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Calmodulin mutation in long QT syndrome 15 associated with congenital heart defects further complicated by a functional 2:1 atrioventricular block: Management from foetal life to postpartum 长 QT 综合征 15 中的钙调素突变与先天性心脏缺陷有关,并因功能性 2:1 房室传导阻滞而进一步复杂化:从胎儿期到产后的管理。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.01.006
Elio Caruso , Silvia Farruggio , Paolo Guccione

We report a long QT syndrome 15 whose diagnosis was suspected during foetal life and confirmed at birth and was associated with congenital heart disease. Genetic testing revealed a rare mutation associated with the CALM2 gene. At 23 weeks of gestation, severe foetal sinus bradycardia (∼100 bpm) was detected. In the third trimester, the foetus developed severe right ventricular hypertrophy. At birth, the electrocardiogram showed a long QT interval of 640 ms, and after 1 hour, the newborn showed functional 2:1 atrioventricular block at ventricular rate of 50 bpm. After further pharmacological therapies, epicardial wires were surgically implanted for transient pacing in VVI mode at 90 bpm. Echocardiogram showed aneurysmatic left atrial appendage, dilated right segments, hypertrophied right ventricle, ostium secundum type atrial septal defect, and muscular ventricular septal defect. At two weeks of postpartum, a permanent dual-chamber pacemaker was implanted in the DDD mode and the patient was discharged with a prescription of beta-blockers and calcium therapy.

我们报告了一名长 QT 综合征 15 患儿,其诊断在胎儿时期就已被怀疑,并在出生时得到确诊,该患儿还伴有先天性心脏病。基因检测发现了一个与 CALM2 基因有关的罕见突变。妊娠 23 周时,发现胎儿出现严重的窦性心动过缓(100 bpm)。妊娠三个月时,胎儿出现严重的右心室肥大。出生时,心电图显示 QT 间期长达 640 毫秒,1 小时后,新生儿出现功能性 2:1 房室传导阻滞,心室率为 50 bpm。在进一步的药物治疗后,通过手术植入心外膜导线,以 90 bpm 的 VVI 模式进行短暂起搏。超声心动图显示左心房阑尾动脉瘤、右心房段扩张、右心室肥大、房间隔缺损和室间隔缺损。产后两周时,医生为患者植入了 DDD 模式的永久性双腔起搏器,并开出了β-受体阻滞剂和钙剂治疗处方。
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引用次数: 0
Ventricular dyssynchrony imaging, echocardiographic and clinical outcomes of left bundle branch pacing and biventricular pacing 左束支起搏和双心室起搏的心室不同步成像、超声心动图和临床结果。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.04.007
Auke A.A. Verstappen , Rick Hautvast , Pavel Jurak , Frank A. Bracke , Leonard M. Rademakers

Background

Left bundle branch pacing (LBBP) is a novel physiological pacing technique which may serve as an alternative to cardiac resynchronization therapy (CRT) by biventricular pacing (BVP). This study assessed ventricular activation patterns and echocardiographic and clinical outcomes of LBBP and compared this to BVP.

Methods

Fifty consecutive patients underwent LBBP or BVP for CRT. Ventricular activation mapping was obtained by ultra-high-frequency ECG (UHF-ECG). Functional and echocardiographic outcomes and hospitalization for heart failure and all-cause mortality after one year from implantation were evaluated.

Results

LBBP resulted in greater resynchronization vs BVP (QRS width: 170 ± 16 ms to 128 ± 20 ms vs 174 ± 15 to 144 ± 17 ms, p = 0.002 (LBBP vs BVP); e-DYS 81 ± 17 ms to 0 ± 32 ms vs 77 ± 18 to 16 ± 29 ms, p = 0.016 (LBBP vs BVP)). Improvement in LVEF (from 28 ± 8 to 42 ± 10 percent vs 28 ± 9 to 36 ± 12 percent, LBBP vs BVP, p = 0.078) was similar. Improvement in NYHA function class (from 2.4 to 1.5 and from 2.3 to 1.5 (LBBP vs BVP)), hospitalization for heart failure and all-cause mortality were comparable in both groups.

