Utilizing Fetal Echocardiography to Risk Stratify and Predict Neonatal Outcomes in Fetuses Diagnosed with Congenital Heart Disease.

Objective:  Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room.

Study Design:  This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk).

The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair.

To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed.

Utility of apical four-chamber longitudinal strain in the assessment of childhood cancer survivors: A multicenter study.

Background: A previous multicenter study showed that longitudinal changes in standard cardiac functional parameters were associated with the development of cardiomyopathy in childhood cancer survivors (CCS). Evaluation of the relationship between global longitudinal strain (GLS) changes and cardiomyopathy risk was limited, largely due to lack of quality apical 2- and 3-chamber views in addition to 4-chamber view. We sought to determine whether apical 4-chamber longitudinal strain (A4LS) alone can serve as a suitable surrogate for GLS in this population.

Anatomical and physiological diagnostic discrepancies in fetuses with single-ventricle congenital heart disease in a contemporary cohort.

Objective: Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE, specifically in single ventricle (SV) congenital heart disease (CHD). This study aimed to assess the ability of FE to correctly predict SV-CHD postnatal anatomy and physiology in a contemporary cohort.

Methods: The contemporary clinical reports of patients with SV-CHD, in which FE was performed between July 2017 and July 2021, were compared with postnatal echocardiograms from a formal quality assurance program.

Rationale and design of the Children's Oncology Group study AAML1831 integrated cardiac substudies in pediatric acute myeloid leukemia therapy.

Background: Pediatric acute myeloid leukemia (AML) therapy is associated with substantial short- and long-term treatment-related cardiotoxicity mainly due to high-dose anthracycline exposure. Early left ventricular systolic dysfunction (LVSD) compromises anthracycline delivery and is associated with inferior event-free and overall survival in pediatric AML. Thus, effective cardioprotective strategies and cardiotoxicity risk predictors are critical to optimize cancer therapy delivery and enable early interventions to prevent progressive LVSD.

Contemporary transesophageal echocardiography practice patterns among paediatric cardiology centres in the United States and Canada.

Objective: To characterise transesophageal echocardiography practice patterns among paediatric cardiac surgical centres in the United States and Canada.

Methods: A 42-question survey was sent to 80 echocardiography laboratory directors at paediatric cardiology centres with surgical programmes in the United States and Canada. Question domains included transesophageal echocardiography centre characteristics, performance and reporting, equipment use, trainee participation, and quality assurance.

A Multicenter Analysis of Abnormal Chromosomal Microarray Findings in Congenital Heart Disease.

Background Chromosomal microarray analysis (CMA) provides an opportunity to understand genetic causes of congenital heart disease (CHD). The methods for describing cardiac phenotypes in patients with CMA abnormalities have been inconsistent, which may complicate clinical interpretation of abnormal testing results and hinder a more complete understanding of genotype-phenotype relationships. Methods and Results Patients with CHD and abnormal clinical CMA were accrued from 9 pediatric cardiac centers.

Contemporary Outcomes in Fetuses Diagnosed with Vascular Rings.

Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017 to 2022.

A multicenter cross-sectional study in infants with congenital heart defects demonstrates high diagnostic yield of genetic testing but variable evaluation practices.

Purpose: For patients with congenital heart disease (CHD), the most common birth defect, genetic evaluation is not universally accepted, and current practices are anecdotal. Here, we analyzed genetic evaluation practices across centers, determined diagnostic yield of testing, and identified phenotypic features associated with abnormal results.

Methods: This is a multicenter cross-sectional study of 5 large children's hospitals, including 2899 children ≤14 months undergoing surgical repair for CHD from 2013 to 2016, followed by multivariate logistics regression analysis.

Impact of the COVID pandemic on quality measures in a pediatric echocardiography lab.

Background: The COVID pandemic necessitated an altered approach to transthoracic echocardiography, especially in COVID cases. Whether this has effected echocardiography lab quality is unknown.

Objectives: We sought to determine whether echocardiography lab quality measures during the COVID pandemic were different from those prior to the pandemic and whether quality and comprehensiveness of echocardiograms performed during the pandemic was different between COVID and non-COVID patients.

Evaluation of Left Ventricular Outflow Gradients During Staged Exercise Stress Echocardiography Helps Differentiate Pediatric Patients With Hypertrophic Cardiomyopathy From Athletes and Normal Subjects.

Background: Elevated left ventricular outflow tract (LVOT) gradients during exercise can occur in patients with hypertrophic cardiomyopathy (HCM) as well as in athletes and normal controls. The authors' staged exercise protocol calls for imaging at rest and during each stage of exercise to evaluate the mechanism of LVOT obstruction at each stage. They investigated whether this staged approach helps differentiate HCM from athletes and normal controls.

Fetal Diagnosis in a Unique Case of Vascular and Cardiac Interdependence in Omphaloischiopagus Conjoined Twins.

• Cardiac interdependence in conjoined twins is a rare phenomenon. • Presence of congenital heart disease may be an important prognostic factor. • There is high risk of high-output heart failure and pulmonary hypertension in the donor twin.

Challenges associated with retrospective analysis of left ventricular function using clinical echocardiograms from a multicenter research study.

Background: Retrospective multicenter research using echocardiograms obtained for routine clinical care can be hampered by issues of individual center quality. We sought to evaluate imaging and patient characteristics associated with poorer quality of archived echocardiograms from a cohort of childhood cancer survivors.

Methods: A single blinded reviewer at a central core laboratory graded quality of clinical echocardiograms from five centers focusing on images to derive 2D and M-mode fractional shortening (FS), biplane Simpson's ejection fraction (EF), myocardial performance index (MPI), tissue Doppler imaging (TDI)-derived velocities, and global longitudinal strain (GLS).

Cost-Effectiveness of the International Late Effects of Childhood Cancer Guideline Harmonization Group Screening Guidelines to Prevent Heart Failure in Survivors of Childhood Cancer.

Purpose: Survivors of childhood cancer treated with anthracyclines and/or chest-directed radiation are at increased risk for heart failure (HF). The International Late Effects of Childhood Cancer Guideline Harmonization Group (IGHG) recommends risk-based screening echocardiograms, but evidence supporting its frequency and cost-effectiveness is limited.

Patients And Methods: Using the Childhood Cancer Survivor Study and St Jude Lifetime Cohort, we developed a microsimulation model of the clinical course of HF.

Longitudinal Changes in Echocardiographic Parameters of Cardiac Function in Pediatric Cancer Survivors.

Background: Childhood cancer survivors undergo serial echocardiograms to screen for cardiotoxicity. It is not clear whether small longitudinal changes in functional or structural parameters over time have clinical significance.

Objectives: To assess the timing of changes in serial echocardiographic parameters in pediatric age childhood cancer survivors and to evaluate their associations with cardiomyopathy development.

The landscape of cardiovascular care in pediatric cancer patients and survivors: a survey by the ACC Pediatric Cardio-Oncology Work Group.

Objective: To enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors.

Study Design: A 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC.

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect.

Background: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results.

Methods: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation.

Myocardial deformation as a predictor of right ventricular pacing-induced cardiomyopathy in the pediatric population.

Introduction: Right ventricular pacing is associated with pacemaker induced cardiomyopathy and lesser degrees of pacing-induced LV dysfunction (PIVD) manifested by a reduction in left ventricular ejection fraction (LVEF). Our objective was to determine whether apical 4 chamber strain (A4C) by echocardiography can identify patients at risk of PIVD before LVEF declines.

Methods And Results: A retrospective chart review of patients (0-21 years) who had a pacemaker with a ventricular lead placed between 2011 and 2017 was performed.