Pub Date : 2025-12-01Epub Date: 2025-11-11DOI: 10.1007/s00399-025-01121-6
Christopher Shepley, Stephen Allen, Vibeke Andersen
This article provides a comprehensive overview of the AuroraTM extravascular implantable cardioverter-defibrillator (EV-ICD) system for the treatment of potentially fatal ventricular arrhythmias. It details its hardware design, including the device and the lead, and examines software features such as antitachycardia pacing (ATP) and the suite of enhanced discriminators specifically designed to address potential sensing challenges with an anterior mediastinal lead. The report includes clinical data from the primary acute human studies, the Pivotal EV-ICD study, and the updated Enlighten Post-Approval Registry, highlighting safety and efficacy. The EV-ICD system represents a step toward minimizing transvenous lead-related complications while maintaining effective arrhythmia detection and therapy, offering a promising alternative for a broad spectrum of patients at risk of sudden cardiac death.
{"title":"A review of the extravascular implantable cardioverter-defibrillator system: technology, development, clinical outcomes, and global experience.","authors":"Christopher Shepley, Stephen Allen, Vibeke Andersen","doi":"10.1007/s00399-025-01121-6","DOIUrl":"10.1007/s00399-025-01121-6","url":null,"abstract":"<p><p>This article provides a comprehensive overview of the Aurora<sup>TM</sup> extravascular implantable cardioverter-defibrillator (EV-ICD) system for the treatment of potentially fatal ventricular arrhythmias. It details its hardware design, including the device and the lead, and examines software features such as antitachycardia pacing (ATP) and the suite of enhanced discriminators specifically designed to address potential sensing challenges with an anterior mediastinal lead. The report includes clinical data from the primary acute human studies, the Pivotal EV-ICD study, and the updated Enlighten Post-Approval Registry, highlighting safety and efficacy. The EV-ICD system represents a step toward minimizing transvenous lead-related complications while maintaining effective arrhythmia detection and therapy, offering a promising alternative for a broad spectrum of patients at risk of sudden cardiac death.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"313-320"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145490690","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01Epub Date: 2025-11-11DOI: 10.1007/s00399-025-01117-2
G Imnadze, K Harutyunyan, T Eitz, T Fink, Y Bocchini, M Didenko, E Akkaya, P Sommer
We are entering a new era of physiological pacing with left bundle branch area pacing (LBBAP), which is becoming an increasingly common in clinical routine. LBBAP has clear advantages over right ventricular (RV) and biventricular (BVP) pacing. Another option is to combine LBBAP and BVP, known as left bundle branch optimized (LOT) cardiac resynchronization therapy (CRT), which shortens the QRS duration more effectively than LBBAP and BVP alone. Implanting LOT CRT in patients with an ICD indication is particularly complicated. In this case, four leads are generally required. Additionally, the ICD device must have DF1/IS1 ports. Placing the ICD lead in the LBBAP position helps to eliminate the aforementioned problem and enables LOT CRTD to be performed with three leads. Furthermore, with an optimally stimulated QRS complex, CRTD therapy can be performed using two leads and a dual-chamber ICD device. With a VDD ICD lead, this therapy can be performed using just one lead and a VVI ICD device. To date, only a few publications have appeared on the implantation of ICD leads in the LBBAP position. Experience with the permanent implantation of standard ICD leads in the LBBAP position showed high implantation success rates and acceptable procedure and fluoroscopy durations. Most cases showed optimal ECG findings, such as shortened QRS duration, V6‑R wave peak time (V6RWPT) and/or V6-V1 interpeak interval. Defibrillation testing was successful in all but one of the patients published to date. To further develop this promising therapy, we need sheaths that have been developed and tested for this purpose (made from new materials and with different curves), as well as leads with fixed helixes and true bipolar sensing (to avoid atrial oversensing). Ideally, the new leads should have a narrow diameter, and the coils should be short yet sufficiently dimensioned. A VDD variant could also be an interesting option. Current ICD devices also need to be modernized to include the possibility of unipolar stimulation. From a clinical perspective, we should observe more patients over a longer period of time and focus on critical points.
