Indications for Cardiac Resynchronization Therapy in Patients with Congenital Heart Disease.

Heart failure in patients with congenital heart disease (CHD) stems from unique causes compared with the elderly. Patients with CHD face structural abnormalities and malformations present from birth, leading to altered cardiac function and potential complications. In contrast, elderly individuals primarily experience heart failure due to age-related changes and underlying cardiovascular conditions.

2023 HRS/APHRS/LAHRS guideline on cardiac physiologic pacing for the avoidance and mitigation of heart failure.

Cardiac physiologic pacing (CPP), encompassing cardiac resynchronization therapy (CRT) and conduction system pacing (CSP), has emerged as a pacing therapy strategy that may mitigate or prevent the development of heart failure (HF) in patients with ventricular dyssynchrony or pacing-induced cardiomyopathy. This clinical practice guideline is intended to provide guidance on indications for CRT for HF therapy and CPP in patients with pacemaker indications or HF, patient selection, pre-procedure evaluation and preparation, implant procedure management, follow-up evaluation and optimization of CPP response, and use in pediatric populations. Gaps in knowledge, pointing to new directions for future research, are also identified.

Efficacy of Infliximab Biosimilar for Maintenance Therapy in Pediatric Inflammatory Bowel Disease Following Infliximab Originator.

Unlabelled: The safety, efficacy, and cost-effectiveness of Infliximab biosimilar agents in the management of inflammatory bowel disease in adults have been shown. These agents have been recommended for pediatric inflammatory bowel disease, and although institutions are initiating therapy with the biosimilar agents (IFX-B), few are switching maintenance therapy from the originator (IFX-O). The aim was to compare biochemical markers of disease activity of children with inflammatory bowel disease on maintenance therapy with IFX-B to their previous markers on IFX-O.

Ventricular Functional Analysis in Congenital Complete Heart Block Using Speckle Tracking: Left Ventricular Epicardial Compared to Right Ventricular Septal Pacing.

Background: Chronic right ventricular (RV) apical pacing in patients with congenital complete atrioventricular block (CCAVB) is associated with left ventricle (LV) dyssynchrony and dysfunction. Hence, alternative pacing sites are advocated. The aim of this study was to compare LV function using STE in selected patients with LV epicardial pacing (LVEp) vs.

Assessing Non-invasive Studies to Evaluate Resynchronization Pacing Effectiveness in the Young.

Appropriate non-invasive assessments (ECHO/ECG) of cardiac resynchronization pacing therapy (CRT) among younger patients (pts) with/without (w/wo) congenital heart disease (CHD) are not established. Ejection fraction (EF) and QRS can be unreliable due to anatomy, surgical repairs, and pre-existing pacemakers (PM). This study correlates updated non-invasive studies, including newer strain values, with clinical and invasive hemodynamic assessments of CRT response in the young.

Nonutility of Diagnostic Testing in Ambulatory Patients With Supraventricular Tachycardia.

Background: Supraventricular tachycardia (SVT) is the most common arrhythmia in the pediatric population. Patients often present to the emergency department and undergo extensive diagnostic assessment. The objective of this study is to determine the frequency of diagnostic studies obtained in such patients and to assess clinical significance of these results.

Anthracycline Treatment and Left Atrial Function in Children: A Real-Time 3-Dimensional Echocardiographic Study.

Anthracycline (AC) therapy is associated with left ventricular (LV) dysfunction. Left atrial (LA) size and function are used to assess LV diastolic function in heart failure in adults. Data on LA size and function following AC therapy in children is limited.

Cardiac Amyloidosis in a Child Presenting with Syncope: The First Reported Case and a Diagnostic Dilemma.

Cardiac amyloidosis is a rare cause of cardiomyopathy, reported exclusively in adults. We report the first known case presenting in childhood. A 12-year-old boy presented with syncope and diagnosed with ventricular non-compaction by echocardiography.

Do current scientific reports of hydronephrosis make the grade?

Background: In December 2014, Nguyen et al. introduced the Upper Tract Dilation (UTD) classification scheme, hoping to unify multiple disciplines when describing ultrasound imaging of congenital hydronephrosis. We hypothesized that the academic community has been slow to adopt its use in publications.

Infantile-onset pompe disease: a tale of two cases.

