Case report: Synergetic effect of ischaemia and increased vagal tone inducing ventricular fibrillation in a patient with Brugada syndrome.

Background: Brugada syndrome (BS) is a hereditary channelopathy associated with syncope, malignant ventricular arrhythmia, and sudden cardiac death. Right ventricular ischaemia and BS have similar underlying substrates precipitating ventricular tachycardia or fibrillation (VF).

Case Summary: A 72-year-old woman with BS and a stenosis on the proximal right coronary artery received several subsequent implantable cardioverter-defibrillator shocks due to VF during an episode of extreme nausea with vomiting.

Speckle tracking echocardiography data in Brugada syndrome patients.

Brugada syndrome is characterized by typical electrocardiogram changes and a high risk for sudden cardiac death (Priori et al., 2013). In addition to the well known electrical substrate, morphological and functional alterations appeared to be present in a subset of the Brugada syndrome patients (Catalano et al.

Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias.

Background: Brugada syndrome (BrS) is characterized by a high risk of sudden cardiac death. The clinical value of deformation imaging in patients with BrS is unknown. We aimed to assess whether echocardiographic speckle tracking parameters differ between: 1) BrS patients and healthy controls, 2) BrS patients with and without life-threatening ventricular arrhythmias.

What the internist should know about hereditary muscle channelopathies.

Objectives: Non-dystrophic myotonia, periodic paralysis and, to a certain extent, myotonic dystrophies are rare hereditary skeletal muscle channelopathies, charactarized by myotonia or episodic muscle weakness. This review highlights the diagnostic challenges and treatment options.

Results: Some of these rare skeletal muscle disorders are associated with a broad range of systemic and nonspecific muscle symptoms.

Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of SCN5A Variant - Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping.

Background: Patients with Brugada syndrome (BrS) and a history of syncope or sustained ventricular arrhythmia have longer right ventricular ejection delays (RVEDs) than asymptomatic BrS patients. Different types ofSCN5Avariants leading to different reductions in sodium current (I) may have different effects on conduction delay, and consequently on electromechanical coupling (i.e.

Comparative study of the failure rates among 3 implantable defibrillator leads.

Background: After the introduction of the Biotronik Linox S/SD high-voltage lead, several cases of early failure have been observed.

Objective: The purpose of this article was to assess the performance of the Linox S/SD lead in comparison to 2 other contemporary leads.

Methods: We used the prospective Erasmus MC ICD registry to identify all implanted Linox S/SD (n = 408), Durata (St.

Mismatch between the origin of premature ventricular complexes and the noncompacted myocardium in patients with noncompaction cardiomyopathy patients: involvement of the conduction system?

Background: Noncompaction cardiomyopathy (NCCM) is considered to be the result of an arrest in the normal myocardial embryogenesis. The histological, developmental, and electrophysiological explanation of ventricular arrhythmias in NCCM is still unknown. The aim of this study was to determine the origin of premature ventricular contractions (PVCs) in NCCM and to identify any predominant arrhythmic foci.

Contribution of Cardiac Sodium Channel β-Subunit Variants to Brugada Syndrome.

Background: Brugada syndrome (BrS) is an inheritable cardiac disease associated with syncope, malignant ventricular arrhythmias and sudden cardiac death. The largest proportion of mutations in BrS is found in the SCN5A gene encoding the α-subunit of cardiac sodium channels (Nav1.5).

SCN4A variants and Brugada syndrome: phenotypic and genotypic overlap between cardiac and skeletal muscle sodium channelopathies.

SCN5A mutations involving the α-subunit of the cardiac voltage-gated muscle sodium channel (NaV1.5) result in different cardiac channelopathies with an autosomal-dominant inheritance such as Brugada syndrome. On the other hand, mutations in SCN4A encoding the α-subunit of the skeletal voltage-gated sodium channel (NaV1.

Prolonged right ventricular ejection delay identifies high risk patients and gender differences in Brugada syndrome.

Background And Objectives: Right ventricular (RV) conduction delay has been suggested as an underlying pathophysiological mechanism in Brugada syndrome (BS). In this cross-sectional study we non-invasively assessed the value of echocardiographic markers reflecting ventricular ejection delay to further assess electromechanical abnormalities in BS and to identify patients at risk for life-threatening arrhythmic events. Furthermore, we sought to assess differences in ejection delays between genders because male BS patients demonstrate a more malignant clinical phenotype.

Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience.

Background: The prognostic value of electrophysiological investigations in individuals with Brugada syndrome remains controversial. Different groups have published contradictory data. Long-term follow-up is needed to clarify this issue.

Safe single-dose administration of propofol in patients with established Brugada syndrome: a retrospective database analysis.

Background: Propofol is an anesthetic drug with a very attractive pharmacokinetic profile, which makes it the induction agent of choice, especially in day-case surgery. Data on its potential proarrhythmic effects in patients with Brugada syndrome (BS) patients are still lacking. The aim of our study was to investigate whether a single dose of propofol triggered any adverse events in consecutive high-risk patients with BS.

Prevalence, clinical characteristics and management of atrial fibrillation in patients with Brugada syndrome.

Atrial fibrillation (AF) can be the first manifestation of latent Brugada syndrome (BS). The aim of our study was to assess the prevalence of AF as the first clinical diagnosis in patients with BS and their demographic and clinical characteristics and diagnosis management in a large cohort of patients. The patient group consisted of 611 patients with BS.

The effect of conductive ventilation heterogeneity on diffusing capacity measurement.

It has long been assumed that the ventilation heterogeneity associated with lung disease could, in itself, affect the measurement of carbon monoxide transfer factor. The aim of this study was to investigate the potential estimation errors of carbon monoxide diffusing capacity (Dl(CO)) measurement that are specifically due to conductive ventilation heterogeneity, i.e.