Torsades de pointes short-coupling

Torsades de Pointes (TdP) refers to a polymorphic ventricular tachycardia (VT) with undulating QRS axis that occurs in long QT syndrome (LQTS), although the term has been used to describe polymorphic ventricular tachyarrhythmias in which QT intervals are not prolonged, such as short-coupled variant of TdP currently known as short-coupled ventricular fibrillation (VF) and Brugada syndrome. Extensive works on LQTS-related TdP over more than 50 years since it was first recognized by Dessertennes who coined the French term meaning "twisting of the points", have led to current understanding of the electrophysiological mechanism that TdP is initiated by triggered activity due to early afterdepolarization (EAD) and maintained by reentry within a substrate of inhomogeneous repolarization...

Ventricular arrhythmias in cardiac channelopathies are linked to autonomic triggers, which are sub-optimally targeted in current management strategies. Improved molecular understanding of cardiac channelopathies and cellular autonomic signalling could refine autonomic therapies to target the specific signalling pathways relevant to the specific aetiologies as well as the central nervous system centres involved in the cardiac autonomic regulation. This review summarizes key anatomical and physiological aspects of the cardiac autonomic nervous system (ANS) and its impact on ventricular arrhythmias in primary inherited arrhythmia syndromes...

UNLABELLED: The presence of J waves in cases of ventricular fibrillation (VF) is known to be a risk for sudden cardiac death. Recently, the effectiveness of radiofrequency catheter ablation (RFCA) for early repolarization syndrome (ERS) has been reported.The patient is a 30-year-old male with elevated J waves of 0.1 mV in the inferior leads, who had previously developed VF and undergone implantable cardioverter defibrillator (ICD) implantation. Because the VF from short coupled premature ventricular contraction (PVC) was presented, the RFCA of the triggered PVC was attempted...

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Harmony valve (Medtronic, Inc.) was recently approved to treat postoperative native outflow tract pulmonary regurgitation. While the 22 mm Harmony valve Early Feasibility Study demonstrated ventricular tachycardia (VT) in only 5% of patients, little is known about ventricular arrhythmias after TPVR with the larger 25 mm valve (TPV25). METHODS: A single center review was performed of patients with TPV25 implant from 2020 to 2021...

Background: The short-coupled variant of torsade de pointes (scTdP) is characterized by a particular electrocardiogram (ECG) pattern that shows a short-coupling interval of the initial Tdp beat and that can degenerate into ventricular fibrillation without the presence of structural heart disease. However, its etiology, epidemiology, clinical characteristics, underlying mechanism, treatment, and prognosis remain unclear. This study aimed to systematically review case reports and series of scTdP to synthesize existing data on the demography, clinical characteristics, ECG features, management, and outcomes...

AIMS: Gain-of-function mutations in RYR2, encoding the cardiac ryanodine receptor channel (RyR2), cause catecholaminergic polymorphic ventricular tachycardia (CPVT). Whereas, genotype-phenotype correlations of loss-of-function mutations remains unknown, due to a small number of analysed mutations. In this study, we aimed to investigate their genotype-phenotype correlations in patients with loss-of-function RYR2 mutations. METHODS AND RESULTS: We performed targeted gene sequencing for 710 probands younger than 16-year-old with inherited primary arrhythmia syndromes (IPAS)...

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No abstract text is available yet for this article.

BACKGROUND: The short-coupled variant of torsade de pointes (sc-TdP) is a malignant arrhythmia that frequently presents with ventricular fibrillation (VF) electrical storm. Verapamil is considered the first-line therapy of sc-TdP while catheter ablation is not widely adopted. The aim of this study was to determine the origin of sc-TdP and to assess the outcome of catheter ablation using 3D-mapping. METHODS AND RESULTS: We retrospectively analyzed five patients with sc-TdP who underwent 3D-mapping and ablation of sc-TdP at five different institutions...

AIMS: Distinctive types of polymorphic ventricular tachycardia (VT) respond differently to different forms of therapy. We therefore performed the present study to define the electrocardiographic characteristics of different forms of polymorphic VT. METHODS AND RESULTS: We studied 190 patients for whom the onset of 305 polymorphic VT events was available. The study group included 87 patients with coronary artery disease who had spontaneous polymorphic VT triggered by short-coupled extrasystoles in the absence of myocardial ischaemia...

Idiopathic ventricular fibrillation (IVF) causes sudden death in young adult patients without structural or ischemic heart disease. Most IVF cases are sporadic and some patients present with short-coupled torsade de pointes, the genetics of which are poorly understood. A man who had a first syncope at the age of 35 presented with frequent short-coupled premature ventricular beats with bursts of polymorphic ventricular tachycardia and then died suddenly. By exome sequencing, we identified three rare variants: p...

