Herzschrittmachertherapie und Elektrophysiologie最新文献

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.

Introduction: Paediatric catheter ablation procedures have traditionally relied heavily on fluoroscopic guidance, exposing children to ionizing radiation and the associated long-term cancer risks ranging from 0.4 to 6.0% of total lifetime cancer risk. This necessitates technological innovations to minimize radiation dependency while maintaining therapeutic effectiveness.

Objectives: To review current state-of-the-art technologies and emerging innovations in paediatric catheter ablation.

Materials and methods: This review examines advanced three-dimensional (3D) mapping algorithms, including low voltage bridge mapping, late annotation electrograms, open window mapping, and omnipolar technology for substrate characterization. Intracardiac echocardiography and digital twin frameworks utilizing cardiac magnetic resonance imaging, computed tomography, and electrocardiogram data for personalized procedural planning are also analyzed.

Results: The implementation of advanced 3D mapping systems demonstrates a marked reduction in radiation exposure while optimizing procedural outcomes across structurally normal and complex congenital heart disease patients. Intracardiac echocardiography provides high-resolution real-time imaging, eliminating fluoroscopic dependence and enabling dynamic clinical decision-making during complex procedures. Focal pulsed field ablation may have a role in paediatric ablations, as it potentially reduces the risk of damaging the conduction tissue and other nearby structures.

Conclusions: The integration of advanced imaging technologies represents transformative progress in paediatric electrophysiology, enabling safer, more precise interventions with significantly reduced radiation exposure. These innovations establish new paradigms for personalized paediatric cardiac care, promising improved long-term outcomes for vulnerable populations requiring catheter ablation procedures.

Background and aim: Cardiac arrhythmias in children require early diagnosis and individualised treatment approaches that differ significantly from those used in adult medicine. This article reviews the role of antiarrhythmic drugs in acute and chronic clinical situations in children, focusing on practical use, safety and integration of non-pharmacological measures.

Results: Adenosine is the drug of choice for the acute treatment of supraventricular tachycardia. The aim of chronic drug prophylaxis is to achieve freedom from recurrence until the arrhythmia resolves spontaneously (more common in young children) or until elective catheter ablation is performed as a causal treatment for the arrhythmia. Several classes of antiarrhythmic drugs are used in long-term treatment. Amiodarone is usually reserved for complicated, treatment-resistant cases. When choosing a beta-blocker, the history of asthmatic-like symptoms in children should be taken into account and cardioselective beta-blockers should be chosen.

Discussion: Antiarrhythmic drugs are an essential part of the management of arrhythmias in children. Weight-adjusted dosing, close monitoring and age-appropriate use ensure safe and effective therapy. Multidisciplinary care and individualised strategies are essential for successful treatment.

Children, adolescents, and patients with congenital heart defects exhibit a unique and broad spectrum of arrhythmias. Diagnosis and treatment of these arrhythmias requires highly specialized medical care structures. In addition to highly qualified medical expertise, this includes appropriate antiarrhythmic drugs and medical devices specifically tailored to this patient group for catheter ablation and device therapy. The increasing economization of the healthcare system and legal requirements-particularly the EU Medical Device Regulation-are jeopardizing existing and urgently needed new care structures due to staff shortages, insufficient training capacities, and the discontinuation of production of relevant medical devices. The resulting threat to this patient group urgently necessitates reforms to the funding of existing highly specialized care structures, as well as more attractive framework conditions for the industry for the development and approval of antiarrhythmic drugs and medical devices specifically tailored to the needs of these patients.

Background: Electrical device therapy is increasingly used for paediatric patients with congenital and acquired arrhythmias and heart diseases.

Objectives: This review evaluates the indications, strategies and limitations of this therapy in the young. Pacing and defibrillation systems in young patients should last longer than in adults. The paediatric population ranges from preterm newborns to young adults, and therefore recommendations and pacing strategies are variable. Recent guidelines have provided new and updated recommendations. Various systems have enhanced our ability to treat arrhythmias. Transvenous and epicardial pacing systems compete for primacy among children; transvenous and subcutaneous defibrillators among adolescents. Outcomes are good, but there are frequent complications.

Conclusion: Electrical device therapy in the paediatric population is effective and safe, but it needs to be adapted to the age, size, and pathophysiology patients.

Background: Inherited arrhythmia syndromes and cardiomyopathies are among the most concerning causes of sudden cardiac death in young individuals, particularly in the context of physical activity. Historically, sports participation in these patients has been broadly restricted due to safety concerns. However, emerging data and updated guidelines suggest that a more individualized approach may be both appropriate and safe.

Objectives: The aim of this study was to review current evidence and evolving recommendations regarding sports participation in young individuals with inherited cardiac diseases.

Materials and methods: This review synthesizes recent studies, expert consensus statements, and current international guidelines (ESC, AHA/ACC).

Results: Recent data indicate that, in selected patients with inherited arrhythmia syndromes and cardiomyopathies who have undergone thorough evaluation and counseling, participation in sports-under appropriate precautions-may be safe and well tolerated. Emerging studies report low incidence of adverse events in appropriately managed athletes. Guidelines have shifted away from blanket restrictions and towards shared decision-making, especially in asymptomatic individuals or those with controlled disease. Key factors include genotype-phenotype correlation, history of arrhythmic events, treatment adherence, and patient/family understanding of risks.

Conclusion: In contrast to traditional dogma, a growing body of evidence supports less restrictive, patient-centered management for young individuals with inherited cardiac conditions. With proper evaluation, risk stratification, and informed decision-making, sports participation and leisure time activities may be possible-and even beneficial-for many of these patients.