Pediatric Cardiology

Double-outlet right atrium is an extremely rare congenital cardiac defect, in which the right atrium drains into both ventricles. Here in, we present a peculiar case involving an 8-year-old female with double-outlet right atrium characterized by three distinct atrioventricular valves and a membranous ventricular septal defect. This is associated with right atrium to left ventricular flow through the accessory atrioventricular valve while maintaining adequate size of the right ventricle. Additionally, we include a comprehensive review of the literature to underscore the variability in clinical presentation and management strategies...

No abstract text is available yet for this article.

The article "Effect of Bedside Ultrasound‑Guided Versus Fluoroscopy‑Guided Transvenous Cardiac Temporary Pacing in Children with Bradyarrhythmia" offers valuable insights into comparative short-term pacing strategies for pediatric bradyarrhythmias. There are some comments that the study overlooks the impact of genetic factors on bradyarrhythmias and treatment outcomes, lacks detailed information on the influence of medications, and does not address the cost-effectiveness of the pacing techniques...

Patients with Fontan physiology are at heightened risk of thrombosis related to passive venous return leading to increased stasis, as well as acquired thrombophilia from congestive hepatopathy. Variability exists for post-Fontan thromboprophylaxis, with no consensus on best practices. Direct oral anticoagulants offer advantages over conventional anticoagulants including fewer drug-drug interactions, no dietary restrictions, and less frequent monitoring. Herein, we report our single center experience utilizing apixaban thromboprophylaxis in children post-Fontan procedure...

A significant percentage of patients with heterotaxy show disharmony between abdominal, bronchopulmonary and atrial situs. This finding is interesting in light of the variable effects of ciliary and laterality genes on different organs and different cardiac segments. Defects in ciliary and laterality genes that usually result in situs inversus or heterotaxy, may occasionally act separately at ventricular and/or great arteries segment, even in patients with normal viscero-atrial situs solitus. Some patients with situs solitus and transposition of great arteries or congenitally corrected transposition of great arteries have been shown to represent the result of partial segmental effects of laterality genes alterations...

The Fontan procedure is used to palliate complex forms of congenital heart disease. This results in adverse hepatic sequelae now known as Fontan-associated liver disease (FALD). Conventional laboratory measures of liver disease do not correlate well with FALD severity. Cytokeratin-18 (CK-18) is a measure of cell death and is sensitive in detecting other causes of liver disease. Our aim was to assess the use of a novel measure of liver disease, CK-18, in Fontan patients. This is a single-center, prospective, cross-sectional study of Fontan patients aged 8-21 years old...

Ductal stenting (DS) is an interesting palliation for neonates with duct-dependent pulmonary circulation. With the introduction of drug-eluting stents (DES), which have a lower rate of restenosis and longer patency, these stents have replaced bare metal stents (BMS) in the global market. DES release Rapamycin group drugs, which have anti-proliferative and immunosuppressive effects. While the released drug amount is negligible in adult patients, it can lead to high blood levels in neonates, potentially increasing their risk of infections...

No abstract text is available yet for this article.

Fetal electrocardiogram (FECG) contains crucial information about the fetus during pregnancy, making the extraction of FECG signal essential for monitoring fetal health. However, extracting FECG signal from abdominal electrocardiogram (AECG) poses several challenges: (1) FECG signal is often contaminated by noise, and (2) FECG signal is frequently overshadowed by high-amplitude maternal electrocardiogram (MECG). To address these issues and enhance the accuracy of signal extraction, this paper proposes an improved Cycle Generative Adversarial Networks (CycleGAN) with integrated contrastive learning for FECG signal extraction...

Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded...

The volumetric data obtained from the cardiac CT scan of congenital heart disease patients is important for defining patient's status and making decision for proper management. The objective of this study is to evaluate the intra-observer, inter-observer, and interstudy reproducibility of left ventricular (LV) and right ventricular (RV) or functional single-ventricle (FSV) volume. And compared those between manual and using semi-automated segmentation tool. Total of 127 patients (56 female, 71 male; mean age 82...

