Impact of Anticoagulation Therapy after Bioprosthetic and Homograft Pulmonary Valve Replacement.

Background: The effect of anticoagulation on early postoperative outcomes following pulmonary valve replacement (PVR) with bioprosthetic valves and homografts is not yet defined. We hypothesized that short-term anticoagulation would be associated with improved valve durability.

Methods: Patients undergoing PVR or right ventricle-pulmonary artery conduits with a bioprosthetic or homograft valve >15 mm in diameter between 1/2015 and 4/2021 at Boston Children's Hospital were retrospectively compared by anticoagulation status.

Biventricular Repair of Univentricular Heart Lowers Risk of Liver Disease Compared With the Fontan Operation.

Background: The Fontan operation is associated with chronic venous hypertension, liver and renal disease, and several other sequelae. The alterative surgical approach, when feasible, a biventricular conversion (BiV), may diminish some of these long-term risks.

Objectives: The aim of this study was to compare long-term outcomes of patients undergoing BiV with those undergoing a destination Fontan operation.

Wide variation in shape of hypoplastic left ventricles undergoing recruitment and biventricular repair: A statistical shape modeling study.

Background: Patients with hypoplastic left ventricles (LV) who undergo volume-loading procedures (recruitment, biventricular [BIV] repair) are at risk for adverse outcomes, including heart failure and death. We investigated pre-BIV LV shape as a predictor of outcome after BIV repair in patients with hypoplastic LVs.

Methods: Baseline and post-recruitment cardiac magnetic resonance imaging and computed tomography data were analyzed in patients with hypoplastic LV (<50 mL/m).

Electrocardiogram-Based Deep Learning to Predict Mortality in Repaired Tetralogy of Fallot.

Background: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to predict mortality in adults with acquired cardiovascular diseases. However, its application to the growing repaired tetralogy of Fallot (rTOF) population remains unexplored.

Objectives: This study aimed to develop and externally validate an AI-ECG model to predict 5-year mortality in rTOF.

Impact of surgical strategy and postrepair transverse aortic arch size on late hypertension after coarctation repair during infancy.

Background: Late hypertension (HTN) after coarctation of the aorta (CoA) repair contributes to higher morbidity and mortality. An association between transverse aortic arch (TAA) hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA.

Nutrition Provision in Children with Heart Disease on Extracorporeal Membrane Oxygenation (ECMO).

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.

Childhood Opportunity and Acute Interstage Outcomes: A National Pediatric Cardiology Quality Improvement Collaborative Analysis.

Background: The interstage period after discharge from stage 1 palliation carries high morbidity and mortality. The impact of social determinants of health on interstage outcomes is not well characterized. We assessed the relationship between childhood opportunity and acute interstage outcomes.

Risk Factors for Death or Transplant After Stage 2 Palliation for Single Ventricle Heart Disease.

Background: For infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur.

Objectives: This study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday.

Methods: Retrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P.

Technical Success and Serious Adverse Events for Fetal Aortic Valvuloplasty in a Large 20-Year Cohort.

Background: FAV is offered to fetuses with severe aortic valve stenosis and evolving hypoplastic left heart syndrome. An inferential analysis of TS and SAE in a large series has not been reported.

Objectives: The purpose of this study was to determine factors associated with fetal aortic valvuloplasty (FAV) technical success (TS) and serious adverse events (SAEs).

Development and Validation of a Mortality Risk Score for Repaired Tetralogy of Fallot.

Background: Robust risk assessment is crucial for the growing repaired tetralogy of Fallot population at risk of major adverse clinical outcomes; however, current tools are hindered by lack of validation. This study aims to develop and validate a risk prediction model for death in the repaired tetralogy of Fallot population.

Methods And Results: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR (International Multicenter Tetralogy of Fallot Registry) cohort with clinical, arrhythmia, cardiac magnetic resonance, and outcome data were included.

Dysregulation of platelet serotonin, 14-3-3, and GPIX in sudden infant death syndrome.

Sudden infant death syndrome (SIDS) is the leading cause of post-neonatal infant mortality, but the underlying cause(s) are unclear. A subset of SIDS infants has abnormalities in the neurotransmitter, serotonin (5-hydroxytryptamine [5-HT]) and the adaptor molecule, 14-3-3 pathways in regions of the brain involved in gasping, response to hypoxia, and arousal. To evaluate our hypothesis that SIDS is, at least in part, a multi-organ dysregulation of 5-HT, we examined whether blood platelets, which have 5-HT and 14-3-3 signaling pathways similar to brain neurons, are abnormal in SIDS.

A prospective multicenter feasibility study of a miniaturized implantable continuous flow ventricular assist device in smaller children with heart failure.

Background: There is no FDA-approved left ventricular assist device (LVAD) for smaller children permitting routine hospital discharge. Smaller children supported with LVADs typically remain hospitalized for months awaiting heart transplant-a major burden for families and a challenge for hospitals. We describe the initial outcomes of the Jarvik 2015, a miniaturized implantable continuous flow LVAD, in the NHLBI-funded Pumps for Kids, Infants, and Neonates (PumpKIN) study, for bridge-to-heart transplant.

Cleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.

Objectives: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes.

Methods: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021.

Erector spinae plane blocks for opioid-sparing multimodal pain management after pediatric cardiac surgery.

Objective: Peripheral regional anesthesia is proposed to enhance recovery. We sought to evaluate the efficacy of bilateral continuous erector spinae plane blocks (B-ESpB) for postoperative analgesia and the impact on recovery in children undergoing cardiac surgery.

Methods: Patients aged 2 through 17 years undergoing cardiac surgery in the enhanced recovery after cardiac surgery program were prospectively enrolled to receive B-ESpB at the end of the procedure, with continuous infusions via catheters postoperatively.

Mitral valve orifice area predicts outcome after biventricular repair in patients with hypoplastic left ventricles.

Background: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV).

Methods: Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m) and no endocardial fibroelastosis who underwent BiV repair from 2005-2022.

Multiomic Analysis of Neuroinflammation and Occult Infection in Sudden Infant Death Syndrome.

Importance: Antemortem infection is a risk factor for sudden infant death syndrome (SIDS)-the leading postneonatal cause of infant mortality in the developed world. Manifestations of infection and inflammation are not always apparent in clinical settings or by standard autopsy; thus, enhanced resolution approaches are needed.

Objective: To ascertain whether a subset of SIDS cases is associated with neuroinflammation and occult infection.

Stress Ulcer Prophylaxis Versus Placebo-A Blinded Pilot Randomized Controlled Trial to Evaluate the Safety of Two Strategies in Critically Ill Infants With Congenital Heart Disease.

Objectives: The routine use of stress ulcer prophylaxis (SUP) in infants with congenital heart disease (CHD) in the cardiac ICU (CICU) is controversial. We aimed to conduct a pilot study to explore the feasibility of performing a subsequent larger trial to assess the safety and efficacy of withholding SUP in this population (NCT03667703).

Design, Setting, Patients: Single-center, prospective, double-blinded, parallel group (SUP vs.

The experience of fathers of children hospitalised with advanced heart disease.

Background: There are little reported data on the perspectives of fathers caring for children with chronic conditions. Although survival of children with advanced heart disease has improved, long-term morbidity remains high. This study describes the experience and prognostic awareness of fathers of hospitalised children with advanced heart disease.

The family burden of paediatric heart disease during the chronic phase of illness.

Background: CHD is a lifelong condition with a significant burden of disease to patients and families. With increased survival, attention has shifted to longer-term outcomes, with a focus on social determinants of health. Among children with CHD, socioeconomic status is associated with disparities in outcomes.

Design and Harmonization Approach for the Multi-Institutional Neurocognitive Discovery Study (MINDS) of Adult Congenital Heart Disease (ACHD) Neuroimaging Ancillary Study: A Technical Note.

Dramatic advances in the management of congenital heart disease (CHD) have improved survival to adulthood from less than 10% in the 1960s to over 90% in the current era, such that adult CHD (ACHD) patients now outnumber their pediatric counterparts. ACHD patients demonstrate domain-specific neurocognitive deficits associated with reduced quality of life that include deficits in educational attainment and social interaction. Our hypothesis is that ACHD patients exhibit vascular brain injury and structural/physiological brain alterations that are predictive of specific neurocognitive deficits modified by behavioral and environmental enrichment proxies of cognitive reserve (e.

Impact of Right Ventricular Surface Area-to-Volume Ratio on Ventricular Remodeling After Pulmonary Valve Replacement.

Optimal reverse remodeling of the right ventricle (RV), a sentinel goal of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot, is not fully predicted by volume-based pre-PVR parameters. Our objectives were to characterize novel geometric RV parameters in patients receiving PVR and in controls, and to identify associations between these parameters and chamber remodeling post-PVR. Secondary analysis was performed on cardiac magnetic resonance (CMR) data from 60 patients enrolled in a randomized trial of PVR with and without surgical RV remodeling.

Magnetic resonance three-dimensional steady-state free precession imaging of the thoracic duct in patients with Fontan circulation and its relationship to outcomes.

Background: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes.

Normative Echocardiographic Left Ventricular Parameters and Reference Intervals in Infants.

Background: In pediatric echocardiography, reference intervals are required to distinguish normal variation from pathology. Left ventricular (LV) parameters are particularly important predictors of clinical outcome. However, data from healthy newborns are limited, and current reference intervals provide an inadequate approximation of normal reference ranges.

Improved Outcomes After Pulmonary Valve Replacement in Repaired Tetralogy of Fallot.

Background: The impact of pulmonary valve replacement (PVR) on major adverse clinical outcomes in patients with repaired tetralogy of Fallot (rTOF) is unknown.

Objectives: The purpose of this study was to determine whether PVR is associated with improved survival and freedom from sustained ventricular tachycardia (VT) in rTOF.

Methods: A PVR propensity score was created to adjust for baseline differences between PVR and non-PVR patients enrolled in INDICATOR (International Multicenter TOF Registry).