{"title":"[ICD electrode implantation for left bundle branch area pacing].","authors":"G Imnadze, K Harutyunyan, T Eitz, T Fink, Y Bocchini, M Didenko, E Akkaya, P Sommer","doi":"10.1007/s00399-025-01117-2","DOIUrl":"10.1007/s00399-025-01117-2","url":null,"abstract":"<p><p>We are entering a new era of physiological pacing with left bundle branch area pacing (LBBAP), which is becoming an increasingly common in clinical routine. LBBAP has clear advantages over right ventricular (RV) and biventricular (BVP) pacing. Another option is to combine LBBAP and BVP, known as left bundle branch optimized (LOT) cardiac resynchronization therapy (CRT), which shortens the QRS duration more effectively than LBBAP and BVP alone. Implanting LOT CRT in patients with an ICD indication is particularly complicated. In this case, four leads are generally required. Additionally, the ICD device must have DF1/IS1 ports. Placing the ICD lead in the LBBAP position helps to eliminate the aforementioned problem and enables LOT CRTD to be performed with three leads. Furthermore, with an optimally stimulated QRS complex, CRTD therapy can be performed using two leads and a dual-chamber ICD device. With a VDD ICD lead, this therapy can be performed using just one lead and a VVI ICD device. To date, only a few publications have appeared on the implantation of ICD leads in the LBBAP position. Experience with the permanent implantation of standard ICD leads in the LBBAP position showed high implantation success rates and acceptable procedure and fluoroscopy durations. Most cases showed optimal ECG findings, such as shortened QRS duration, V6‑R wave peak time (V6RWPT) and/or V6-V1 interpeak interval. Defibrillation testing was successful in all but one of the patients published to date. To further develop this promising therapy, we need sheaths that have been developed and tested for this purpose (made from new materials and with different curves), as well as leads with fixed helixes and true bipolar sensing (to avoid atrial oversensing). Ideally, the new leads should have a narrow diameter, and the coils should be short yet sufficiently dimensioned. A VDD variant could also be an interesting option. Current ICD devices also need to be modernized to include the possibility of unipolar stimulation. From a clinical perspective, we should observe more patients over a longer period of time and focus on critical points.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"292-299"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12638341/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145490621","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01Epub Date: 2025-11-10DOI: 10.1007/s00399-025-01123-4
Carsten W Israel, Christian Butter
{"title":"[Update device therapy].","authors":"Carsten W Israel, Christian Butter","doi":"10.1007/s00399-025-01123-4","DOIUrl":"10.1007/s00399-025-01123-4","url":null,"abstract":"","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"277-279"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145483716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01Epub Date: 2025-10-30DOI: 10.1007/s00399-025-01118-1
Jürgen Kuschyk, Javed Butler, Timothy Fendler, Niraj Varma
Cardiac contractility modulation (CCM) represents an innovative device-based therapy for patients with heart failure (HF) who remain symptomatic despite optimal guideline-directed medical therapy (GDMT). It delivers biphasic, non-excitatory electrical signals during the absolute refractory period, enhancing myocardial contractility and relaxation. It improves calcium cycling and facilitates diastolic calcium reuptake. Therapeutic effects from key clinical studies and real-world experiences in HF with reduced ejection fraction (HFrEF) are reviewed and the safety profile is examined. The design and objectives of the ongoing AIM HIGHer clinical trial in HF patients with mildly reduced (HFmrEF) or preserved (HFpEF) ejection fraction are summarized. The design of the next-generation device combining CCM with defibrillator functionality (CCM-D) is presented. The objectives of the ongoing clinical study with CCM‑D, INTEGRA‑D, are discussed in detail.
