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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the last 14 years - since the Brugada syndrome was first recognized as a distinct clinical/electrocardiographical entity - a considerable number of papers have been published on its various aspects. It has been defined as the combination of a typical ST-segment elevation in the right precordial leads and a predisposition for malignant ventricular arrhythmias occurring in the absence of structural heart disease. From the outset, controversy arose about the diagnostic criteria to be applied. This issue has been clarified since the announcement of a first (2002) and second (2005) consensus report. Our review will discuss the clinical characteristics and different possible pathophysiological mechanisms underlying the specific ECG-abnormalities and susceptibility for malignant arrhythmias. Nowadays, the main issue of discussion revolves essentially around its prognostic features, especially in asymptomatic patients. This review will compare the results of different follow-up studies and yields a possible explanation for the differences in event rates and in the identification of useful sudden-death predictors. Finally, the most recent data concerning new diagnostic techniques, gene identification and future therapeutic options will also be discussed.
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PMID:The Brugada syndrome: update 2006. 1803 18

The Brugada syndrome is an important cause of sudden cardiac death primarily among males without structural heart disease. Although not widely known, it may be unmasked by an acute febrile illness. An association between mastitis and Brugada syndrome has not been previously reported. We describe an 18-year-old postpartum female who developed mastitis, fever, and syncope. Electrocardiography revealed type 1 Brugada pattern, which disappeared once her fever resolved. Although the role of pregnancy in this syndrome is unknown, clinicians should be aware that fever may precipitate Brugada syndrome among peripartum women.
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PMID:Mastitis unmasks Brugada syndrome. 1803 75

Brugada syndrome is characterized by the right bundle branch block type electrocardiogram (ECG) with ST-segment elevation and ventricular fibrillation (Vf) attack in patients without obvious heart disease. Its background is considered to be the genetic Na channelopathy. The coved type is a typical morphology and is classified into type 1 in the European Society of Cardiology (ESC). In general, the coved type and the saddel-back type (type 2, 3 in the ESC) are interchangeable, and the latter is more frequently detected clinically. For diagnosis of the Brugada syndrome, confirmation of the type 1 morphology is needed. Administration of Na channel blocker, pilsicainide, is a sensitive test for confirmation of the type 1. When the type 1 has one of 7 items recommended by the ESC, the patient is diagnosed with Brugada syndrome. ECG finding alone is classified into patient with Brugada type ECG. As there is no reliable medical treatment, implantation of a cardioverter defibrillator is indicated in patients with Brugada syndrome, and observation alone in those with Brugada type ECG.
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PMID:[Brugada syndrome]. 1807 85

The J wave syndrome is characterized by a prominent J wave accompanied by ST-segment elevation in the absence of structural heart disease. It includes the benign early repolarization syndrome, the highly arrhythmogenic Brugada syndrome and idiopathic ventricular fibrillation. Although acute coronary syndromes are one of the leading causes of ST-segment deviation, no clinical reports that specifically describe the modulating effects of an ischemic injury current on the ECG manifestations of the J wave syndrome have been found. This report describes four cases of patients with acute inferior ST-segment elevation myocardial infarction who had J wave (or negative deplacement of the J point) and ST-segment depression in the right precordial leads. Later, these precordial ECG alterations disappeared and were progressively replaced by prominent J (R') waves and anterior ST-segment elevations, suggesting the presence of a J wave syndrome. In conclusion, the J wave syndrome may be obscured by an acute inferior myocardial infarction with concomitant ST-segment depression in the right precordial leads. In such circumstances, early detection of the J wave (or depressed J point) may be used as ECG marker of the early repolarization syndrome or Brugada syndrome.
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PMID:[Acute inferior myocardial infarction masking the J wave syndrome. Based on four observations]. 1822 13

We report a 30 year old male without structural heart disease who presented with recurrent nocturnal syncope and aborted sudden cardiac death. 12-lead ECG showed elevated ST in inferior leads and short coupled premature ventricular complexes (PVCs). Propafenone challenge suggested a diagnosis of an atypical Brugada syndrome. Two morphological types of PVCs and ventricular fibrillation (VF) were induced during propafenone challenge test. He underwent two ablation procedures in right ventricular inflow tract and left ventricular post-inferior septum region by pace-mapping, respectively. After ablation, VF could not be induced and the elevated ST segments normalized. Two subsequent propafenone challenge tests were also negative. Nonetheless, elevated ST segments and PVCs reappeared by 1 month follow-up. An implantable defibrillator was recommended, but the patient declined for financial reasons. Unfortunately, he suffered a sudden cardiac death at home 10 weeks post-ablation. These findings suggest that short-term normalization of ventricular repolarization possibly due to radiofrequency ablation may occur in Brugada syndrome. However, the transient nature of this finding suggests that it is not a reliable indicator of protection against sudden cardiac death.
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PMID:Short-term normalization of ventricular repolarization by transcatheter ablation in a patient with suspected Brugada Syndrome. 1829 74

