Real-world use of intravenous iron sucrose in children with cardiac disease.

Iron deficiency has been associated with heart failure severity and mortality in children and adults. Intravenous iron therapy has been associated with improved outcomes for adults with heart failure. However, little is known about its impact and safety in children.

Body Mass Index Trajectory in Children With Kawasaki Disease.

Acquired cardiovascular risk is a concern for children with Kawasaki disease, which might influence obesity. Patients with Kawasaki disease in a single-instruction, retrospective cohort exhibited only a minimal increase in BMI z score in the first 6 weeks after diagnosis that resolved at 1 year. Neither corticosteroid treatment nor the presence of coronary artery aneurysms had adverse impacts on the BMI z score trajectory.

Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein Stenosis Using a Comprehensive Standardized Catheterization Assessment: A Prospective Study.

Background: Pediatric intraluminal pulmonary vein stenosis (PVS) occurs in a heterogeneous patient population but is unified by its cellular composition in affected pulmonary veins. Enhanced delineation of the hemodynamic and physiological vein consequences of PVS and its impact on vein lumen, vein pressure, wall structure, vein compliance, and wall shear stress has not been studied. The objective is to assess PVS characteristics using multimodal catheterization techniques (angiography, pulmonary vein pressure, intravascular ultrasound, balloon compliance testing, and wall shear stress calculation) and determine their association with vein outcomes.

Paediatric extracorporeal membrane oxygenation use by social determinants: a multicentre retrospective cohort study.

Background: Social determinants of health have upstream effects on health-care access and decision making to influence outcomes. We aimed to study the use of extracorporeal membrane oxygenation (ECMO) in children according to social determinants of health.

Methods: This retrospective, multicentre cohort study used data from 47 children's hospitals in the USA that contributed to the Pediatric Health Information System.

Risk of Recurrence and Long-Term Outcomes of Ectopic Atrial Tachycardia After Surgery for Congenital Heart Disease.

Introduction: Ectopic atrial tachycardia (EAT) is a postoperative complication of congenital heart surgery, however its postoperative course and risk of recurrence after hospital discharge is not well described. This study aims to describe the characteristics and outcomes of patients with postoperative EAT and the risk of recurrence during follow-up.

Methods: Retrospective cohort study including patients who underwent surgery for congenital heart disease (2010-2022) with EAT within 45 days of surgery.

Mid-term ventricular function in patients with tetralogy of Fallot after transcatheter pulmonary valve replacement: Relationship to baseline right ventricular loading conditions.

Background: We investigated the mid-term systolic ventricular response to transcatheter pulmonary valve replacement (TPVR) in patients with repaired tetralogy of Fallot (TOF) and pulmonary stenosis (PS), pulmonary regurgitation (PR) and a MIXED subgroup that included patients with both PR and PS.

Methods: We included patients with repaired TOF with PS, atresia and absent pulmonary valve underwent TPVR (2007-2011) and followed at BCH until 2021. We compared their serial clinical, echo imaging as well as cardiopulmonary exercise test data among PS, PR and MIXED subgroups.

Donor History of Drug Use and Graft Survival in Pediatric Heart Transplant Recipients.

Importance: Older children awaiting a heart transplant (HT) sometimes receive a heart offer from a donor with a history of drug use (HDU). The effect of using such donor hearts on posttransplant survival in pediatric recipients is unclear.

Objective: To assess the association of using hearts from donors with HDU on posttransplant graft survival in pediatric HT recipients.

Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula.

Infants with hypoplastic left heart syndrome (HLHS) with mitral stenosis/aortic atresia (MS/AA) have worse outcomes compared to other anatomic variants; this may be related to left ventricle-coronary artery (LV-CA) fistula. We reviewed patients with HLHS (MS/AA) referred to Boston Children's Hospital and managed from birth during 2008 to 2023 and compared those with and without LV-CA fistula defined angiographically. Among 90 patients, 58 (64%) had LV-CA fistula.

Congenital Cardiac Catheterization Risk Assessment in Infants Under 2.5 kg.

Premature and small-for-gestational-age neonates with congenital heart disease increasingly require congenital cardiac catheterization (CCC). These patients present unique procedural and patient-specific risks that standard risk models do not fully capture. This study aims to assess risk in infants < 2.

Comparative Congenital Cardiac Catheterization Registry Analysis From the United States and Low- and Middle-Income Countries.

Background: Disparities in congenital heart disease care exist between high-income and low- and middle-income countries (LMICs), likely extending to congenital cardiac catheterization (CCC).

Objectives: This study compares patient characteristics and outcomes of CCC in the U.S.

Ventricular-arterial coupling is associated with clinical outcomes in patients with systemic right ventricle.

Background: Adults with transposition of the great arteries (TGA) and systemic right ventricle (SRV) are at risk for heart failure (HF) and decreased survival. Ventricular-arterial coupling (VAC) quantifies the interaction between myocardial contractile function and the load imposed by the arterial circulation and may be valuable in identifying subclinical SRV dysfunction. The purpose of our study is to determine the association of VAC with adverse clinical outcomes in adults with TGA and SRV.

Association between procedure duration and adverse events in congenital cardiac catheterization.

Background: Procedure duration is an important predictor of patient outcomes in surgery. However, the relationship between procedure duration and adverse events in congenital cardiac catheterization is largely unexplored.

Methods: All cases entered into the Congenital Cardiac Catheterization Project on Outcomes from 2014 to 2017 were included.

Associations With Adverse Postoperative Outcomes in Children Undergoing Atrial Septal Defect Closure in Lower-Resource Settings.

Background: Atrial septal defects (ASDs) are a common cause of congenital heart disease worldwide.