心脏收缩性调节(CCM)是一种创新的基于设备的治疗心力衰竭(HF)患者,尽管有最佳的指导药物治疗(GDMT),但仍有症状。它在绝对不应期传递双相非兴奋性电信号,增强心肌收缩性和舒张性。它能改善钙循环,促进舒张期钙的再吸收。本文回顾了主要临床研究和实际经验对心力衰竭伴射血分数降低(HFrEF)的治疗效果,并检查了安全性。本文总结了正在进行的AIM HIGHer临床试验在射血分数轻度降低(HFmrEF)或保持(HFpEF)的HF患者中的设计和目标。介绍了结合CCM和除颤器功能(CCM- d)的下一代设备的设计。正在进行的CCM - D (INTEGRA - D)临床研究的目标进行了详细讨论。
{"title":"Cardiac contractility modulation: an update.","authors":"Jürgen Kuschyk, Javed Butler, Timothy Fendler, Niraj Varma","doi":"10.1007/s00399-025-01118-1","DOIUrl":"10.1007/s00399-025-01118-1","url":null,"abstract":"<p><p>Cardiac contractility modulation (CCM) represents an innovative device-based therapy for patients with heart failure (HF) who remain symptomatic despite optimal guideline-directed medical therapy (GDMT). It delivers biphasic, non-excitatory electrical signals during the absolute refractory period, enhancing myocardial contractility and relaxation. It improves calcium cycling and facilitates diastolic calcium reuptake. Therapeutic effects from key clinical studies and real-world experiences in HF with reduced ejection fraction (HFrEF) are reviewed and the safety profile is examined. The design and objectives of the ongoing AIM HIGHer clinical trial in HF patients with mildly reduced (HFmrEF) or preserved (HFpEF) ejection fraction are summarized. The design of the next-generation device combining CCM with defibrillator functionality (CCM-D) is presented. The objectives of the ongoing clinical study with CCM‑D, INTEGRA‑D, are discussed in detail.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"329-335"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12638370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145410734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01Epub Date: 2025-09-22DOI: 10.1007/s00399-025-01106-5
Carsten W Israel
The Clinical Consensus Statement of the European Society of Cardiology (ESC) updates expert opinions on conduction system pacing (CSP). It does not represent a focused update of the ESC pacemaker guidelines of 2021 since that would require the publication of new results of randomized trials. But an official statement became necessary because at the time of preparing the ESC guidelines on pacing and cardiac resynchronization, only very limited data on left bundle branch pacing were available rendering this form of pacing almost completely ignored in the recommendations. Second, daily implantation practice has changed substantially worldwide since the advent of CSP. Third, basically all international heart rhythm societies apart from the ESC have published new guidelines on the use CSP in 2023 which differ significantly from the European recommendations. The Clinical Consensus Statement on CSP of the ESC differs from the 2021 ESC guidelines only in nuances since ESC statutes do not allow that an ESC expert opinion paper substantially contradict current ESC guidelines. However, it is important for daily practice that CSP be recommended as a potential alternative to right ventricular pacing in patients with good left ventricular function or rare ventricular pacing is expected. Similarly, CSP is recommended as an alternative to biventricular pacing (BiVP) in reduced left ventricular function, in context with ablation of the atrioventricular node, in heart failure with wide QRS complex and for upgrading in pacing-induced cardiomyopathy. Finally, CSP is recommended in nonresponders to BiVP.
{"title":"[Comment on the European Society of Cardiology (ESC) Clinical Consensus Statement on the indication for conduction system pacing].","authors":"Carsten W Israel","doi":"10.1007/s00399-025-01106-5","DOIUrl":"10.1007/s00399-025-01106-5","url":null,"abstract":"<p><p>The Clinical Consensus Statement of the European Society of Cardiology (ESC) updates expert opinions on conduction system pacing (CSP). It does not represent a focused update of the ESC pacemaker guidelines of 2021 since that would require the publication of new results of randomized trials. But an official statement became necessary because at the time of preparing the ESC guidelines on pacing and cardiac resynchronization, only very limited data on left bundle branch pacing were available rendering this form of pacing almost completely ignored in the recommendations. Second, daily implantation practice has changed substantially worldwide since the advent of CSP. Third, basically all international heart rhythm societies apart from the ESC have published new guidelines on the use CSP in 2023 which differ significantly from the European recommendations. The Clinical Consensus Statement on CSP of the ESC differs from the 2021 ESC guidelines only in nuances since ESC statutes do not allow that an ESC expert opinion paper substantially contradict current ESC guidelines. However, it is important for daily practice that CSP be recommended as a potential alternative to right ventricular pacing in patients with good left ventricular function or rare ventricular pacing is expected. Similarly, CSP is recommended as an alternative to biventricular pacing (BiVP) in reduced left ventricular function, in context with ablation of the atrioventricular node, in heart failure with wide QRS complex and for upgrading in pacing-induced cardiomyopathy. Finally, CSP is recommended in nonresponders to BiVP.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"280-291"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145114748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-11-17DOI: 10.1007/s00399-025-01125-2