Outline a quality initiative establishing an institutional service line for neonatal transcatheter device closure of the patent ductus arteriosus (TDC-PDA). A retrospective descriptive observational study surrounds programmatic approach to TDC-PDA in premature neonates with process measure spanning education, implementation, referral, and post-procedural care. Metrics tracked pre- and post-program creation with statistical analyses performed. Neonatal TDC-PDA referrals increased exponentially since program inception (n = 13 in year prior; n = 42 year 1; n = 74 year 2), especially in patients weighing less than 1...

Nutrition provision for children with heart disease supported with extracorporeal membrane oxygenation (ECMO) involves nuanced decision making. We examined nutrition provision while on ECMO in the CICU and the relationship between energy and protein adequacy and end organ function as assessed by pediatric sequential organ failure assessment (pSOFA) scores in children with heart disease supported with ECMO. Children (≤ 21 years-old) with congenital or acquired heart disease who received ECMO in the cardiac intensive care unit were included...

The gut microbiome of infants with congenital heart disease (CHD) undergoing cardiopulmonary bypass surgery (CPB) is at risk of profound alteration. The aim of this study was to examine the gut microbiome pre- and post-bypass surgery to explore potential implications of altered gut biodiversity. A prospective cohort study involving infants with CHD who underwent CPB was performed. Faecal samples were collected from infants alongside the collection of demographic and clinical data in order to examine gut microbiome changes before and after surgery...

This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplastic left heart syndrome and variants were treated using this procedure at our center...

Dobutamine stress cardiac magnetic resonance (dsCMR) has demonstrated value in identifying patients at risk for adverse cardiovascular events in adults with coronary artery disease, but its prognostic value in younger patients is unknown. This study sought to evaluate the relationship between dsCMR results and the risk of subsequent cardiovascular events in children. Patients age < 23 years who underwent dsCMR at Boston Children's Hospital were eligible for inclusion. Patients were excluded if no follow-up data were available after dsCMR or the dsCMR protocol was not completed...

Children with cardiac disease suffer higher rates of in-hospital cardiac arrest. Cardiac arrest outside of the intensive care unit (ICU) is an infrequent event, which is a threat for suboptimal code team performance. Shorter time to the first epinephrine dose during cardiac arrest has been associated with improved survival and neurologic outcomes. Moderate fidelity in situ simulation training was implemented in the pediatric cardiac acute care unit (PCACU) to improve code team performance in August 2015. A small interprofessional team of simulation facilitators was developed that included nurses, physicians, and advanced practice providers...

The patent ductus arteriosus (PDA) is associated with significant morbidity and mortality in preterm infants. While pharmacologic closure of the PDA is common and effective, it can be difficult to identify which patients will respond. As such, the objective of this study was to identify factors associated with successful pharmacologic closure of the PDA. We hypothesized that clinical factors such as gestational age, birth weight, and hypertensive disorders of pregnancy would be associated with successful closure...

Outpatient cardiologists provide longitudinal care for Fontan patients. As these patients age, they face mounting morbidities, necessitating challenging conversations about prognosis and goals of care. We created a novel survey to evaluate cardiologists' attitudes surrounding risk counseling for patients/caregivers. Cardiologists were recruited during concomitant outpatient enrollment of individuals with Fontan operation > age 10. Physician demographic data, expectations of timing in discussing adverse event risk, and perceived barriers were collected...

Studies have suggested that pediatric patients with heart transplants (HT) due to congenital heart disease (CHD) perform differently on cardiopulmonary exercise testing compared to pediatric patients with HT due to cardiomyopathy (CM). However, it is not known if this relationship changes over time. The aim of this study was to examine the differences in cardiopulmonary exercise test (CPET) parameters over time between patients with HT due to CHD versus CM. A large single-institution CPET database was used for this study...