Coronary Events Following Dobutamine Stress Cardiac Magnetic Resonance Imaging in Pediatric Patients.

Dobutamine stress cardiac magnetic resonance (dsCMR) has demonstrated value in identifying patients at risk for adverse cardiovascular events in adults with coronary artery disease, but its prognostic value in younger patients is unknown. This study sought to evaluate the relationship between dsCMR results and the risk of subsequent cardiovascular events in children. Patients age < 23 years who underwent dsCMR at Boston Children's Hospital were eligible for inclusion.

Deep Learning-Based Electrocardiogram Analysis Predicts Biventricular Dysfunction and Dilation in Congenital Heart Disease.

Background: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to detect biventricular pathophysiology. However, AI-ECG analysis remains underexplored in congenital heart disease (CHD).

Objectives: The purpose of this study was to develop and externally validate an AI-ECG model to predict cardiovascular magnetic resonance (CMR)-defined biventricular dysfunction/dilation in patients with CHD.

Perinatal Factors Associated with Successful Pharmacologic Closure of the Patent Ductus Arteriosus in Premature Infants.

The patent ductus arteriosus (PDA) is associated with significant morbidity and mortality in preterm infants. While pharmacologic closure of the PDA is common and effective, it can be difficult to identify which patients will respond. As such, the objective of this study was to identify factors associated with successful pharmacologic closure of the PDA.

HVSMR-2.0: A 3D cardiovascular MR dataset for whole-heart segmentation in congenital heart disease.

Patients with congenital heart disease often have cardiac anatomy that deviates significantly from normal, frequently requiring multiple heart surgeries. Image segmentation from a preoperative cardiovascular magnetic resonance (CMR) scan would enable creation of patient-specific 3D surface models of the heart, which have potential to improve surgical planning, enable surgical simulation, and allow automatic computation of quantitative metrics of heart function. However, there is no publicly available CMR dataset for whole-heart segmentation in patients with congenital heart disease.

mRNA Display in Cell Lysates Enables Identification of Cyclic Peptides Targeting the BRD3 Extraterminal Domain.

mRNA display is a powerful technology to screen libraries of >10 cyclic peptides against a protein target, enabling the rapid discovery of high affinity ligands. These cyclic peptides are particularly well suited to challenging protein targets that have been difficult to drug with small molecules. However, target choice can still be limited as screens are typically performed against purified proteins which often demands the use of isolated domains and precludes the use of aggregation-prone targets.

The FAM13A Long Isoform Regulates Cilia Movement and Coordination in Airway Mucociliary Transport.

Single nucelotide polymorphisms (SNPs) at the locus are among the most commonly reported risk alleles associated with chronic obstructive pulmonary disease (COPD) and other respiratory diseases; however, the physiological role of FAM13A is unclear. In humans, two major protein isoforms are expressed at the locus: "long" and "short," but their functions remain unknown, partly because of a lack of isoform conservation in mice. We performed in-depth characterization of organotypic primary human airway epithelial cell subsets and show that multiciliated cells predominantly express the FAM13A long isoform containing a putative N-terminal Rho GTPase-activating protein (RhoGAP) domain.

The impact of audiovisual breathing guidance on respiratory-triggered cardiac magnetic resonance cine imaging.

Background: In patients who have difficulty holding their breath, a free breathing (FB) respiratory-triggered (RT) bSSFP cine technique may be used. However, this technique may have inferior image quality and a longer scan time than breath-hold (BH) bSSFP cine acquisitions. This study examined the effect of an audiovisual breathing guidance (BG) system on RT bSSFP cine image quality, scan time, and ventricular measurements.

Evolving Role of Three-Dimensional Echocardiography for Right Ventricular Volume Analysis in Pediatric Heart Disease: Literature Review and Clinical Applications.

Accurate knowledge of right ventricular (RV) volumes and ejection fraction is fundamental to providing optimal care for pediatric patients with congenital and acquired heart disease, as well as pulmonary hypertension. Traditionally, these volumes have been measured using cardiac magnetic resonance because of its accuracy, reproducibility, and freedom from geometric assumptions. More recently, an increasing number of studies have described the measurement of RV volumes using three-dimensional (3D) echocardiography.

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.

Aortic arch shape after arch repair predicts exercise capacity: a multicentre analysis.