Arian Sultan, David Duncker, Henning Jansen, Micaela Ebert, Victoria Johnson, Till Althoff, Tillmann Maurer, Sascha Rolf, Christian Heeger, Heidi Estner, Andreas Rillig, Philipp Sommer, Leon Iden, K R Julian Chun, Sonia Busch, Roland Tilz, Tillmann Dahme, Daniel Steven
{"title":"Erratum zu: Kabellose Herzschrittmacher.","authors":"Arian Sultan, David Duncker, Henning Jansen, Micaela Ebert, Victoria Johnson, Till Althoff, Tillmann Maurer, Sascha Rolf, Christian Heeger, Heidi Estner, Andreas Rillig, Philipp Sommer, Leon Iden, K R Julian Chun, Sonia Busch, Roland Tilz, Tillmann Dahme, Daniel Steven","doi":"10.1007/s00399-025-01125-2","DOIUrl":"10.1007/s00399-025-01125-2","url":null,"abstract":"","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145543874","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-29DOI: 10.1007/s00399-025-01112-7
Tobias Tönnis, Nina Becher, Ralf Birkemeyer, David Duncker, Lars Eckardt, Klaus Gröschel, Dominik Linz, Christian Meyer, Florian Straube, Arian Sultan, Rolf Wachter, Maura Zylla, Paulus Kirchhof
Device-detected atrial fibrillation (DDAF), like clinical atrial fibrillation (AF), is also associated with an increased risk of thromboembolic events, although the risk appears to be significantly lower. The efficacy and safety of oral anticoagulation in DDAF were investigated in two large randomized trials (NOAH-AFNET 6 and ARTESIA [13, 19]). They showed a low rate of ischemic strokes without anticoagulation (about 1% per year). This risk can still be reduced by therapeutic anticoagulation, but the risk of severe bleeding increases. In the recently published position paper [36] of the German Society of Cardiology, the current study situation was presented and expert recommendations were given on how to deal with DDAF.
器械检测心房颤动(DDAF)与临床心房颤动(AF)一样,也与血栓栓塞事件的风险增加相关,尽管风险似乎明显较低。两项大型随机试验(NOAH-AFNET 6和ARTESIA[13,19])研究了口服抗凝治疗DDAF的疗效和安全性。结果显示,没有抗凝治疗的缺血性中风发生率很低(每年约1%)。这种风险仍然可以通过抗凝治疗来降低,但严重出血的风险会增加。在最近发表的德国心脏病学会(German Society of Cardiology)立场文件[36]中,介绍了目前的研究现状,并就如何应对DDAF给出了专家建议。
{"title":"[What to do with device-detected atrial fibrillation? : Executive summary of the position paper of the German Society of Cardiology].","authors":"Tobias Tönnis, Nina Becher, Ralf Birkemeyer, David Duncker, Lars Eckardt, Klaus Gröschel, Dominik Linz, Christian Meyer, Florian Straube, Arian Sultan, Rolf Wachter, Maura Zylla, Paulus Kirchhof","doi":"10.1007/s00399-025-01112-7","DOIUrl":"https://doi.org/10.1007/s00399-025-01112-7","url":null,"abstract":"<p><p>Device-detected atrial fibrillation (DDAF), like clinical atrial fibrillation (AF), is also associated with an increased risk of thromboembolic events, although the risk appears to be significantly lower. The efficacy and safety of oral anticoagulation in DDAF were investigated in two large randomized trials (NOAH-AFNET 6 and ARTESIA [13, 19]). They showed a low rate of ischemic strokes without anticoagulation (about 1% per year). This risk can still be reduced by therapeutic anticoagulation, but the risk of severe bleeding increases. In the recently published position paper [36] of the German Society of Cardiology, the current study situation was presented and expert recommendations were given on how to deal with DDAF.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145402836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-06DOI: 10.1007/s00399-025-01108-3
Christian-Hendrik Heeger, Henning Rolfes
The proposed introduction of outpatient electrophysiological procedures challenges physicians with the task of safely discharging patients after only a few hours of monitoring. Despite the widely established use of ultrasound-guided vascular access in many centers, access-site complications remain the most common complications associated with electrophysiological interventions. Modern vascular closure devices have made postprocedural care safer and more comfortable. Nevertheless, the traditional Z‑suture continues to play an important role, particularly in cases of individual anatomical challenges or as a fallback option in the event of device failure. The choice of the closure method should always be made on an individual basis, taking into account vascular access, patient safety, available resources, and physician expertise. This review article focuses on techniques and systems for the periprocedural management of vascular access in electrophysiology.