Conclusions

Ventricular dyssynchrony imaging is an appropriate way to gain a better insight into activation patterns of LBBP and BVP. LBBP resulted in greater resynchronization (e-DYS and QRS duration) with comparable improvement in LVEF, NYHA functional class, hospitalization for heart failure and all-cause mortality at one year of follow up.

背景左束支起搏(LBBP)是一种新型生理起搏技术,可作为双室起搏(BVP)心脏再同步化治疗(CRT)的替代方法。这项研究评估了 LBBP 的心室激活模式、超声心动图和临床结果,并将其与 BVP 进行了比较。通过超高频心电图(UHF-ECG)获得心室活化图。结果LBBP与BVP相比,其再同步化程度更高(QRS宽度:170±16 ms至128±20 ms vs 174±15 至144±17 ms,p = 0.002(LBBP vs BVP);e-DYS 81±17 ms至0±32 ms vs 77±18 至16±29 ms,p = 0.016(LBBP vs BVP))。LVEF 的改善(从 28 ± 8% 到 42 ± 10% vs 28 ± 9% 到 36 ± 12%,LBBP vs BVP,p = 0.078)相似。两组患者的 NYHA 功能分级(从 2.4 降至 1.5 和从 2.3 降至 1.5(LBBP vs BVP))、心衰住院率和全因死亡率的改善程度相当。随访一年后,LBBP 会导致更大程度的再同步化(e-DYS 和 QRS 持续时间),而 LVEF、NYHA 功能分级、心衰住院率和全因死亡率的改善程度相当。
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引用次数: 0
Erratum to “Absent ventriculo-atrial conduction during right ventricular apical pacing but nodal response during para-Hisian pacing – What is the mechanism?” [Indian Pacing Electrophysiol J 24 (1) (January–February 2024) 45–48] 右心室心尖起搏时心室-心房传导缺失,但副希氏起搏时出现结节反应--机制是什么?[印度起搏与电生理学杂志》第 24 卷第 1 期,2024 年 1-2 月,第 45-48 页]。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.05.006
Debabrata Bera , Sanjeev S. Mukherjee , Ashesh Halder , Saroj Kumar Choudhury
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引用次数: 0
The efficacy and safety of cryoballoon atrial fibrillation ablation in patients with heart failure: A systematic review and meta-analysis 冷冻球囊心房颤动消融术对心力衰竭患者的疗效和安全性:系统回顾和荟萃分析。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.01.001
Nithi Tokavanich , Witina Techasatian , Narut Prasitlumkum , Wisit Cheungpasitporn , Leenhapong Navaravong , Krit Jongarangsin , Ronpichai Chokesuwattanaskul

Introduction

While atrial fibrillation (AF) ablation has proven beneficial for heart failure (HF) patients, most reports were performed with radiofrequency ablation. We aimed to evaluate the efficacy and safety of cryoballoon AF ablation in patients with HFrEF.

Method

We comprehensively searched the databases of MEDLINE, EMBASE, and Cochrane database from inception to December 2022. Studies that reported the outcomes of freedom from atrial arrhythmia, complications, NYHA functional class (NYHA FC), and left ventricular ejection fraction (LVEF) after Cryoballoon AF ablation in HF patients were included. Data from each study were combined with a random-effects model.

Result

A total of 9 studies observational studies with 1414 HF patients were included. Five studies had only HF with reduced ejection fraction (HFrEF), 1 study with HF with preserved ejection fraction (HFpEF), and others with mixed HF types. Freedom from AA in HFrEF at 12 months was 64% (95% CI 56–71%, I2 58%). There was a significant improvement of LVEF in these patients with a standard mean difference of 13% (95% CI 8.6–17.5%, I2 99% P < 0.001. The complication rate in HFrEF group was 6% (95% CI 4–10%, I2 0%). The risk of recurrence of atrial arrhythmia was not significantly different between HF and no HF patients (RR 1.34, 95% CI 0.8–2.23, I2 76%).

Conclusion

Cryoballoon AF ablation is effective in HFrEF patients comparable to radiofrequency ablation. The complication rate was low.