Objectives: The purpose of the study was to assess change over time in surgical outcomes for ASD repair and identify patient-level risk factors for adverse postoperative outcomes.

Methods: We analyzed cases of isolated ASD in patients <18 years from 2010 to 2020 from 71 sites participating in the International Quality Improvement Collaborative for Congenital Heart Disease.

Formulation of Quality Improvement Methodology for Risk Mitigation in Congenital Cardiac Catheterization.

The C3PO collaborative, with a history of successful quality improvement (QI) initiatives, leveraged registry participants to develop a multi-center QI initiative to reduce adverse events (AEs) in congenital cardiac catheterization. A 32-person, interdisciplinary working group analyzed audited data for all congenital cardiac catheterization cases from 2014-2017. The primary outcome was the occurrence of any high-severity (level 3/4/5) AE.

Electrocardiographic Changes in Pregnant Patients With Congenital Heart Disease.

Background: Electrocardiograms (EKGs) are routinely performed in pregnant patients with pre-existing cardiovascular disease. However, in pregnant patients with congenital heart disease (CHD), EKG changes during gestation have not been explored.

Methods: We performed a retrospective study of pregnant patients with CHD enrolled in the STORCC initiative.

Propensity Score Analysis of Possible Medication Effects on Outcomes in Patients With Systemic Right Ventricles.

Background: Patients with systemic right ventricle (SRV), either d-transposition of the great arteries following an atrial switch procedure or congenitally corrected transposition of the great arteries, develop severe right ventricular dysfunction, prompting appropriate medical therapy. However, the efficacy of beta-blockers and angiotensin receptor blockers or angiotensin-converting enzyme inhibitors (ACEI) in SRV patients is unproven.

Objectives: The objective of this study was to determine the effects of ACEI/ARB and beta-blockers on outcomes in SRV patients after accounting for likely cofounders affecting their use.

Exercise testing in clinical context: Reference ranges for interpreting anaerobic threshold as an outcome for congenital heart disease patients.

Background: Change in the oxygen consumption (VO) at the ventilatory anaerobic threshold (VAT) is an important outcome in research studies of children with congenital heart disease (CHD). The range of values reported by different raters for any given VAT is needed to contextualize a change in VAT in intervention studies.

Methods: Sixty maximal cardiopulmonary exercise tests (CPET) for CHD patients 8-21 years old were independently reviewed by six exercise physiologists and four pediatric cardiologists.

Cardiac Comorbidity and Exercise Intolerance in Bilateral Lung Transplant Recipients Followed at a Pediatric Center.

Reduced exercise capacity is common in young bilateral lung transplantation (Bi-LTx) recipients, but longer-term data on cardiac comorbidities are limited. We evaluate potential cardiac contributions to long-term exercise intolerance in this population. All Bi-LTx recipients at a single pediatric center, who completed routine clinical post-transplant cardiac assessment, including echocardiogram, cardiac exam, and cardiopulmonary exercise testing (CPET), were included.

Outcomes in Adult Congenital Heart Disease Patients With Down Syndrome Undergoing a Cardiac Surgical Procedure.

Background: As the life expectancy of patients with Down syndrome (DS) improves, the number of older patients with DS who require a cardiac surgical procedure for congenital heart disease will increase. Perioperative risk factors and outcomes in these patients are unknown.

Methods: In a multicenter retrospective study, teenaged and adult patients with DS who underwent a cardiac surgical procedure between 2008 and 2018 were matched by age and surgical procedure with patients who did not have DS.

Socioeconomic Status and Major Adverse Transplant Events in Pediatric Heart Transplant Recipients.

Importance: Low socioeconomic status (SES) has been associated with higher risk of rejection and graft loss in pediatric heart transplant (HT) recipients. The association of SES with other posttransplant morbidities is unknown.

Objective: To assess whether low SES is associated with higher risk of a major adverse transplant event (MATE) among pediatric HT recipients.

Postoperative aortic isthmus size after arch reconstruction with patch augmentation predicts arch reintervention.

Background: Rates of reintervention (RI) after patch-augmented reconstruction for hypoplastic aortic arch (HAA) remain moderately high. We analyzed mid-term outcomes of aortic arch reconstruction to define modifiable reintervention risk factors.

Methods: Excluding Damus-Kaye-Stansel anastomoses and previous arch repair, 338 patients underwent arch reconstruction between 2000 and 2021 at median age of 6 days (interquartile range [IQR], 4-13 days) and a median weight of 3.

Real-World Use and Outcomes of Apixaban for Early Post-Surgical Fontan Thromboprophylaxis.

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.

Impact of permanent pacemaker for iatrogenic atrioventricular block on outcomes after congenital heart surgery.

Objective: The need for permanent pacemaker (PPM) for iatrogenic atrioventricular block (AVB) after congenital heart surgery is approximately 1%. We aimed to evaluate the long-term outcomes of patients with PPM for iatrogenic AVB and compare them with patients with an optimal repair (trivial/no residual) Residual Lesion Score (RLS) Class 1 repair without PPM need.

Methods: We reviewed 183 patients discharged with PPM for iatrogenic AVB from 2011 to 2022.

Preoperative Echocardiographically Derived Mean dP/dTic Predicts Early Post-operative Dysfunction in Children Undergoing Mitral Valve Surgery.

Mean dP/dt is a quantitative measurement of ventricular function that can be obtained noninvasively by echocardiography. In adults with mitral regurgitation (MR), it has been shown to be a more sensitive predictor of postoperative left ventricular ejection fraction (EF). The utility of dP/dt in pediatric congenital heart diseases with MR has been underexplored.