Pediatric cardiomyopathies (CMs) and electrical diseases constitute a heterogeneous spectrum of disorders distinguished by structural and electrical abnormalities in the heart muscle, attributed to a genetic variant. They rank among the main causes of morbidity and mortality in the pediatric population, with an annual incidence of 1.1–1.5 per 100,000 in children under the age of 18. The most common conditions are dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). Despite great enthusiasm for research in this field, studies in this population are still limited, and the management and treatment often follow adult recommendations, which have significantly more data on treatment benefits. Although adult and pediatric cardiac diseases share similar morphological and clinical manifestations, their outcomes significantly differ. This review summarizes the latest evidence on genetics, clinical characteristics, management, and updated outcomes of primary pediatric CMs and electrical diseases, including DCM, HCM, arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), long QT syndrome (LQTS), and short QT syndrome (SQTS).
Atrial fibrillation, representing the most prevalent sustained cardiac arrhythmia, significantly impacts stroke risk and cardiovascular mortality. Historically managed with antiarrhythmic drugs with limited efficacy, and more recently, catheter ablation, the interventional approach field is still evolving with technological advances. This review highlights pulsed field ablation (PFA), a revolutionary technique gaining prominence in interventional electrophysiology because of its efficacy and safety. PFA employs non-thermal electric fields to create irreversible electroporation, disrupting cell membranes selectively within myocardial tissue, thus preventing the non-selective damage associated with traditional thermal ablation methods like radiofrequency or cryoablation. Clinical studies have consistently shown PFA’s ability to achieve pulmonary vein isolation—a cornerstone of AF treatment—rapidly and with minimal complications. Notably, PFA reduces procedure times and has shown a lower incidence of esophageal and phrenic nerve damage, two common concerns with thermal techniques. Emerging from oncological applications, the principles of electroporation provide a unique tissue-selective ablation method that minimizes collateral damage. This review synthesizes findings from foundational animal studies through to recent clinical trials, such as the MANIFEST-PF and ADVENT trials, demonstrating PFA’s effectiveness and safety. Future perspectives point towards expanding indications and refinement of techniques that promise to improve AF management outcomes further. PFA represents a paradigm shift in AF ablation, offering a safer, faster, and equally effective alternative to conventional methods. This synthesis of its development and clinical application outlines its potential to become the new standard in AF treatment protocols.
Background: Artificial Intelligence (AI) is a transformative innovation designed to enable machines to perform tasks typically requiring human intelligence. Among various medical fields, cardiology—and particularly electrophysiology—has seen rapid integration of AI technologies. The ability of AI to analyze large and complex datasets is reshaping diagnostic and therapeutic approaches. Objectives: This review aims to provide a comprehensive overview of AI models and their applications in cardiac electrophysiology. The focus is on understanding how AI contributes to clinical practice through ECG interpretation, arrhythmia detection, atrial mapping, and catheter ablation, while also exploring its limitations and future potential. Methods: The review discusses various AI approaches, including Machine Learning (ML) and Deep Learning (DL), and highlights relevant literature illustrating their implementation in electrophysiological settings. Key clinical applications are examined thematically, with a narrative synthesis of current capabilities, technologies, and outcomes. Results: AI-based tools have demonstrated effectiveness in identifying supraventricular arrhythmias like atrial fibrillation (AF) and atrial flutter (AFL), as well as complex conditions such as ventricular tachycardias (VTs) and long QT syndrome (LQTS). In procedural contexts, AI enhances electro-anatomical mapping, reduces operative time, and supports tailored post-ablation management. Discussion: While AI offers clear advantages in diagnostic accuracy and procedural efficiency, challenges remain regarding data security, ethical transparency, and clinical adoption. Addressing these limitations will be crucial for integrating AI into routine electrophysiology and maximizing its potential in future cardiology practice.
19 February 2025 in European Heart Journal Supplements
Heart failure (HF) represents one of the leading causes of morbidity and mortality worldwide. In recent years, remote monitoring (RM) and telemedicine have emerged as a promising strategy to improve the management of patients with HF, reducing hospitalizations and enhancing the quality of life. Through the integration of technologies such as implantable sensors, home monitoring devices, and mobile applications, it is possible to detect clinical changes early, enabling timely interventions. This article provides an overview of available technologies for RM in HF, analyses the clinical benefits observed in various studies, and addresses the remaining challenges, such as the need for standardization, long-term sustainability, and widespread adoption. Remote monitoring offers significant potential to improve clinical outcomes but requires further research and development to optimize its use in clinical practice.
Parenteral prostanoids are being recommended in pulmonary arterial hypertension (PAH) treatment, but the prognostic relevance of delayed treatment initiation is still debated. This study assessed the impact of the timing of prostacyclin treatment initiation on reducing PVR and achieving a low-risk profile in PAH patients. The study enrolled 151 patients who started on parenteral prostanoids with different treatment strategies. All patients underwent right heart catheterization, clinical evaluation, and risk assessments at baseline and after 1-year follow-up. Patients with an upfront strategy including parenteral prostanoid plus one oral drug had −5.3 ± 6.2 WU (−50 ± 19%) reduction in PVR, patients with an upfront strategy including parenteral prostanoid plus double oral drug had −12.8 ± 5.9 WU (−68 ± 17%) reduction in PVR, while patients with an add-on strategy including parenteral prostanoid after oral drugs had −3.9 ± 3.5 WU (−23 ± 19%) reduction in PVR. An upfront strategy including parenteral prostanoids was independently associated with an increased likelihood of achieving the greater reduction of PVR compared with an add-on strategy. Additionally, the greater the severity of PH at the time of diagnosis, in terms of PVR and RV reverse remodeling, the higher the probability of treatment failure. An upfront strategy including a parenteral prostanoid is associated with the highest likelihood of achieving a low-risk profile and a greater reduction of PVR compared with parenteral prostanoid as an add-on to oral treatment.