Limited data exist to guide exercise participation in pediatric hypertrophic cardiomyopathy (HCM) patients. Exercise is traditionally restricted in this population, despite research in adults with HCM demonstrating exercise-associated cardiovascular and psychological benefit. This pilot study seeks to assesses the safety and feasibility of a moderate-intensity exercise program in...
Protein-losing enteropathy (PLE) is a serious complication after the Fontan operation with limited treatment options. This phase 2, multi-center, open-label trial evaluated the efficacy and safety of Camostat Mesylate (CM), a serine protease inhibitor, as adjunctive therapy for PLE. Nineteen patients aged 4 years and older with PLE after the Fontan operation were enrolled. CM was...
The Blalock Taussig Thomas Shunt (BTTS) is a palliative procedure for children with congenital heart disease (CHD). This operation may involve significant morbidity and mortality, and its effect on pulmonary arteries has been ambivalent. Ductal stenting is an alternative option. We aimed to evaluate pulmonary artery growth and clinical course following an operative BTTS as...
Twin Reversed Arterial Perfusion (TRAP) sequence is a rare complication in monochorionic pregnancies, often leading to fetal heart failure (FHF) in the pump twin. We present a 17-year-old primigravida with a monochorionic monoamniotic twin pregnancy presented with an acardiac twin, single umbilical artery (SUA), and multicystic placenta appearance. Throughout the pregnancy, the...
The number of adults living with congenital heart disease continues to rise, emphasizing the need to understand the challenges they encounter to enhance disease management. This study aimed to evaluate adults with CHD using the International Classification of Functioning (ICF) framework and explore factors associated with community integration. Holistic assessments were conducted...
Pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD) is a severely progressive condition with complex pathogenesis. The aim of this study was to evaluate the contribution of genetic variants to the development of PAH in patients with APAH-CHD. Fifteen children and twenty-seven adults diagnosed with APAH-CHD were enrolled. Targeted next-generation...
We sought to determine the impact of the COVID-19 on prenatal diagnosis (PND) of clinically significant congenital heart disease (CHD) and the role of socioeconomic status (SES), complexity of diagnosis, and proximity to advance testing. This single-center retrospective study evaluated 2 eras of infants (COVID (born July 1, 2020–July 31, 2023) and pre-COVID (born June 1, 2017...
We share a single-center experience of transcatheter pulmonary valve replacement (PVR) with the Venus-P valve, discussing procedural insights, considerations for valve sizing in relation to valve expansion and geometry, and associated outcomes. Retrospective review of 42 consecutive cases from January 2017 to March 2024. The median age and weight of the cohort were 39.6 (27.3, 58...
Bioelectrical impedance analysis (BIA) is a noninvasive tool that can estimate volume status using fluid compartment ratios. Previous studies have demonstrated that BIA can be used to help manage heart failure using the edema index (EI), which is the ratio of extracellular water (ECW) to total body water (TBW). This study set out to better define the relationship between BIA...
Pulmonary complications are known to occur in patients after Fontan palliation. Cardiac MRI is performed in the follow-up of Fontan patients to assess single ventricular function, hemodynamics and potential collateral flow. To date, pulmonary function tests have been used to detect functional lung impairment, but lung MRI has not been integrated into imaging follow-up. In this...
Non-invasive assessment of myocardial work is a newly described technique to assess myocardial energetics. This has not been previously studied to assess the effects of right ventricular pacing at different sites or at different heart rates in children. We aimed to study the effects of right ventricular apical, septal, and His bundle pacing on myocardial work along with the...
Tetralogy of Fallot often requires transannular patch repair, leading to pulmonary insufficiency. Percutaneous pulmonary valve implantation (PPVI) with self-expandable valves offers a promising alternative, especially for enlarged right ventricular Queryoutflow tracts (RVOT). Five RVOT types identified in patients with Tetralogy of Fallot reflect anatomical variations due to...
Idiopathic premature ventricular contractions (PVCs) can occur in up to 40% of children with structurally normal hearts. The study aims to assess the impact of high-burden idiopathic PVCs on children in comparison with adults in terms of symptoms, distribution of origin, management strategies, and safety considerations. This is a single-center retrospective cohort study that...
Research establishing factors associated with duration of mechanical ventilation after Tetralogy of Fallot repair, is mainly based on population presenting at early infancy. There are fewer reports regarding repair after infancy, during childhood and preadolescence. To compare two groups of late TOF repair based on post-operative invasive mechanical ventilation duration and...
We reviewed the outcomes of truncus arteriosus repair (primary vs. staged repair incorporating bilateral pulmonary artery banding), focusing on survival, reintervention, and functional data. We analyzed 39 patients who underwent a first intervention for truncus arteriosus (staged, n = 19; primary, n = 20) between 1992 and 2022. The median follow-up period was 8.0 (2.2–13.2) years...
Lead strangulation is a dangerous complication of epicardial pacemaker insertion. This complication has been increasingly highlighted lately. Our institution has recently identified four cases over the past five years. This study’s aim was to 1) identify risk factors for strangulation and 2) prospectively screen existing epicardial pacemaker patients for unrecognized...
The study sought to assess the clinical utility of complete blood count-derived composite scores, suggesting their potential as markers of inflammation and disease severity in Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) with Kawasaki-like features. This retrospective study analyzed data from 71 KD and 73 MIS-C patients and 70 healthy controls...
Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to...
Although the long-term outcomes of the surgical grafts are well defined and reported, the data regarding the mid-and long-term results of the balloon-expandable percutaneous valves in the native right ventricular outflow tract (RVOT) is limited. We retrospectively evaluated 42 patients who underwent PPVI (Sapien® XT valve) to native RVOT due to severe pulmonary regurgitation (PR...
The EDUCATE study investigated the acute impact of energy drink (ED) consumption on heart rate variability (HRV) in children and adolescents, with a focus on how these stimulant-rich beverages influence cardiac autonomic function. Given the popularity of EDs among young people, this study assessed the immediate cardiovascular response to ED intake. This randomized, double-blind...
The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged...
Kawasaki disease (KD) is a febrile vasculitis disorder, with coronary artery lesions (CALs) being the most severe complication. Early detection of CALs is challenging due to limitations in echocardiographic equipment (UCG). This study aimed to develop and validate an artificial intelligence algorithm to distinguish CALs in KD patients and support diagnostic decision-making at...