Pediatric arrhythmias

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter defibrillators (ICDs) are commonly advised. However, there are limited data on the outcomes of ICD use in children. OBJECTIVE: To compare the risk of arrhythmic events in pediatric CPVT patients with and without ICDs. METHODS: We compared the risk of SCD in RYR2 variant and phenotype positive symptomatic CPVT patients with and without ICDs, who were <19 years of age and had no history of sudden cardiac arrest (SCA) at phenotype diagnosis...

BACKGROUND: Diagnosing myocarditis in children presenting with complete AV block (CAVB) is challenging. Temporary permanent pacing support using standard transvenous active fixation lead can be inserted percutaneously until recovery. However, access to cardiac magnetic resonance (CMR) imaging may be limited due to safety concerns. CASES: We report three cases where CMR was performed using temporary permanent pacemaker in situ. We evaluated the effect of device artefacts on image quality and examined any instances of device malfunction...

BACKGROUND: Arrhythmia is one of the most common complications after cardiac surgery. The objectives of this study were to determine the prevalence and analyze the risk factors of postoperative arrhythmia in pediatric patients after cardiac surgery for congenital heart defects (CHD) at a single center in Bali, Indonesia over 2 years period. METHODS: A cross-sectional study, among 120 pediatric patients with CHD who underwent cardiac surgery, 92 patients met inclusion criteria in this study...

OBJECTIVE: To explore the effects of a multidisciplinary team (MDT) continuous care model on psychological behavior and quality of life in children with β-thalassemia major (β-TM). METHODS: A retrospective study was conducted on 90 children with β-TM who were hospitalized in the Department of Pediatrics, Affiliated Hospital of Youjiang Medical University for Nationalities from October 2021 to May 2022. According to nursing methods, the children were divided into a routine group and an experimental group, with 45 cases in each group...

INTRODUCTION: Long QT syndrome (LQTS) is a common congenital cause of fatal cardiac arrhythmia. Characteristic clinical findings are prolonged QT interval and ventricular arrhythmia on electrocardiogram (ECG), syncope, seizure, and sudden death. It is a genetically heterogeneous disease. To date, disease-causing variant have been reported in seventeen genes. The AKAP9 is still considered controversial among those genes. CASE REPORT: We report the case of a 10-year-old female who was born from a non-consanguineous Turkish couple...

BACKGROUND: Annually 15,200 children suffer an in-hospital cardiac arrest (IHCA) in the US. Ventricular fibrillation or pulseless ventricular tachycardia (VF/pVT) is the initial rhythm in 10-15% of these arrests. We sought to evaluate the association of number of shocks and early dose escalation with survival for initial VF/pVT in pediatric IHCA. METHODS: Using 2000-2020 data from the American Heart Association's (AHA) Get with the Guidelines® -Resuscitation (GWTG-R) registry, we identified children > 48 hours of life and ≤ 18 years who had an IHCA from initial VF/pVT and received defibrillation...

BACKGROUND: Acute myocarditis is an inflammatory condition that may precede the development of dilated or arrhythmogenic cardiomyopathy. OBJECTIVES: The aim of this study was to investigate the reported prevalence of pathogenic or likely pathogenic (P/LP) variants in cardiomyopathy-associated genes in patients with acute myocarditis. METHODS: For this systematic review and meta-analysis, the PubMed and Embase databases were searched on March 4, 2023...

BACKGROUND: Premature ventricular contractions (PVCs) are relatively common arrhythmias in the pediatric population, with implications that range from benign to potentially life-threatening. The management of PVCs in children poses unique challenges, and recent advancements in diagnostic and therapeutic options call for a comprehensive review of current practices. METHODS: This review synthesizes the latest literature on pediatric PVCs, focusing on publications from the past decade...

OBJECTIVE: Pediatric heart failure is an important cause of morbidity and mortality in childhood. Left ventricular assist devices (L-VAD) are used for bridging to transplantation in patients with indications for heart transplantation. PATIENTS AND METHODS: The children included in the study were patients who underwent implantation of an L-VAD due to advanced heart failure at Ege University Faculty of Medicine Hospital between January 2009 and January 2023. RESULTS: Of the 33 patients who underwent L-VAD implantation, 16 (48...

Congenital long QT syndrome (LQTS) is an inherited arrhythmia syndrome associated with sudden cardiac death. Accurate interpretation and classification of genetic variants in LQTS patients are crucial for effective management. All patients with LQTS with a positive genetic test over the past 18 years (2002-2020) in our single tertiary pediatric cardiac center were identified. Reevaluation of the reported variants in LQTS genes was conducted using the American College of Genetics and Genomics (ACMG) guideline after refinement by the US ClinGen SVI working group and guideline by Walsh et al...

