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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 contractility modulation (CCM) is a novel therapeutic approach for heart failure patients, which utilizes nonexcitatory electrical myocardial stimulation in the absolute refractory period of the cardiac cycle. This stimulation has been shown to increase contractility, leading to improved heart failure symptoms, functional status, and quality of life. CCM is FDA approved for heart failure patients with an LVEF between 25% and 45% who remained symptomatic despite optimal medical therapy and not candidate of cardiac resynchronization therapy. CCM offers expanded treatment options for heart failure patients who have continued symptoms while on optimal medical therapy.

We discuss a case of symptomatic atrial arrhythmia in a 31 year old man who had previously undergone extra-cardiac Fontan surgery and a MAZE procedure. Besides the peculiar anatomy that restricted percutaneous atrial access only by perforation through the extra-cardiac tunnel, changing cycle lengths of the tachycardua necessitated innovative mapping to define the electro-morphology of the arrhythmia.

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.

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.

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.

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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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.

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.

A 22-year-old man presented with aborted sudden cardiac arrest due to ventricular fibrillation and recurrent tachyarrhythmia related to a right inferoparaseptal accessory pathway. A previous electrophysiology study with both endocardial and epicardial ablation had failed. Repeat electrophysiology study demonstrated diffuse early activation on endocardial mapping but a focal earliest site during epicardial mapping. The successful ablation site showed a sharp QS in the unipolar electrogram with a contact force vector directed toward the visceral pericardium/myocardium and required careful catheter positioning away from the distal right coronary artery. Accessory pathway conduction was abolished within 4.9 seconds of radiofrequency delivery. This case highlights the importance of properly analyzing the electroanatomic map, detailed electrogram analysis, and procedural safety considerations during epicardial accessory pathway ablation.

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.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Remote patient monitoring (RPM) in patients with heart failure (HF) involves transmitting physiological data from devices to a health-care provider via a wireless connection with targeted interventions when values exceed the preset threshold. Devices used in telemonitoring range from weighing scales, blood pressure cuffs, and pulse oximeters to devices used to measure cardiac filling pressure and intrathoracic impedance using cardiac implantable electronic devices and wearables. Accordingly, RPM devices can potentially engage patients in their cardiovascular care and reduce the burden of HF in society.

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.

Ventricular arrhythmia remains a challenge in the heart failure population, resulting in significant morbidity and mortality despite modern heart failure therapies. Patients with heart failure who have ventricular arrhythmia experience morbidity related to electrical storm, cardiogenic shock, and device therapies and are at an increased risk for sudden cardiac arrest and death. The management of ventricular arrhythmia involves antiarrhythmic drugs, cardiac implantable electronic device, catheter ablation, and other adjunctive procedural management strategies. Early intervention can reduce recurrent ventricular arrhythmia, device therapies, and associated morbidity and mortality and is essential in the management of ventricular arrhythmia in the patient with heart failure.

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.

This case presents a 67-year-old man with familial hypertrophic cardiomyopathy, severe heart failure (LVEF 31%, NYHA III), and significant interatrial conduction delay. After ruling out cardiac resynchronization therapy due to unsuitable ventricular activation characteristics, a dual-chamber implantable cardioverter-defibrillator was implanted, using Bachmann bundle pacing for the atrial lead to optimize interatrial synchrony. BBP successfully corrected the IACD, evidenced by P-wave shortening and normalization. This led to remarkable clinical improvements within 1 month: LVEF increased from 31% to 49%, and NYHA class improved from III to II. Echocardiography confirmed enhanced left atrial-left ventricular coupling. This case demonstrates BBP combined with ICD as a novel and effective strategy for HCM patients with IACD, offering both sudden cardiac death protection and substantial hemodynamic and functional benefits by optimizing atrial activation and atrioventricular synchrony.