiagnosis and inappropriate treatment, such as unnecessary implantation of cardioverter-defibrillators. The reversible aspect of BrP underlines the necessity for an etiology-specific approach to treatment, which not only prevents cardiac death but also highlights the significance of understanding the dynamic nature of ECG patterns. Through an exploration of case studies and current research, this review advocates for increased awareness and further investigation into BrP. It aims to enhance the diagnostic accuracy and management strategies, thereby improving the prognosis for patients presenting with Brugada-like ECG patterns. The review culminates in a call for further research to close existing knowledge gaps and improve patient outcomes.


Asunto(s)
Síndrome de Brugada , Electrocardiografía , Fenotipo , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Síndrome de Brugada/fisiopatología , Humanos , Diagnóstico Diferencial , Pronóstico , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología , Desfibriladores Implantables
7.
Eur Heart J ; 45(14): 1255-1265, 2024 Apr 07.
Artículo en Inglés | MEDLINE | ID: mdl-38445836

RESUMEN

BACKGROUND AND AIMS: Available data on continuous rhythm monitoring by implantable loop recorders (ILRs) in patients with Brugada syndrome (BrS) are scarce. The aim of this multi-centre study was to evaluate the diagnostic yield and clinical implication of a continuous rhythm monitoring strategy by ILRs in a large cohort of BrS patients and to assess the precise arrhythmic cause of syncopal episodes. METHODS: A total of 370 patients with BrS and ILRs (mean age 43.5 ± 15.9, 33.8% female, 74.1% symptomatic) from 18 international centers were included. Patients were followed with continuous rhythm monitoring for a median follow-up of 3 years. RESULTS: During follow-up, an arrhythmic event was recorded in 30.7% of symptomatic patients [18.6% atrial arrhythmias (AAs), 10.2% bradyarrhythmias (BAs), and 7.3% ventricular arrhythmias (VAs)]. In patients with recurrent syncope, the aetiology was arrhythmic in 22.4% (59.3% BAs, 25.0% VAs, and 15.6% AAs). The ILR led to drug therapy initiation in 11.4%, ablation procedure in 10.9%, implantation of a pacemaker in 2.5%, and a cardioverter-defibrillator in 8%. At multivariate analysis, the presence of symptoms [hazard ratio (HR) 2.5, P = .001] and age >50 years (HR 1.7, P = .016) were independent predictors of arrhythmic events, while inducibility of ventricular fibrillation at the electrophysiological study (HR 9.0, P < .001) was a predictor of VAs. CONCLUSIONS: ILR detects arrhythmic events in nearly 30% of symptomatic BrS patients, leading to appropriate therapy in 70% of them. The most commonly detected arrhythmias are AAs and BAs, while VAs are detected only in 7% of cases. Symptom status can be used to guide ILR implantation.


Asunto(s)
Síndrome de Brugada , Desfibriladores Implantables , Marcapaso Artificial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arritmias Cardíacas/complicaciones , Arritmias Cardíacas/diagnóstico , Síndrome de Brugada/complicaciones , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Electrocardiografía/métodos , Electrocardiografía Ambulatoria/métodos , Adulto
10.
J Cardiovasc Electrophysiol ; 35(4): 701-707, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38329163

RESUMEN

INTRODUCTION: Most patients with Brugada syndrome (BrS) are first diagnosed in their 40s, with sudden cardiac death (SCD) often occurring in their 50s. Ventricular fibrillation (VF) may occur in some patients with BrS despite having been asymptomatic for a long period. This study aimed to assess the incidence and risk factors for late life-threatening arrhythmias in patients with BrS. METHODS: Patients with BrS (n = 523; mean age, 51 ± 13 years; male, n = 497) were enrolled. The risk of late life-threatening arrhythmia was investigated in 225 patients who had experienced no cardiac events (CEs: SCD or ventricular tachyarrhythmia) for at least 10 years after study enrollment. The incidence of CEs during the follow-up period was examined. RESULTS: During the follow-up of the 523 patients, 59 (11%) experienced CEs. The annual incidences of CEs were 2.87%, 0.77%, and 0.09% from study enrollment to 3, 3-10, and after 10 years, respectively. Among 225 patients who had experienced no CEs for at least 10 years after enrollment, four patients (1.8%) subsequently experienced CEs. Kaplan-Meier analysis revealed significant differences in the incidence of late CEs between patients with and without a history of symptoms (p = .032). The positive and negative predictive values of late CEs for the programmed electrical stimulation (PES) test were 2.9% and 100%, respectively. CONCLUSION: Our results suggest that patients with BrS who are asymptomatic and have no ventricular tachycardia/VF inducibility by PES are at extremely low risk of experiencing late life-threatening arrhythmias.


Asunto(s)
Síndrome de Brugada , Humanos , Masculino , Adulto , Persona de Mediana Edad , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Síndrome de Brugada/complicaciones , Estudios de Seguimiento , Japón/epidemiología , Electrocardiografía/métodos , Arritmias Cardíacas/complicaciones , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/epidemiología , Fibrilación Ventricular/terapia , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología
11.
J Cardiovasc Electrophysiol ; 35(4): 708-714, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38348526

RESUMEN

BACKGROUND: 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. AIM: In present study, we aim to evaluate presentation, long-term outcome, genetic findings, and therapeutic interventions in patients with BrS. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.