Pompe disease is a type-II glycogen storage disease, and clinical manifestations include hypertrophic cardiomyopathy and generalised muscular hypotonia. Enzyme replacement therapy has proven to be effective in reversing the ventricular hypertrophy. The outcomes are variable depending on time to diagnosis and severity of the cardiac disease.

Comparative Cardiorespiratory Fitness in Children: Racial Disparity May Begin Early in Childhood.

African American (AA) adults are reported to have lower levels of cardiorespiratory fitness (CRF) as compared to Caucasian adults. CRF is linked to cardiovascular morbidity and mortality. We hypothesized that the disparities start early in childhood.

Closure of insufficient, native right ventricular outflow tract with AMPLATZER™ muscular ventricular septal defect occluder in a patient with tetralogy of Fallot post-Melody valve.

AMPLATZER™ muscular ventricular septal defect occluder is used commonly for off-label purposes. We describe an unusual case of a patient with tetralogy of Fallot who underwent repair with a right ventricle to pulmonary artery homograft due to abnormal coronary artery pattern. During the initial surgery, the native right ventricular outflow tract was left open.

Utility of Neuroimaging in Children Presenting to a Pediatric Emergency Department With Ataxia.

Objectives: The aim of this study was to evaluate the utility of neuroimaging in children who present to the pediatric emergency department with acute-/subacute-onset ataxia. Neuroimaging is performed in many children with ataxia to rule out serious intracranial pathology. There is, however, limited evidence to support such practice.

Characteristics and outcomes of patients with formiminoglutamic aciduria detected through newborn screening.

Background: Glutamate formiminotransferase deficiency (FTCD deficiency) or formiminoglutamic aciduria is the second most common of the known inherited disorders of folate metabolism. Initial case reports suggested that patients may have severe intellectual disability and megaloblastic anemia. However, these cases were obtained from screening cohorts of patients with developmental delay.

16 Years of Cardiac Resynchronization Pacing Among Congenital Heart Disease Patients: Direct Contractility (dP/dt-max) Screening When the Guidelines Do Not Apply.

Objectives: The purpose of this study was to use direct cardiac resynchronization therapy (CRT)-paced contractility (dP/dt-max) response as a pre-implantation evaluation among patients with congenital heart disease (CHD) and follow clinical parameters and contractility indexes after CRT implantation.

Background: Patients with CHD often develop early heart failure with few therapeutic options, leading to heart transplantation (HT). Unfortunately, guidelines for CRT do not apply, and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies.

Outcome of pediatric heart transplantation in blood culture positive donors in the United States.

Active donor infection at the time of organ procurement poses a potential infection risk and may increase post-transplant morbidity and mortality in recipients. Our hypothesis was that pediatric heart transplant recipients from blood culture positive donors (BCPD) would have increased morbidity and mortality compared to non-blood culture positive donors (NBCPD). A retrospective analysis of pediatric heart transplant recipients using the organ procurement and transplant network (OPTN) between 1987 and 2015 was conducted.

NT-pro BNP-A marker for worsening respiratory status and mortality in infants and young children with pulmonary hypertension.

Aim: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed.

Methods: A retrospective review of all infants and children < 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman's rank correlation coefficients.

Prospective parents' perspectives on antenatal decision making for the anticipated birth of a periviable infant.

Objective: To examine prospective parents' perceptions of management options and outcomes in the context of threatened periviable delivery, and the values they apply in making antenatal decisions during this period.

Study Design: Qualitative analysis of 46 antenatal interviews conducted at three tertiary-care hospitals with 54 prospective parents (40 pregnant women, 14 partners) who had received counseling for threatened periviable delivery (40 cases).

Results: Participants most often recalled being involved in resuscitation, cerclage, and delivery mode decisions.

Short-Term Propofol Infusion and Associated Effects on Serum Lactate in Pediatric Patients.

Objectives: This study aimed to determine if short-duration procedural sedation in children with propofol is related to an adverse metabolic stress response measured by serum lactate. Propofol infusion syndrome is associated with high-dose and long-duration infusion. It has not been studied with short-duration, outpatient propofol administration.

Testing Placement of Gastric Feeding Tubes in Infants.

Background: Inadvertent positioning of a nasogastric tube in the lung can cause serious complications, so identifying methods to detect improperly inserted tubes is imperative.

Objectives: To compare the sensitivity, specificity, and negative and positive predictive values of 4 pH cut points (< 4.0, < 4.