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Pediatric cardiac arrest in the emergency department is rare. We emphasize the importance of preparedness for pediatric cardiac arrest and offer strategies for the optimal recognition and care of patients in cardiac arrest and peri-arrest. This article focuses on both prevention of arrest and the key elements of pediatric resuscitation that have been shown to improve outcomes for children in cardiac arrest. Finally, we review changes to the American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care that were published in 2020.

Cardiac amyloidosis is an infiltrative myocardial disease whose prevalence significantly increased in recent years. Its clinical history is changing due to the advent of novel therapies, and careful risk stratification has become impelling. Arrhythmias, frequently found during the course of the disease, include conduction system disease, atrial fibrillation (AF), and ventricular arrhythmias (VAs). Arrhythmic risk stratification is a key to identify those patients at higher risk to develop advanced atrioventricular blocks, asymptomatic AFs, or major VAs. This carries significant clinical implications concerning the indication and timing of implantable cardiac devices, as well as the initiation of anticoagulation therapy.

Cardiac electrophysiology (EP) procedures, such as pacemaker and implantable cardioverter-defibrillator (ICD) implantations, are increasingly shifting from inpatient hospitals to outpatient settings. Ambulatory surgery centers (ASCs) may offer cost advantages over hospital outpatient departments (HOPDs), but national trends and payment differences in the Medicare population remain undercharacterized. This study aimed to evaluate trends in site-of-service utilization and Medicare payment differences for EP device implantation procedures between 2016 and 2023. This cross-sectional study analyzed 100% Medicare fee-for-service claims data from 2016 to 2023 using the Physician/Supplier Procedure Summary files and 2023 payment data from the Medicare Procedure Price Lookup Tool. Pacemaker and ICD implantations were identified using Current Procedural Terminology codes and categorized by site of service: inpatient, HOPD, or ASC. Annual procedural volumes were normalized per 10,000 Medicare Part B beneficiaries. Linear regression was used to assess volume trends and ASC uptake. Payment comparisons between ASC and HOPD settings were performed for 5 representative procedures. From 2016 to 2023, pacemaker volumes declined from 47.2 to 44.2 per 10,000 beneficiaries, and ICD volumes declined from 22.8 to 16.6 per 10,000. Concurrently, ASC utilization increased from 1.5% to 7.2% for pacemakers and from 1.4% to 6.9% for ICDs (P < .001 for both trends). In 2023, Medicare reimbursed 15%-26% less for device implantations performed in ASCs compared with HOPDs. For example, dual-chamber pacemaker implantation (Current Procedural Terminology code 33208) cost $8131 in ASCs vs $10,673 in HOPDs. Aggregate Medicare savings from shifting 5 EP procedures to ASCs totaled $59.3 million in 2023. The use of ASCs for EP device implantation is increasing and is associated with substantially lower Medicare payments without affecting physician reimbursement. These findings highlight opportunities for cost savings and support the case for expanded ASC utilization and site-neutral payment reform.

Congenital long QT syndrome is the most common inherited arrhythmia syndrome and is characterized by prolongation of the QT interval in the absence of structural heart disease or external factors. Disease will often manifest during childhood resulting in life-threatening arrhythmias or sudden cardiac death. Diagnosis involves detailed personal and family history in combination with clinical testing. Genetic testing has a high diagnostic yield, though a negative result does not rule out the presence of disease. The incidence of arrhythmia events is significantly decreased with trigger avoidance and appropriate medical therapy allowing sports participation in the majority of patients.

Short QT Syndrome is a rare inherited arrhythmia disorder marked by accelerated repolarization and a short QT interval. It carries a high risk of atrial and ventricular arrhythmias, including sudden cardiac arrest. Genetic mutations are identified in 20% to 30% of cases, most commonly in potassium channel genes (KCNH2, KCNQ1, KCNJ2). Management includes ICD implantation for secondary prevention and antiarrhythmic therapy, with quinidine being the most established agent for QT prolongation and arrhythmia suppression.

This review intends to summarize the present landscape of translating electrophysiologic therapies designed for adults to the pediatric and congenital heart disease (CHD) populations. Unique challenges faced by pediatric and congenital electrophysiologists with respect to patient size, cardiac anatomy, and arrhythmia substrate are explored. An analysis of presently relevant key technologies at the forefront of adult electrophysiologic innovation is performed, along with a discussion of their potential utilities in the pediatric and CHD populations.

The ryanodine receptor-related inherited-cardiac syndromes are a group of disorders characterized predominantly by variants associated with both gain of function and loss of function in the ryanodine receptor type 2 (RyR2) gene. The prototypical RyR2 channelopathy is catecholaminergic polymorphic ventricular tachycardia (CPVT) whereas exon 3 deletion syndrome and calcium release deficiency syndrome (CRDS) are rare with comparatively less data on diagnosis, treatment, and prognosis. These syndromes are linked by the gene involved, with CPVT being the dominant condition recognized, which probably contributed to misdiagnoses of other unique conditions like CRDS.