{"title":"[Vascular closure following electrophysiological procedures-a practical guide].","authors":"Christian-Hendrik Heeger, Henning Rolfes","doi":"10.1007/s00399-025-01108-3","DOIUrl":"https://doi.org/10.1007/s00399-025-01108-3","url":null,"abstract":"<p><p>The proposed introduction of outpatient electrophysiological procedures challenges physicians with the task of safely discharging patients after only a few hours of monitoring. Despite the widely established use of ultrasound-guided vascular access in many centers, access-site complications remain the most common complications associated with electrophysiological interventions. Modern vascular closure devices have made postprocedural care safer and more comfortable. Nevertheless, the traditional Z‑suture continues to play an important role, particularly in cases of individual anatomical challenges or as a fallback option in the event of device failure. The choice of the closure method should always be made on an individual basis, taking into account vascular access, patient safety, available resources, and physician expertise. This review article focuses on techniques and systems for the periprocedural management of vascular access in electrophysiology.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145233968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-09-01Epub Date: 2025-07-24DOI: 10.1007/s00399-025-01093-7
Harilaos Bogossian, Nana-Yaw Bimpong-Buta, Sebastian Robl, Konstantinos Iliodromitis
{"title":"[Modern WPW-syndrome treatment: from ECG via double-check-mapping to ablation].","authors":"Harilaos Bogossian, Nana-Yaw Bimpong-Buta, Sebastian Robl, Konstantinos Iliodromitis","doi":"10.1007/s00399-025-01093-7","DOIUrl":"10.1007/s00399-025-01093-7","url":null,"abstract":"","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"245-248"},"PeriodicalIF":0.0,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144700333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-09-01Epub Date: 2025-08-05DOI: 10.1007/s00399-025-01098-2
Ulrich Krause
Cardiac channelopathies are a group of hereditary diseases that expose affected children and adolescents to an increased risk of syncope and sudden cardiac death (SCD) due to malignant ventricular tachyarrhythmias. Although cardiac channelopathies are rare, with an estimated prevalence of 1:2000-1:10,000, early recognition in order to start treatment and prevent SCD is warranted. The following article provides an overview of current recommendations and facts on the diagnosis and treatment of cardiac channelopathies in children and adolescents. The most commonly encountered cardiac channelopathies during childhood and adolescence include long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada syndrome (BrS) and short QT syndrome (SQTS). While subjects with LQTS and CPVT commonly respond well to β‑blocker medication, and flecainide is an additional option in patients with CPVT, no such highly effective drug therapy exists for the treatment of patients with BrS or SQTS. Left cardiac sympathetic denervation is an additional treatment option in subjects with LQTS or CPVT. Implantable cardioverter-defibrillator implantation is indicated in patients with channelopathies and life-threatening ventricular tachyarrhythmias despite adequate antiarrhythmic medication.
{"title":"Management of channelopathies in children.","authors":"Ulrich Krause","doi":"10.1007/s00399-025-01098-2","DOIUrl":"10.1007/s00399-025-01098-2","url":null,"abstract":"<p><p>Cardiac channelopathies are a group of hereditary diseases that expose affected children and adolescents to an increased risk of syncope and sudden cardiac death (SCD) due to malignant ventricular tachyarrhythmias. Although cardiac channelopathies are rare, with an estimated prevalence of 1:2000-1:10,000, early recognition in order to start treatment and prevent SCD is warranted. The following article provides an overview of current recommendations and facts on the diagnosis and treatment of cardiac channelopathies in children and adolescents. The most commonly encountered cardiac channelopathies during childhood and adolescence include long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada syndrome (BrS) and short QT syndrome (SQTS). While subjects with LQTS and CPVT commonly respond well to β‑blocker medication, and flecainide is an additional option in patients with CPVT, no such highly effective drug therapy exists for the treatment of patients with BrS or SQTS. Left cardiac sympathetic denervation is an additional treatment option in subjects with LQTS or CPVT. Implantable cardioverter-defibrillator implantation is indicated in patients with channelopathies and life-threatening ventricular tachyarrhythmias despite adequate antiarrhythmic medication.</p>","PeriodicalId":52403,"journal":{"name":"Herzschrittmachertherapie und Elektrophysiologie","volume":" ","pages":"186-191"},"PeriodicalIF":0.0,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144785895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}