In this case report, we describe a 73 year old female with structuraly normal heart that developed shortcoupled torsades de pointes (TdP) resulting in an electrical storm unresponsible to several antiarrhythmic drugs, but fully controlled with verapamil. The critical timing of the ventricular premature beats that initiated TdP corresponded to those that occurred at the peak of the previous T wave. This behavior differentiates this entity from other forms of malignant ventricular arrhythmias in patients with structurally normal heart...

BACKGROUND: Torsades de pointes arrhythmia is a potentially lethal polymorphic ventricular tachyarrhythmia (pVT) in the setting of long QT syndrome. Arrhythmia susceptibility is influenced by risk factors modifying repolarization. OBJECTIVE: The purpose of this article was to characterize repolarization duration and heterogeneity in relation to pVT inducibility and maintenance. METHODS: Sotalol was infused regionally or globally in isolated Langendorff blood-perfused pig hearts (N = 7) to create repolarization time (RT) heterogeneities...

Idiopathic ventricular fibrillation (IVF) is a diagnosis of exclusion made when no underlying cause is identified in a cardiac arrest survivor. Although the frequency of this diagnosis has declined over time due to advances in diagnostic techniques, it remains a substantial cause of sudden cardiac arrest. Further, IVF tends to recur. This article reviews the criteria for diagnosis, patient characteristics, the two primary arrhythmic phenotypes-short-coupled variant of torsades de pointes and recurrent paroxysmal IVF-and the electrophysiologic features, treatment, and ablation of premature ventricular complexes that can trigger IVF...

BACKGROUND: Short-coupled variant of torsades de pointes (scTdP) is a disease characterized by TdP without QT prolongation, which is initiated by extremely short-coupled ventricular extra-systoles. Its genetic background remains rarely unveiled. OBJECTIVE: We aimed to identify genetic variations in patients with scTdP and to analyze the functional change of the mutant Na+ channel identified in a scTdP patient. METHODS AND RESULTS: We performed genetic analysis for inherited arrhythmia-related 45 genes using next-generation sequencer (MiSeq, Illumina) among seven consecutive scTdP patients...

A 40-year-old male visited our institute complaining of transient loss of consciousness. He had been implanted with an implantable cardioverter defibrillator (ICD) due to idiopathic ventricular fibrillation for secondary prevention. His past genetic screening detected a single nucleotide SCN5A mutation (pR18Q), while neither QT prolongation nor ST segment elevation in the right precordial leads was observed. An interrogation of the ICD revealed that a shock therapy successfully terminated ventricular fibrillation at the time syncope occurred...

Introduction : Ventricular arrhythmias are often seen in association with structural heart disease. However, approximately a tenth of affected patients have apparently normal hearts, where such arrhythmias typically occur in young patients, are sometimes inherited and can occasionally lead to sudden cardiac death (SCD). Over the past two decades, increased understanding of the underlying pathophysiology resulted in improved targeted pharmacological therapy. Areas covered : This article reviews current knowledge regarding drug therapy for inherited arrhythmia syndromes (Brugada, early repolarization, long QT and short QT syndromes, and catecholaminergic polymorphic ventricular tachycardia), and acquired arrhythmias (idiopathic ventricular fibrillation, short-coupled torsade de pointes, outflow tract ventricular tachycardia, idiopathic left, papillary muscle and annular ventricular tachycardias)...

BACKGROUND: A short-coupled variant of torsade de pointes (ScTdP) is rare and resistant to medical treatment. There has not been a reported catheter ablation (CA) of a short-coupled premature ventricular contraction (PVC) triggering ScTdP in an infant. CASE SUMMARY: A neonate was referred to our hospital on the day of birth for Wolff-Parkinson-White syndrome, repeated episodes of supraventricular tachycardia, and a left ventricular non-compaction. She underwent CA of an accessory pathway at 72 days of age...

A 44-year-old woman with previous myocarditis underwent several syncopal events due to self-terminated ventricular fibrillation (VF) with hypokalemia. Electrocardiogram showed a normal QT duration and premature ventricular contraction with a short coupling interval (280 ms), and the R-on-T phenomenon induced Torsade de pointes (TdP) that deteriorated into VF, a condition known as short coupled TdP (ScTdP). Cardiac magnetic resonance imaging showed left ventricular dilatation and diffuse high T2 signal intensity, and endomyocardial biopsy exhibited a failing myocardium...

INTRODUCTION: Idiopathic short-coupled ventricular tachyarrhythmias make up a considerable proportion of ventricular tachyarrhythmias in structurally normal hearts and are the cause of 5-10% of unexpected sudden cardiac deaths. There is disparity in the literature regarding their description and a lack of formal diagnostic criteria to define them. OBJECTIVE: To validate ECG indices for the diagnosis of these ventricular tachyarrythmias and to subsequently unify their differing descriptions in the literature under a new terminology: Idiopathic Short-Coupled Ventricular Tachyarrhythmias ...