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Electrosurgery has emerged as a groundbreaking tool in the field of structural cardiac interventions, revolutionizing the approach to complex cardiac conditions. This review delves into the core principles, procedural techniques, outcomes, and potential challenges associated with various electrosurgical procedures within the realm of structural cardiology. Five key electrosurgical procedures performed in complex structural interventions are highlighted in this review. They are the Transcaval Access, BASILICA, LAMPOON, ELASTIC/ELASTA-Clip, and SESAME procedures. While these electrosurgery procedures hold promise and have demonstrated positive outcomes, their technical intricacies, patient selection criteria, and the need for further research remain important considerations. As technology continues to evolve and more data becomes available, electrosurgery is poised to continue shaping the landscape of cardiac care, offering minimally invasive alternatives, and improving patient outcomes in complex structural cardiac interventions.

In the pediatric cardiology clinic, Brugada syndrome (BrS) most commonly presents through cascade screening following the diagnosis of a parent or sibling. However, some children may present for evaluation of syncope, or with documented atrial or ventricular arrhythmias. Unlike in adults, pediatric patients are frequently asymptomatic and require individualized risk stratification based on electrocardiogram findings, clinical history, and genetic data. The cardiology outpatient setting plays a pivotal role in guiding longitudinal care, including anticipatory counseling and decisions regarding rhythm monitoring or device implantation. This article offers a practical framework for managing BrS in children and adolescents from a clinical perspective.

Real-world data on atrial fibrillation (AF) contemporary management from the Gulf region are scarce and nonexistent from Kuwait. Despite recent guideline updates favoring early rhythm control, clinical characteristics, treatment strategies, and outcomes remain poorly characterized in this region. This study aimed to evaluate AF management patterns at a tertiary center in Kuwait, focusing on rhythm vs. rate control strategies and direct oral anticoagulant (DOAC) prescribing practices. An observational study on adult patients diagnosed with AF at Al Dabbous Cardiac Center between 2013 to 2022 was conducted. Demographic data, relevant clinical characteristics, and investigations were extracted. The primary outcome was the prevalence and predictors of rhythm control. Secondary outcomes included all-cause and cardiovascular-related hospitalizations. The appropriateness of DOAC dose-reduction was assessed based on guideline-based criteria. Of 558 records screened, 457 patients with non-valvular AF were included. Rhythm control was pursued in 28.9% of cases, most commonly via direct current cardioversion (47.3%). Patients selected for rhythm control were more likely to be younger (mean age 64 vs. 70&#x2009;years, p&#x2009;<&#x2009;0.001), male (61.1% vs. 38.9%, p&#x2009;=&#x2009;0.024), and followed in electrophysiology clinics (33% vs. 5%, p&#x2009;<&#x2009;0.001). Rhythm control strategy was independently associated with greater odds of cardiovascular-related hospitalizations (adjusted OR&#x2009;=&#x2009;2.89, 95% CI: 1.12-7.48, p&#x2009;=&#x2009;0.028). Among patients receiving reduced-dose DOACs (68.8%), 11.6% of patients were underdosed despite the absence of criteria for dose reduction. Rhythm control was underutilized in our study population, falling short of international benchmarks and guideline recommendations. Disparities in rhythm control utilization based on sex and referral patterns were observed. Additionally, among patients on dose-reduced DOACs, a significant proportion of them were underdosed. These findings highlight the need to enhance awareness of evidence-based AF management and to promote adherence to guideline-directed therapy.

Antiarrhythmic medications play a crucial role in the acute management of ventricular arrhythmias. Determining the most appropriate, effective, and safe antiarrhythmic medication to use in each clinical scenario requires careful consideration of the underlying disease, rhythm, and clinical presentation. Important initial distinctions to be made in the acute management of ventricular tachycardia (VT) are whether structural heart disease is present and if the patient has decompensated heart failure and borderline cardiac output, which makes the use of first-line agent beta blockers hazardous. In those with borderline or low cardiac output intravenous amiodarone is the favored agent for suppression of VT.