Sudden death (SD) in childhood is rare, representing only 10% of paediatric mortality after one year of age. The individual risk is estimated between 1 in 20.000 and 1 in 50.000 per year. In case of a negative autopsy for cardiac morphologic anomalies, the most presumable cause remains a genetically-determined malignant primary ventricular arrhythmia. Rhythmic sudden cardiac death can be categorized as a complication of a cardiomyopathy (dilated or hypertrophic), or as a primary channelopathy without any structural heart disease. Primary ventricular arrhythmias include long QT syndrome, Brugada syndrome, short QT syndrome and Polymorphic Ventricular Tachycardia. The diagnosis of such syndromes relies upon specific ECG anomalies, personal history of family members, eventually echocardiography and drug challenge. For some of these diseases, morbid genes have been identified thus rendering possible the management of pre symptomatic or undiagnosed family members within specialized multidisciplinary teams. In case of sudden arrhythmic death in children, the parents and siblings must be examined Rescued sudden death exposes to a high risk of recurrence. In such patients, the automatic implantable defibrillator has dramatically improved survival.
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PMID:Arrhythmic sudden death in children. 1839 97

Brugada syndrome is believed to be the cause of up to 50% of sudden cardiac death (SCD) cases due to ventricular dysrhythmias in young healthy individuals with no structural heart disease. This syndrome was first reported in 1992 and is rarely seen in the Emergency Department (ED). Given the life-threatening nature of Brugada syndrome, we hope to increase awareness in Emergency Medicine practitioners. We report the case of a 22-year-old man who was referred to the ED with a history of intermittent palpitations, near-syncope and electrocardiogram findings of RSR' and ST elevation in V1-V2 characteristic of Brugada syndrome. It is crucial that emergency physicians search for this diagnosis, as an implantable cardioverter-defibrillator is the only recognized life-saving intervention, and the risk of SCD is high if the diagnosis is missed.
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PMID:Brugada syndrome presenting as an "acute myocardial infarction". 1932 Dec 85

The Brugada syndrome is an inherited arrhythmogenic and nonstructural heart disease that may cause syncope and sudden cardiac death (SCD). It is characterized by ST segment elevation in the right precordial leads (V1-V3) and male predominance. Mutation on SCN5A encoding the alpha-subunit of the sodium channel is seen in 20%-30% of patients with the syndrome. Because of low penetrance of gene mutation, clinical manifestations and prognosis can be variable in individuals. Therefore, asymptomatic population with Brugada-type electrocardiogram (ECG) exists in community and SCD risk stratification of the individual can be problematic. Although understanding of the cellular mechanism of the syndrome has advanced, there are conflicting data about its prevalence and prognosis. Population-based genetic epidemiology studies with longer follow-up periods may be able to elucidate clinical outcome of the syndrome, particularly asymptomatic patients.
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PMID:Brugada syndrome and Brugada-type electrocardiogram. 1843 88

A 51-year-old woman presented with an episode of syncope. Upon further review she was found to have a typical Brugada type pattern on her electrocardiogram. She did not have evidence for structural heart disease. At electrophysiological testing she was found to have marked infrahisian conduction disease and had easily inducible polymorphic ventricular tachycardia. She underwent implantation of a dual-chamber implantable cardioverter defibrillator (ICD) and family screening was recommended. Genetic analysis revealed a novel nonsense mutation in the gene encoding for the sodium channel (SCN5A). Five months after ICD implantation the patient had an episode of ventricular fibrillation documented on ICD interrogation. This case is unique as it is consistent with an overlap syndrome, namely both Brugada Syndrome and distal atrioventricular (AV) conduction disease secondary to a novel SCN5A mutation in a young female. This finding highlights the phenotypic heterogeneity of novel SCN5A mutations.
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PMID:Brugada syndrome with marked conduction disease: dual implications of a SCN5A mutation. 1843 84

An increasing number of basic and clinical studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (T(p-e)) may correspond to the transmural dispersion of repolarization and that amplification of the T(p-e) interval is associated with malignant ventricular arrhythmias. In this review, we outline the utility of the T(p-e) interval and the T(p-e)/QT ratio as an electrocardiographic index of arrhythmogenesis for both congenital and acquired ion channel disease leading to ventricular arrhythmias. In healthy individuals, the T(p-e)/QT ratio has a mean value of approximately 0.21 in the precordial leads and it remains relatively constant between the heart rates from 60 to 100 beats per minute. Interestingly, the T(p-e)/QT ratio is significantly greater in the patients at risk for arrhythmic event such as those with long QT syndrome, Brugada syndrome, short QT syndrome, and also in patients with organic heart disease such as acute myocardial infarction. Functional reentry is the underlying mechanism for arrhythmogenesis associated with an increased T(p-e)/QT ratio.
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PMID:T(p-e)/QT ratio as an index of arrhythmogenesis. 1879 Apr 99


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