Inotropes for the prevention of low cardiac output syndrome and mortality for paediatric patients undergoing surgery for congenital heart disease: a network meta-analysis.

Background: Paediatric patients undergoing surgery for congenital heart disease (CHD) are at risk for postoperative low cardiac output syndrome (LCOS) and mortality. LCOS affects up to 25% of children after heart surgery. It consists of reduced myocardial function and increases postoperative morbidity, prolongs mechanical ventilation, and lengthens the duration of intensive care unit (ICU) stay.

Accelerated cardiac magnetic resonance imaging using deep learning for volumetric assessment in children.

Background: Ventricular volumetry using a short-axis stack of two-dimensional (D) cine balanced steady-state free precession (bSSFP) sequences is crucial in any cardiac magnetic resonance imaging (MRI) examination. This task becomes particularly challenging in children due to multiple breath-holds.

Objective: To assess the diagnostic performance of accelerated 3-RR cine MRI sequences using deep learning reconstruction compared with standard 2-D cine bSSFP sequences.

Clinical impact of novel cardiovascular magnetic resonance technology on patients with congenital heart disease: a scientific statement of the Association for European Pediatric and Congenital Cardiology and the European Association of Cardiovascular Imaging of the European Society of Cardiology.

Cardiovascular magnetic resonance (CMR) imaging is recommended in patients with congenital heart disease (CHD) in clinical practice guidelines as the imaging standard for a large variety of diseases. As CMR is evolving, novel techniques are becoming available. Some of them are already used clinically, whereas others still need further evaluation.

Tamoxifen may contribute to preserve cardiac function in Duchenne muscular dystrophy.

Unlabelled: Duchenne muscular dystrophy is life-limiting. Cardiomyopathy, which mostly ensues in the second decade of life, is the main cause of death. Treatment options are still limited.

Possible effects of left pulmonary artery stenting in single ventricle patients on bronchial area, lung volume and lung function.

Background: Left pulmonary artery (LPA) stenting is often required in single ventricle (SV) patients. Due to their close anatomical relationship an LPA stent could potentially compress the left main bronchus (LMB). We assessed the impact of LPA stenting on bronchial size, pulmonary volumes, and lung function in a cohort of SV patients.

Flow evaluation software for four-dimensional flow MRI: a reliability and validation study.

Purpose: Four-dimensional time-resolved phase-contrast cardiovascular magnetic resonance imaging (4D flow MRI) enables blood flow quantification in multiple vessels, which is crucial for patients with congenital heart disease (CHD). We investigated net flow volumes in the ascending aorta and pulmonary arteries by four different postprocessing software packages for 4D flow MRI in comparison with 2D cine phase-contrast measurements (2D PC).

Material And Methods: 4D flow and 2D PC datasets of 47 patients with biventricular CHD (median age 16, range 0.

Signal Thresholding Segmentation of Ventricular Volumes in Young Patients with Various Diseases-Can We Trust the Numbers?

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings.

Feasibility of Non-Gated Dynamic Fetal Cardiac MRI for Identification of Fetal Cardiovascular Anatomy.

Introduction: The aim of this study was to evaluate the feasibility of identifying the fetal cardiac and thoracic vascular structures with non-gated dynamic balanced steady-state free precession (SSFP) MRI sequences.

Methods: We retrospectively assessed the visibility of cardiovascular anatomy in 60 fetuses without suspicion of congenital heart defect. Non-gated dynamic balanced SSFP sequences were acquired in three anatomic planes of the fetal thorax.

Safety, Pharmacokinetics, and Pharmacodynamics of CC-90001 (BMS-986360), a c-Jun N-terminal Kinase Inhibitor, in Phase 1 Studies in Healthy Participants.

CC-90001 selectively inhibits c-Jun N-terminal kinase (JNK), a stress-activated protein implicated in fibrosis. In 3 phase 1 trials evaluating CC-90001 pharmacokinetics, pharmacodynamics, and safety, healthy adults (N = 184) received oral CC-90001 in a single dose (10-720 mg) or multiple doses (30-480 mg once daily for 7-18 days) or placebo. CC-90001 was rapidly absorbed (median time to maximum concentration, 1-4 hours) and eliminated with a mean terminal elimination half-life of 12-28 hours.

Quantitative evaluation of aortic valve regurgitation in 4D flow cardiac magnetic resonance: at which level should we measure?

Purpose: To find the best level to measure aortic flow for quantification of aortic regurgitation (AR) in 4D flow CMR.

Methods: In 27 congenital heart disease patients with AR (67% male, 31 ± 16 years) two blinded observers measured antegrade, retrograde, net aortic flow volumes and regurgitant fractions at 6 levels in 4D flow: (1) below the aortic valve (AV), (2) at the AV, (3) at the aortic sinus, (4) at the sinotubular junction, (5) at the level of the pulmonary arteries (PA) and (6) below the brachiocephalic trunk. 2D phase contrast (2DPC) sequences were acquired at the level of PA.

Physical Activity in Fontan Patients Relates to Quality of Life and Sleep Quality.

Background And Aim: Fontan patients tend to have reduced physical exercise capacity. This study investigates physical activity (PA) and its relationship to exercise capacity, heart rates, cardiac function, biomarkers, health-related quality of life (HRQoL), and sleep quality.

Methods: Cardiovascular magnetic resonance (CMR), exercise testing (CPET), 24 h-ECG, and blood samples were prospectively performed in 38 patients, age 13 (11-16) years.

Myocardial Deformation in Fontan Patients Assessed by Cardiac Magnetic Resonance Feature Tracking: Correlation with Function, Clinical Course, and Biomarkers.