Genetic analysis using massive parallel sequencing is crucial for the accurate and early diagnosis of hereditary hypertrophic cardiomyopathies and their phenocopies, especially transthyretin cardiac amyloidosis (ATTR-CA) and Fabry disease (FD). This study extends the cardio next-generation sequencing (NGS) pilot study by investigating the detection rate of gene variants causing increased left ventricular wall thickness (LVWT) using an expanded 19-gene NGS panel in a larger global cohort. This study included 2068 patients with unexplained increased LVWT enrolled at cardiological clinics across 22 countries/regions between 2020 and 2022. The NGS panel comprised 19 genes associated with hypertrophic cardiomyopathy (HCM) and its phenocopies. Sequencing was performed using the Illumina NextSeq 500 and NovaSeq 6000 systems, with variant interpretation performed according to the American College of Medical Genetics and Genomics guidelines. Novel variants were analyzed using the Human Gene Mutation Database (HGMD&#xae;), Franklin, and VarSome. Among the 2068 patients, 453 patients were positive for pathogenic/likely pathogenic variants (21.9%). The diagnostic yield for HCM was 18.4%, while that of HCM phenocopies was 3.5%, including ATTR-CA (1.5%), and FD (0.9%). In patients with a positive test (HCM or HCM phenocopies), the most prevalent HCM-related variants were MYBPC3 and MYH7 (36.4% and 34.4% of all positive samples, respectively), whereas TTR (7.1%) and GLA (4.0%) were the most common phenocopy variants. Other classical phenocopies, Noonan syndrome, Danon disease, and PRKAG2, comprised another 2.0%, 0.9%, and 0.7% of the cohort, respectively. The mean ages for patients with HCM sarcomeric gene variants, HCM phenocopy variants, FD, and ATTR-CA were 45.1&#x2009;&#xb1;&#x2009;17.6, 50.9&#x2009;&#xb1;&#x2009;23.7, 51.1&#x2009;&#xb1;&#x2009;19.4, and 64.6&#x2009;&#xb1;&#x2009;19.0 years, respectively. This study demonstrates the need to include GLA and TTR in NGS panels for patients with increased unexplained LVWT. NGS effectively identifies phenocopies often missed by imaging. Using a large, diverse cohort, this study reveals the prevalence of FD and ATTR-CA in patients with unexplained increased LVWT, reinforcing the importance of NGS for early diagnosis and targeted therapy. The online version contains supplementary material available at 10.1186/s12872-026-05834-5.

Cardiac imaging is pivotal in evaluating ventricular function, residual lesions, and long-term complications in patients with adult congenital heart disease (ACHD). Longitudinal imaging in ACHD is key for the timely identification of patients requiring evaluation for advanced therapies. The guidelines recommend routine imaging surveillance. In all patients undergoing evaluation with cardiac imaging, it is critical that studies are performed at centers with expertise and that the imaging protocols are tailored to the specific condition. The authors briefly highlight the utility and diagnostic yield of different modalities, review pertinent considerations for special populations, and focus on imaging for transplant planning.

Pharmacologic management remains indispensable in supraventricular tachycardia despite ablation's curative potential. In stable patients, vagal maneuvers precede drugs; adenosine's ultrashort action makes it first-line, with intravenous calcium-channel or &#x3b2;-blockers and procainamide selected by QRS morphology and accessory-pathway risk. Chronic suppression relies on atrioventricular-nodal agents or class IC/III drugs selected by cardiac substrate and comorbidities, while special populations demand dose and drug modifications. Emerging self-administered therapies-particularly intranasal etripamil and selective A1 agonists-promise convenient, rapid conversion outside hospital settings. An individualized, guideline-driven strategy balancing efficacy, safety, and patient preference remains central to optimal supraventricular tachycardia care.

This review explores the ionic and cellular mechanisms that underlie antiarrhythmic drug therapy, focusing on how modulation of ionic currents influences cardiac action potentials and refractory periods. Reentrant arrhythmias, the most common type of tachyarrhythmias, are managed by strategies that prolong the effective refractory period. The review also discusses the proarrhythmic risks associated with use-dependence and reverse use-dependence of ion channel blockers. Special attention is given to J-wave syndromes, where Ito blockade plays a pivotal role in preventing phase 2 reentry and polymorphic ventricular tachycardia. Mechanistic understanding is essential for enhancing drug safety and therapeutic efficacy in clinical practice.

Patent foramen ovale (PFO) is a common finding in the general population but may lead to serious conditions such as stroke from paradoxical embolism and platypnea orthodeoxia. A thorough understanding of the interatrial septal anatomy along with its assessment by different imaging modalities is critical in performing safe transcatheter PFO closure. Investigating the anatomy and right heart to left heart flows using transesophageal echocardiography or intracardiac echocardiography imaging must be done before undertaking closure of a PFO.

Critical care principles and techniques continue to hold promise for improving patient outcomes in time-dependent diseases encountered by emergency medical services such as cardiac arrest, acute ischemic stroke, and hemorrhagic shock. In this review, the authors discuss several current and evolving advanced critical care modalities, including extracorporeal cardiopulmonary resuscitation, resuscitative endovascular occlusion of the aorta, prehospital thrombolytics for acute ischemic stroke, and low-titer group O whole blood for trauma patients. Two important critical care monitoring technologies-capnography and ultrasound-are also briefly discussed.

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