Asunto(s)
Síndrome de Brugada , Desfibriladores Implantables , Masculino , Humanos , Femenino , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Síndrome de Brugada/terapia , Estudios Longitudinales , Irán , Electrocardiografía/métodos
13.
Telemed J E Health ; 30(5): 1499-1503, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38294864

RESUMEN

Telemedicine and remote monitoring devices, including implantable loop recorders (ILR), are increasingly adopted in the cardiologic setting. These are valuable tools in the arrhythmic stratification of patients at risk of sudden cardiac death, providing a tailored therapeutic management to prevent lethal arrhythmias. We report a case of an asymptomatic 18-year-old boy with a family history of syncope and cardiac arrest, who had a diagnosis of Brugada syndrome with an inducible type 1 pattern and carrier of a missense mutation of the SCN5A gene. In light of the risk factors, although not recommended by current guidelines, we decided to proceed with the implantation of an ILR with remote monitoring service. A few months later, an episode of asymptomatic sustained polymorphic ventricular tachycardia was promptly observed by the remote monitoring, leading to a timely implantation of a subcutaneous cardiac implantable defibrillator.


Asunto(s)
Síndrome de Brugada , Desfibriladores Implantables , Telemedicina , Humanos , Masculino , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Síndrome de Brugada/terapia , Adolescente , Telemedicina/métodos , Medición de Riesgo/métodos , Canal de Sodio Activado por Voltaje NAV1.5/genética , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/terapia , Electrocardiografía , Electrocardiografía Ambulatoria/instrumentación , Electrocardiografía Ambulatoria/métodos , Mutación Missense , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología
14.
Pacing Clin Electrophysiol ; 47(8): 1054-1056, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38240394

RESUMEN

Allergic reactions to components of cardiac implantable electronic devices are rare and often go undiagnosed, which can lead to a misdiagnosis of device infection. Contact allergy to subcutaneous implantable cardioverter defibrillator (S-ICD) is extremely rare. In this report, we present a case of cobalt-related contact allergy in a pediatric patient with Brugada syndrome who underwent S-ICD implantation.


Asunto(s)
Síndrome de Brugada , Desfibriladores Implantables , Humanos , Desfibriladores Implantables/efectos adversos , Síndrome de Brugada/terapia , Masculino , Niño , Dermatitis Alérgica por Contacto/etiología , Dermatitis Alérgica por Contacto/diagnóstico
15.
Artículo en Inglés | MEDLINE | ID: mdl-38085327

RESUMEN

Over the last three decades, what is referred to as Brugada syndrome (BrS) has developed from a clinical observation of initially a few cases of sudden cardiac death (SCD) in the absence of structural heart disease with ECG signs of "atypical right bundle brunch block" to a predominantly electrocardiographic, and to a lesser extent genetic, diagnosis. Today, BrS is diagnosed in patients without overt structural heart disease and a spontaneous Brugada type 1 ECG pattern regardless of symptoms. The diagnosis of BrS is less clear in those with an only transient or drug-induced type 1 Brugada pattern, but should be considered in the presence of an arrhythmic syncope, family history of BrS, or family history of sudden death. In addition to survived cardiac arrest, syncope is probably the single most decisive risk marker for future arrhythmias. For asymptomatic BrS, risk stratification remains challenging. General recommendations to lower the risk in BrS include avoidance of drugs/agents known to induce and/or increase right precordial ST-segment elevation, including treatment of fever with antipyretic drugs. Several ECG markers that have been associated with an increased risk of SCD have been incorporated into a recently published risk score for BrS. The aim of this article is to provide an overview of the status of risk stratification and to illustrate open issues und gaps in evidence in BrS.


Asunto(s)
Síndrome de Brugada , Humanos , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Síndrome de Brugada/terapia , Arritmias Cardíacas , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Factores de Riesgo , Electrocardiografía , Síncope/diagnóstico , Síncope/etiología
16.
Circ Arrhythm Electrophysiol ; 17(1): e012072, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-38099441

RESUMEN

Although there is consensus on the management of patients with Brugada Syndrome with high risk for sudden cardiac arrest, asymptomatic or intermediate-risk patients present clinical management challenges. This document explores the management opinions of experts throughout the world for patients with Brugada Syndrome who do not fit guideline recommendations. Four real-world clinical scenarios were presented with commentary from small expert groups for each case. All authors voted on case-specific questions to evaluate the level of consensus among the entire group in nuanced diagnostic and management decisions relevant to each case. Points of agreement, points of controversy, and gaps in knowledge are highlighted.


Asunto(s)
Síndrome de Brugada , Paro Cardíaco , Humanos , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Electrocardiografía , Paro Cardíaco/diagnóstico , Paro Cardíaco/terapia , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Consenso
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