Cardiac MR (CMR) is a standard modality for assessing ventricular function of single ventricles. CMR feature-tracking (CMR-FT) is a novel application enabling strain measurement on cine MR images and is used in patients with congenital heart diseases. We sought to assess the feasibility of CMR-FT in Fontan patients and analyze the correlation between CMR-FT strain values and conventional CMR volumetric parameters, clinical findings, and biomarkers.

Left Ventricular Torsion Obtained Using Equilibrated Warping in Patients with Repaired Tetralogy of Fallot.

Patients after surgical repair of Tetralogy of Fallot (rTOF) may suffer a decrease in left ventricular (LV) function. The aim of our study is to evaluate a novel method of assessing LV torsion in patients with rTOF, as an early indicator of systolic LV dysfunction. Motion tracking based on image registration regularized by the equilibrium gap principle, known as equilibrated warping, was employed to assess LV torsion.

Advanced Imaging and New Cardiac Biomarkers in Long-term Follow-up After Childhood Cancer.

Objectives: Pathologic ejection fraction (EF), shortening fraction (FS), and standard heart failure biomarkers (high sensitive troponin T and N-terminal brain natriuretic peptide) during follow-up after childhood cancer have been associated with irreversible cardiac damage. We aimed to evaluate strain imaging values by echocardiography and new biomarkers for heart failure with preserved ejection fraction (HFpEF) as potential more sensitive parameters for cardiac deterioration in childhood cancer survivors (CCS).

Materials And Methods: Prospective study with 50 CCS (median 16.

Assessment of ventricular flow dynamics by 4D-flow MRI in patients following surgical repair of d-transposition of the great arteries.

Objectives: To use 4D-flow MRI to describe systemic and non-systemic ventricular flow organisation and energy loss in patients with repaired d-transposition of the great arteries (d-TGA) and normal subjects.

Methods: Pathline tracking of ventricular volumes was performed using 4D-flow MRI data from a 1.5-T GE Discovery MR450 scanner.

Retraining Convolutional Neural Networks for Specialized Cardiovascular Imaging Tasks: Lessons from Tetralogy of Fallot.

Ventricular contouring of cardiac magnetic resonance imaging is the gold standard for volumetric analysis for repaired tetralogy of Fallot (rTOF), but can be time-consuming and subject to variability. A convolutional neural network (CNN) ventricular contouring algorithm was developed to generate contours for mostly structural normal hearts. We aimed to improve this algorithm for use in rTOF and propose a more comprehensive method of evaluating algorithm performance.

Additional value and new insights by four-dimensional flow magnetic resonance imaging in congenital heart disease: application in neonates and young children.

Cardiovascular MRI has become an essential imaging modality in children with congenital heart disease (CHD) in the last 15-20 years. With use of appropriate sequences, it provides important information on cardiovascular anatomy, blood flow and function for initial diagnosis and post-surgical or -interventional monitoring in children. Although considered as more sophisticated and challenging than CT, in particular in neonates and infants, MRI is able to provide information on intra- and extracardiac haemodynamics, in contrast to CT.

Real-World Experience Measurement of Liver Iron Concentration by R2 vs. R2 Star MRI in Hemoglobinopathies.

Background: Non-invasive determination of liver iron concentration (LIC) is a valuable tool that guides iron chelation therapy in transfusion-dependent patients. Multiple methods have been utilized to measure LIC by MRI. The purpose of this study was to compare free breathing R2* (1/T2*) to whole-liver Ferriscan R2 method for estimation of LIC in a pediatric and young adult population who predominantly have hemoglobinopathies.

Newborn girl with coarctation of the aorta and anomalous left coronary artery from pulmonary artery, with retrograde perfusion of left circumflex artery: a case report.

Background: Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare coronary abnormality. Although it exists usually as an isolated abnormality, ALCAPA has been described with aortic pathologies like coarctation or aortopulmonary window.

Case Summary: An 18-day-old female was admitted to the paediatric intensive care unit because of a heart murmur and weak femoral pulses.

Flow quantification dependency on background phase correction techniques in 4D-flow MRI.

Purpose: To analyze the dependence of flow volume measurements on 3D cine phase-contrast MRI (4D-flow MRI) background phase correction.

Methods: In 31 subjects scanned on a 1.5T MRI scanner, flow volume measurements at 4 vessels were made using phantom corrected 2D phase contrast and 4D flow with background phase correction performed by linear, second, third, and fourth-order polynomial fitting to static tissue.

Normal myocardial native T values in children using single-point saturation recovery and modified look-locker inversion recovery (MOLLI).

Background: T mapping is useful to quantify diffuse myocardial processes such as fibrosis, edema, storage disorders, or hemochromatosis. Normal pediatric myocardial T values are scarce using modified Look-Locker inversion recovery (MOLLI) sequences and unavailable using Smart1Map, a single-point saturation recovery sequence that measures true T .

Purpose/hypothesis: To establish normal pediatric myocardial T values by Smart1Map and to compare them with T by MOLLI.

Ductus arteriosus-associated infective endarteritis: Lessons from the past, future perspective.

Background: Since routine clinical use of antibiotics as well as surgical and catheter-based closure of a patent arterial duct (PDA), PDA-associated infective endarteritis (PDA-IE) is rare but can still occur when the ductus is still open or as it closes. Thus, clinicians should maintain a high index of concern for patients with unexplained fever.

Methods: We report on a PDA-IE in a young infant shortly after potentially delayed obliteration of a PDA.

Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root.

Familial hypercholesterolemia (FH) is an autosomal dominant disorder that affects 1 in 250 people. Aortic stiffness, measured by pulse wave velocity (PWV), is an independent predictor for cardiovascular events. Young FH patients are a unique group with early vessel wall disease that may serve to elucidate the determinants of aortic stiffness.