Aims: Coarctation of the aorta is associated with long-term morbidity including decreased exercise capacity, despite successful repair. In the absence of discrete recoarctation, the haemodynamic mechanism remains unknown. This multicentre study evaluated the relationship between aorta shape, flow, and exercise capacity in patients after arch repair, specifically through the lens of aortic size mismatch and descending aortic (DAo) flow and their association with exercise.

Automated segmentation of 3D cine cardiovascular magnetic resonance imaging.

Introduction: As the life expectancy of children with congenital heart disease (CHD) is rapidly increasing and the adult population with CHD is growing, there is an unmet need to improve clinical workflow and efficiency of analysis. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging modality for monitoring patients with CHD. CMR exam is based on multiple breath-hold 2-dimensional (2D) cine acquisitions that should be precisely prescribed and is expert and institution dependent.

Cardiac magnetic resonance parameters associated with successful conversion from a single ventricular to a one-and-a-half or biventricular circulation in patients with a hypoplastic right ventricle.

Background: Some patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.

Right ventricular contraction patterns in healthy children using three-dimensional echocardiography.

Background: The right ventricle (RV) has complex geometry and function, with motion along three separate axes-longitudinal, radial, and anteroposterior. Quantitative assessment of RV function by two-dimension echocardiography (2DE) has been limited as a consequence of this complexity, whereas newer three dimensional (3D) analysis offers the potential for more comprehensive assessment of the contributors to RV function. The aims of this study were to quantify the longitudinal, radial and anteroposterior components of global RV function using 3D echocardiography in a cohort of healthy children and to examine maturational changes in these parameters.

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.

A comparison between the apical and subcostal view for three-dimensional echocardiographic assessment of right ventricular volumes in pediatric patients.

Background: Accurate measurement of ventricular volumes is an important clinical imaging goal. Three-dimensional echocardiography (3DEcho) is used increasingly as it is more available and less costly than cardiac magnetic resonance (CMR). For the right ventricle (RV), the current practice is to acquire 3DEcho volumes from the apical view.

Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects.

Background: To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications.

Methods: Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects.

Pediatric Normal Values and Z Score Equations for Left and Right Ventricular Strain by Two-Dimensional Speckle-Tracking Echocardiography Derived from a Large Cohort of Healthy Children.

Background: Strain values vary with age in children and are both vendor and platform specific. Philips QLAB 10.8 and TomTec AutoSTRAIN are two widely used strain analysis platforms, and both incorporate recent European Association of Cardiovascular Imaging/American Society of Echocardiography/Industry Task Force to Standardize Deformation Imaging guidelines.

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation.

Background: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known.

Methods: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (mass), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time.

Predicting Hemodynamic Performance of Fontan Operation for Glenn Physiology using Computational Fluid Dynamics: Ten Patient-specific Cases.

Single ventricle hearts have only one ventricle that can pump blood effectively and the treatment requires three stages of operations to reconfigure the heart and circulatory system. At the second stage, Glenn procedure is performed to connect superior vena cava (SVC) to the pulmonary arteries (PA). For the third and most complex operation, called Fontan, an extracardiac conduit is used to connect inferior vena cava (IVC) to the PL and thereafter no deoxygenated blood goes to the heart.

Non-contrast free-breathing whole-heart 3D cine cardiovascular magnetic resonance with a novel 3D radial leaf trajectory.

Purpose: To develop and validate a non-contrast free-breathing whole-heart 3D cine steady-state free precession (SSFP) sequence with a novel 3D radial leaf trajectory.

Methods: We used a respiratory navigator to trigger acquisition of 3D cine data at end-expiration to minimize respiratory motion in our 3D cine SSFP sequence. We developed a novel 3D radial leaf trajectory to reduce gradient jumps and associated eddy-current artifacts.

An Automated View Classification Model for Pediatric Echocardiography Using Artificial Intelligence.

Background: View classification is a key step toward building a fully automated system for interpretation of echocardiograms. However, compared with adult echocardiograms, creating a view classification model for pediatric echocardiograms poses additional challenges, such as greater variation in anatomy, structure size, and views. The aim of this study was to develop a computer vision model to autonomously perform view classification on pediatric echocardiographic images.

Learned iterative segmentation of highly variable anatomy from limited data: Applications to whole heart segmentation for congenital heart disease.

Training deep learning models that segment an image in one step typically requires a large collection of manually annotated images that captures the anatomical variability in a cohort. This poses challenges when anatomical variability is extreme but training data is limited, as when segmenting cardiac structures in patients with congenital heart disease (CHD). In this paper, we propose an iterative segmentation model and show that it can be accurately learned from a small dataset.