Short-coupled variant of torsade de pointes

Bogaard, Kjell; Steen, Michael; Tan, Hanno L.; Tukkie, Raymond

doi:10.1007/bf03086155

Cited by 25 publications

(15 citation statements)

References 13 publications

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“…TdP with a normal QT interval was originally described in 1994 by Leenhardt et al These investigators described 14 patients with structurally normal hearts, short‐coupled PVCs preceding the TdP, and no electrocardiographic evidence of a long QT interval, as was the case with our patient. Since this original description, several other series have been published describing similar patients …”

Section: Discussionmentioning

confidence: 97%

“…Since this original description, several other series have been published describing similar patients. [3][4][5][6][7] In short-coupled TdP, the QT is of normal duration and the initial coupling interval of the TdP is less than 300 ms. Specifically, the patients described by Leenhardt In the series by Leenhardt et al, verapamil and ICD placement were the only effective therapies in suppressing and treating episodes of VF, consistent with our patient's dramatic response to verapamil.…”

Section: Discussionmentioning

confidence: 99%

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Pregnancy and Short‐Coupled Torsades de Pointes

Burrows

Fox

Biblo

et al. 2010

Pacing Clinical Electrophis

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This 24-year-old woman had incessant polymorphic ventricular tachycardia (PVT) during week 24 of her pregnancy and received over 200 implantable cardioverter-defibrillator discharges. She failed to respond to quinidine, magnesium, isoproterenol, amiodarone, esmolol, and cilostazol during her PVT storm, although her dramatic response to verapamil was consistent with the diagnosis of short-coupled variant of torsades de pointes. The case illustrated the utility of extracorporeal membrane oxygenation during refractory PVT, while attempting diagnostic and therapeutic pharmacologic maneuvers.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Pregnancy and Short‐Coupled Torsades de Pointes

Burrows

Fox

Biblo

et al. 2010

Pacing Clinical Electrophis

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“… Short-coupled variant of Torsades de Pointes (TdP) : This is a rare cause of polymorphic VT in young patients with syncope which results in ventricular fibrillation (VF). A coupling interval of the first TdP beat is always <300 ms [ 6 ]. In this patient, VPB's preceding VT was not always short-coupled (Fig.…”

Section: Discussionmentioning

confidence: 99%

Catecholaminergic polymorphic ventricular tachycardia: a rare cause of recurrent syncope

et al. 2015

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia characterized by adrenergically induced polymorphic or bidirectional ventricular tachycardia (VT). Although a rare disease, its recognition is important because of its high mortality rate when left untreated. We report an index case of a 32-year-old woman who presented with recurrent syncope. The diagnosis was confirmed by exercise-induced polymorphic ventricular premature beats and episodes of non-sustained VT, in the absence of structural heart abnormalities. She remained event free with beta-blocker therapy. CPVT is a potentially life-threatening disease and should be considered in the case of recurrent syncope, in young individuals. Diagnosis is based on clinical history and exercise testing, which is the gold standard. Therapy is mandatory in all diagnosed individuals. Exercise testing in first-degree relatives is recommended, even in the case of a mutation-negative index patient.

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“…Short‐coupled variant of torsades de pointes (scTdP) is initiated by extremely short‐coupled ventricular extra‐systoles (<300 ms) in patients without QT prolongation and structural heart disease. A family history of sudden cardiac death (SCD) in scTdP was reportedly positive in up to 30% of the probands, 1 and previous several case reports have described that scTdP was one of the cases of SCD 1–4 . Recently, we reported four cases of scTdP who had RYR2 variants and performed the functional analysis of the cardiac ryanodine channels with these variants 5 .…”

Section: Introductionmentioning

confidence: 97%

SCN5A mutation identified in a patient with short‐coupled variant of torsades de pointes

Sonoda

Ohno

Shimizu

et al. 2020

Pacing Clinical Electrophis

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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 extrasystoles. 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. We identified an SCN5A mutation R800H in a 38-year-old male who suffered ventricular fibrillation during dinner and was resuscitated. Two months later, he lost his consciousness at work. His Holter electrocardiogram showed scTdP. He had no family history of sudden cardiac death or heart disease. Functional analysis of the SCN5A-R800H channels showed a significantly shortened recovery time from inactivation. Peak sodium current densities in SCN5A-R800H were larger than those in wild type but the difference was not statistically significant. Conclusions:We identified an SCN5A mutation in a scTdP patient and confirmed that the mutant channel caused the shortness of recovery time from inactivation. SCN5A might be a candidate gene for scTdP.

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Short-coupled variant of torsade de pointes

Cited by 25 publications

References 13 publications

Pregnancy and Short‐Coupled Torsades de Pointes

Pregnancy and Short‐Coupled Torsades de Pointes

Catecholaminergic polymorphic ventricular tachycardia: a rare cause of recurrent syncope

SCN5A mutation identified in a patient with short‐coupled variant of torsades de pointes

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