导言:虽然心房颤动(AF)消融术已被证明对心力衰竭(HF)患者有益,但大多数报告都是通过射频消融术进行的。我们旨在评估冷冻球囊心房颤动消融术对心衰患者的疗效和安全性:我们全面检索了 MEDLINE、EMBASE 和 Cochrane 数据库中从开始到 2022 年 12 月的内容。方法:我们全面检索了自 2022 年 12 月起的 MEDLINE、EMBASE 和 Cochrane 数据库,纳入了报告 HF 患者冷冻球囊房颤消融术后无房性心律失常、并发症、NYHA 功能分级(NYHA FC)和左心室射血分数(LVEF)的研究。采用随机效应模型对每项研究的数据进行综合分析:结果:共纳入了9项观察性研究,1414名心房颤动患者。结果:共纳入9项观察性研究,1414名心房颤动患者,其中5项研究仅涉及射血分数降低的心房颤动(HFrEF),1项研究涉及射血分数保留的心房颤动(HFpEF),其他研究涉及混合型心房颤动。12 个月后,HFrEF 的 AA 自由度为 64% (95% CI 56-71%,I2 58%)。这些患者的 LVEF 有明显改善,标准平均差为 13% (95% CI 8.6-17.5%, I2 99% P 2 0%)。心房颤动患者和无心房颤动患者的房性心律失常复发风险无明显差异(RR 1.34,95% CI 0.8-2.23,I2 76%):结论:低温球囊房颤消融术对高频房颤患者的疗效与射频消融术相当。并发症发生率较低。
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引用次数: 0
Erratum regarding missing Ethical statements in previously published articles 关于以前发表的文章中缺少伦理声明的更正。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.04.003
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引用次数: 0
Erratum regarding missing Ethical statements in previously published articles 关于以前发表的文章中缺少伦理声明的更正。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.04.002
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引用次数: 0
Erratum regarding missing ethical statements in previously published articles 关于以前发表的文章中缺少伦理声明的更正。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.04.004
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引用次数: 0
Crazy accessory pathway- going round the bend! 疯狂的附属通道--拐弯处!
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.04.001
Abhinav B. Anand , Pratap J. Nathani , Yash Y. Lokhandwala

A 15-year-old boy with manifest preexcitation and recurrent palpitations had undergone an unsuccessful ablation procedure elsewhere and was subsequently referred to us. The ECG suggested a left free wall pathway but there was a pattern break in lead V2. This helped localise the accessory pathway to the summit region and achieve success.

一名 15 岁的男孩患有明显的心动过速和反复心悸,曾在其他地方接受过一次不成功的消融手术,后来转到我们这里。心电图显示为左侧游离壁通路,但在 V2 导联出现模式断裂。这有助于将附属通路定位在心尖区,并取得了成功。
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引用次数: 0
We promise to keep your candle burning 我们承诺让您的蜡烛继续燃烧
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.05.003
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引用次数: 0
Cardiac resynchronization therapy with conduction system pacing in a long-term heart transplant recipient: A case report 一名长期心脏移植受者的传导系统起搏心脏再同步治疗:病例报告。
Q3 Medicine Pub Date : 2024-05-01 DOI: 10.1016/j.ipej.2024.01.003
Simone Taddeucci , Carmine Marallo , Giacomo Merello , Amato Santoro

We performed cardiac resynchronization therapy by means of conduction system pacing in a heart transplant patient suffering from heart failure with reduced ejection fraction and atrial fibrillation with conduction disturbance (bifascicular block and QRS >160 ms). ECG monitoring showed paroxysmal atrioventricular block. Biventricular pacing was not feasible due to the absence of a suitable coronary sinus branch for pacing. His bundle pacing was performed, and an implantable cardioverter-defibrillator was implanted due to severe left ventricular dysfunction. Cardiac allograft vasculopathy was excluded. During follow-up, the patient's left ventricular function improved, and symptoms alleviated with a high percentage of ventricular stimulation.

我们通过传导系统起搏对一名心脏移植患者进行了心脏再同步化治疗,患者患有射血分数降低的心力衰竭和伴有传导障碍(双筋膜阻滞和 QRS >160ms)的心房颤动。心电图监测显示存在阵发性房室传导阻滞。由于没有合适的冠状窦分支,无法进行双室起搏。由于左心室功能严重障碍,他接受了束起搏,并植入了植入式心律转复除颤器。心脏同种移植血管病变被排除。随访期间,患者的左心室功能有所改善,高比例的心室刺激使症状得到缓解。
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引用次数: 0
期刊
Indian Pacing and Electrophysiology Journal
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