Dataset Information

Multifocal Purkinje-related premature contractions and electrical storm suppressed by quinidine and verapamil in a case with short-coupled ventricular fibrillation.

ABSTRACT: In some cases with idiopathic ventricular fibrillation, short-coupled premature ventricular contractions (PVCs) initiate fatal arrhythmia, which has recently been proposed as short-coupled ventricular fibrillation (SCVF). In the present case of SCVF, catheter ablation for trigger PVCs originating from the His-Purkinje system in both ventricles caused transient complete atrioventricular block, and a combination of quinidine and verapamil suppressed ventricular tachyarrhythmia. <Learning objective: In some cases with short-coupled ventricular fibrillation, a combination of quinidine and verapamil can suppress fatal ventricular tachyarrhythmia.>.

SUBMITTER: Kataoka N

PROVIDER: S-EPMC9169001 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Multifocal Purkinje-related premature contractions and electrical storm suppressed by quinidine and verapamil in a case with short-coupled ventricular fibrillation.

Kataoka Naoya N Nagase Satoshi S Okawa Keisuke K Aiba Takeshi T Kinugawa Koichiro K Kusano Kengo K

Journal of cardiology cases 20211230 6

In some cases with idiopathic ventricular fibrillation, short-coupled premature ventricular contractions (PVCs) initiate fatal arrhythmia, which has recently been proposed as short-coupled ventricular fibrillation (SCVF). In the present case of SCVF, catheter ablation for trigger PVCs originating from the His-Purkinje system in both ventricles caused transient complete atrioventricular block, and a combination of quinidine and verapamil suppressed ventricular tachyarrhythmia. <<b>Learning object ...[more]

PMID: 35685260

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Project description:BackgroundWe identified a novel SCN5A variant, E171Q, in a neonate with very frequent ectopy and reduced ejection fraction which normalized after arrhythmia suppression by flecainide. This clinical picture is consistent with multifocal ectopic Purkinje-related premature contractions (MEPPC). Most previous reports of MEPPC have implicated SCN5A variants such as R222Q that neutralize positive charges in the S4 voltage sensor helix of the channel protein NaV1.5 and generate a gating pore current.Methods and resultsE171 is a highly conserved negatively-charged residue located in the S2 transmembrane helix of NaV1.5 domain I. E171 is a key component of the Gating Charge Transfer Center, a region thought to be critical for normal movement of the S4 voltage sensor helix. We used heterologous expression, CRISPR-edited induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs), and molecular dynamics simulations to demonstrate that E171Q generates a gating pore current, which was suppressed by a low concentration of flecainide (IC50 = 0.71±0.07 µM). R222Q shifts voltage dependence of activation and inactivation in a negative direction but we observed positive shifts with E171Q. E171Q iPSC-CMs demonstrated abnormal spontaneous activity and prolonged action potentials. Molecular dynamics simulations revealed that both R222Q and E171Q proteins generate a water-filled permeation pathway that underlies generation of the gating pore current.ConclusionPreviously identified MEPPC-associated variants that create gating pore currents are located in positively-charged residues in the S4 voltage sensor and generate negative shifts in the voltage dependence of activation and inactivation. We demonstrate that neutralizing a negatively charged S2 helix residue in the Gating Charge Transfer Center generates positive shifts but also create a gating pore pathway. These findings implicate the gating pore pathway as the primary functional and structural determinant of MEPPC and widen the spectrum of variants that are associated with gating pore-related disease in voltage-gated ion channels.

Project description:ObjectiveFetal heart-rate irregularities occur in 1-2% of pregnancies and are usually caused by premature atrial contractions (PAC). Although PAC are considered benign, they may be associated with cardiac defects and tachyarrhythmia. We aimed to determine the incidence of congenital heart defects (CHDs) and complications in fetuses with PAC.MethodsThis was a systematic review and meta-analysis conducted in accordance with the PRISMA statement for reporting items for systematic reviews and meta-analyses. MEDLINE and EMBASE were searched from 1990 to June 2021 to identify studies on fetuses with PAC. The primary outcome was CHD; secondary outcomes were complications using the endpoints supraventricular tachyarrhythmia (SVT), cardiac failure and intrauterine fetal demise. Meta-analysis of proportions was performed, subdivided into high-risk and low-risk populations based on reason for referral. Pooled incidences with 95% CIs were calculated.ResultsOf 2443 unique articles identified, 19 cohort studies including 2260 fetuses were included. The pooled incidence of CHD in fetuses with PAC was 2.8% (95% CI, 1.5-4.1%), when 0.6% is the incidence expected in the general population. The pooled incidence of CHD was 7.2% (95% CI, 3.5-10.9%) in the high-risk population and 0.9% (95% CI, 0.0-2.0%) in the low-risk population. SVT occurred in 1.4% (95% CI, 0.6-3.4%) of fetuses diagnosed with PAC. Cardiac failure was described in 16 fetuses (1.4% (95% CI, 0.5-3.5%)), of which eight were CHD-related. Intrauterine fetal demise occurred in four fetuses (0.9% (95% CI, 0.5-1.7%)) and was related to CHD in two cases.ConclusionsOur findings suggest that the risk of CHD in fetuses with PAC is 4-5 times higher than that in the general population. CHD was present more frequently in the high-risk population. Consequently, an advanced ultrasound examination to diagnose PAC correctly and exclude CHD is recommended. Complications of PAC are rare but can result in fetal demise, thus weekly fetal heart-rate monitoring remains advisable to enable early detection of SVT and to prevent cardiac failure. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Dataset Information

Multifocal Purkinje-related premature contractions and electrical storm suppressed by quinidine and verapamil in a case with short-coupled ventricular fibrillation.

Publications

Multifocal Purkinje-related premature contractions and electrical storm suppressed by quinidine and verapamil in a case with short-coupled ventricular fibrillation.

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