Objective : AtriAmp is a new medical device that displays a continuous real-time atrial electrogram on telemetry using temporary atrial pacing leads. Our objective was to evaluate early adoption of this device into patient care, understand how it affected clinical workflow, and identify unforeseen benefits or limitations. Design : Qualitative study using inductive analysis of semi-structured interviews to identify dominant themes Setting : Single center, tertiary, academic 21-bed mixed pediatric intensive care unit (PICU) Subjects : PICU multidisciplinary team members (Pediatric Intensivists, PICU Nurse Practitioners, PICU nurses and Pediatric Cardiologists) who were early adopters of the AtriAmp (n=14) Results : Three prominent themes emerged from qualitative analysis of the early adopters' experiences...

INTRODUCTION: Children receiving extracorporeal membrane oxygenation are prone to delirium. This case report describes the nursing care of a child with delirium who received venoarterial extracorporeal membrane oxygenation. Relevant interventions and precautions are also discussed. CLINICAL FINDINGS: A 6-year-old girl was admitted to the pediatric intensive care unit with a 2-day history of vomiting and fever. The child underwent cannulation for venoarterial extracorporeal membrane oxygenation...

BACKGROUND: The stretching of the lead caused by somatic growth may lead to complications (dislodgement, fracture, failure) of transvenous leads implanted in pediatric patients. Atrial loop and absorbable ligatures may prevent it. Periodical lead advancement with lead pushing from the pocket may be an option to growth-induced stretching. Our aim was to analyze retrospectively the outcome of periodical transvenous lead advancement in children with pacemaker (PM). METHODS: A procedure of lead advancement was performed in patients with a single-chamber PM implanted for isolated congenital complete atrioventricular block or sinus node dysfunction with growth-induced lead straightening/stretching...

Introduction: We report a case of accelerated idioventricular rhythm (AIVR) identified by Emergency Medical Services (EMS) monitoring of an infant presenting with lethargy and respiratory distress. Accelerated idioventricular rhythms are rare ventricular rhythms originating from the His-Purkinje system or ventricular myocytes, consisting of >3 monomorphic beats with gradual onset and termination. 1 An AIVR is usually well-tolerated and does not require treatment, though sustained arrythmia may induce syncope, and the rhythm has been seen in newborn infants with congenital heart diseases...

Mitral annular disjunction (MAD) is a rare and under-recognized entity in the pediatric population. We present 2 cases of MAD in previously healthy pediatric patients and highlight clinical scenarios where MAD should be suspected.

TNNI3 is a gene that causes hypertrophic cardiomyopathy (HCM). A 14-year-old girl who was diagnosed with nonobstructive HCM presented with cardiopulmonary arrest due to ventricular fibrillation. Genetic testing revealed a novel de novo heterozygous missense variant in TNNI3, NM_000363.5:c.583A>T (p.Ile195Phe), which was determined to be the pathogenic variant. The patient exhibited progressive myocardial fibrosis, left ventricular remodeling, and life-threatening arrhythmias. Genetic testing within families is useful for risk stratification in pediatric HCM patients...

Purpose : Myocarditis is frequently a sporadic disease, but may also occur in the context of genetic disorders which may increase susceptibility to cardiac inflammation. Cardiac involvement in Wolfram syndrome type 1 (WS1) has been scarcely characterized. To our knowledge, no cases of virus-negative myocarditis have been reported in the WS1 pediatric population. Methods : We report the description of a pediatric case of acute myocarditis in the context of WS1, followed by a literature review of cardiovascular involvement associated with wolframin variants, and discuss potential pathophysiological mechanisms and therapeutic options...

Brugada Syndrome is an "inherited" channelopathy characterized by a predisposition to syncope and sudden death. It typically presents in young adults but is also known to affect the pediatric population, even if the prevalence is low compared to the adult population. The diagnostic ECG pattern shows coved-type ST-segment elevation in the right precordial leads, occurring spontaneously or after provocative drug tests with IV administration of Class I antiarrhythmic drugs. However, the electrocardiographic findings may vary, and transient or concealed forms of the syndrome further complicate diagnosis, necessitating thorough evaluation and close clinical follow-up...

Objective: To summarize the clinical manifestations, experiences in diagnosis and treatment of arrhythmogenic cardiomyopathy (ACM) in children. Methods: A retrospective analysis of the clinical manifestations, laboratory tests, radiological features, treatment and follow-up results was conducted in 11 children diagnosed with ACM at the center of congenital heart disease, Beijing anzhen hospital from May 2010 to March 2022. Results: A total of 11 patients aged 2 to 16 years, including 5 males and 6 females were diagnosed with ACM...

BACKGROUND: Isolated mitral valve aneurysm is rarely reported in children. In most cases it is associated with an underlying disease such as infective endocarditis. MVA can lead to severe complications that needs surgical intervention. CASE: In this report, we present a 9-year old asymptomatic male patient with anterior mitral valve aneurysm and rhythm disturbance diagnosed incidentally during pre-operative evaluation. CONCLUSIONS: Being rare in children, isolated MVA should be kept in mind in the differential diagnosis of mass lesions seen on the atrial side of the mitral valve...