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.

Improving Mechanical Circulatory Support Outcomes in Failing Bidirectional Glenn Physiology.

There remains high morbidity and mortality with mechanical circulatory support (MCS) in failing bidirectional Glenn (BDG) physiology. We performed a retrospective analysis of children with BDG physiology supported with MCS before and after 2018. Fourteen patients met inclusion criteria (median age 1.

Epicardial pacing outcomes in infants with heart block: Lead and device complications from a multicenter experience.

Background: Infants with complete heart block (CHB) require epicardial pacemaker (PM) insertion. Prior studies described epicardial pacing outcomes in infants and children, although they were limited by small or heterogeneous populations.

Objective: This study aimed to explore patient- and procedure-level associations with device complications in infants with CHB who received a permanent PM.

Combined Exercise and Mindset Training During a Pediatric Cardiac Fitness and Rehabilitation Program Benefits Youth with a Range of Congenital Heart Disease.

Pediatric cardiac fitness and rehabilitation programs vary widely in structure and content. The Cardiac Fitness Program (CFP) is built on traditional training pillars of aerobic, strength, and flexibility, and adds a fourth, training a positive mindset. This study assesses whether the systematic and comprehensive framework of the CFP results in broad benefits for a range of patients with congenital heart disease (CHD).

Mobile Cardiac Telemetry Use to Predict Adverse Pregnancy Outcomes in Patients With Congenital Heart Disease.

Background: Patients with congenital heart disease (CHD) have a higher incidence of arrhythmias during pregnancy, yet the utility of mobile cardiac telemetry (MCT) to predict adverse outcomes is unknown.

Objectives: The purpose of this study is to determine whether arrhythmias on screening MCT correlate with adverse pregnancy outcomes.

Methods: Patients with CHD prospectively enrolled in the Standardized Outcomes in Reproductive Cardiovascular Care initiative underwent 24-hour MCT (within 18 months prior to pregnancy).

Preoperative Malnutrition Increases Risk of In-Hospital Mortality, Major Infection, and Longer Intensive Care Unit Stay After Ventricular Septal Defect Closure.

Background: High energy requirements and poor feeding can lead to growth failure in patients with ventricular septal defect (VSD), but effects of preoperative malnutrition on surgical outcomes are poorly understood, especially in low-resource settings.

Methods And Results: We analyzed a cohort of children <5 years of age undergoing VSD closure at 60 global centers participating in the International Quality Improvement Collaborative for Congenital Heart Disease, 2015 to 2020. We calculated adjusted odds ratios (ORs) for in-hospital death and major infection and adjusted coefficients for duration of intensive care unit stay for 4 measures of malnutrition: severe wasting (weight-for-height score, <-3), moderate wasting (-3 View Article and Find Full Text PDF

Child Opportunity Index and Pediatric Extracorporeal Membrane Oxygenation Outcomes; the Role of Diagnostic Category.

Objectives: To study the impact of social determinants of health (SDoH) on pediatric extracorporeal membrane oxygenation (ECMO) outcomes.

Design, Setting, And Patients: Retrospective study of children (< 18 yr) supported on ECMO (October 1, 2015 to March 1, 2021) using Pediatric Health Information System (44 U.S.

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.

Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome.

Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018.

Exercise-Induced Pulmonary Hypertension in Long-Term Survivors of Congenital Diaphragmatic Hernia.

Objective: To determine the prevalence of exercise-induced pulmonary hypertension (PH) among long-survivors of congenital diaphragmatic hernia repair.

Study Design: This is a single-center, retrospective cohort study of CDH survivors who underwent exercise stress echocardiography (ESE) at Boston Children's Hospital from January 2006 to June 2020. PH severity was assessed by echocardiogram at baseline and after exercise.

Impact of a positive crossmatch on pediatric heart transplant outcomes.

Background: Pediatric heart transplant (HT) candidates experience high waitlist mortality due to a limited donor pool that is constrained in part by anti-HLA sensitization. We evaluated the impact of CDC and Flow donor-specific crossmatch (XM) results on pediatric HT outcomes.

Methods: All pediatric HTs between 1999 and 2019 in the OPTN database were included.

Social Determinants of Health Including Child Opportunity Index Leading to Gaps in Care for Patients With Significant Congenital Heart Disease.

Background: Gaps in care (GIC) are common for patients with congenital heart disease (CHD) and can lead to worsening clinical status, unplanned hospitalization, and mortality. Understanding of how social determinants of health (SDOH) contribute to GIC in CHD is incomplete. We hypothesize that SDOH, including Child Opportunity Index (COI), are associated with GIC in patients with significant CHD.

Catheterization for Congenital Heart Disease Adjustment for Risk Method II.

Background: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk.

Methods: Data from diagnostic only and interventional cases with defined case types were collected for patients ≤18 years of age and ≥2.

Growth of the right ventricular outflow tract in repaired tetralogy of Fallot: A longitudinal CMR study.

Background: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral.

Prospective randomized controlled trial of the safety and feasibility of a novel mesenchymal precursor cell therapy in hypoplastic left heart syndrome.

Objective: To assess the safety and feasibility of low-dose, novel, allogenic mesenchymal precursor cell (MPC) therapy as an adjunct to left ventricular (LV) recruitment for patients with hypoplastic left heart syndrome (HLHS) and borderline left ventricles. MPC injections into the hypoplastic left ventricle may stimulate neovascularization and beneficial LV remodeling and may improve the likelihood of achieving biventricular (BiV) or 1.5 ventricle (1.

Trends in surgical management of anomalous aortic origin of the coronary artery over 2 decades.

Objective: To evaluate outcomes of patients undergoing surgery for anomalous aortic origin of the coronary artery (AAOCA) at a tertiary care center and determine the influences of a coronary artery program on management strategies and outcomes.

Methods: This retrospective review of consecutive surgical patients who had isolated AAOCA at a tertiary care center between August 1, 1999, and October 31, 2022, compared patient characteristics, interventional timing, and surgical strategies before and after program inception in 2018. Comparisons between time periods and anatomical subgroups were performed using Fisher exact and Wilcoxon rank-sum tests.

Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy.

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) typically presents in infancy; however, there are cases of patients who survive the infant period and present later in life. We aimed to characterize patients with late ALCAPA diagnoses and to assess perioperative and functional outcomes. A retrospective chart review of patients who underwent ALCAPA repair between 1996 and 2020 at Boston Children's Hospital was performed.