Correlation between three-dimensional echocardiography and cardiopulmonary fitness in patients with univentricular heart: A cross-sectional multicentre prospective study.

Background: The prognosis of patients with a functional single ventricle has improved, with better cardiopulmonary fitness, health-related quality of life and survival. Conventional echocardiography remains the first-line technique in single ventricle follow-up. Three-dimensional (3D) echocardiography has shown recent value in congenital cardiology, but its ability to predict functional status in patients with a single ventricle remains unknown.

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure.

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

Unlabelled: The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.

Background: Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient.

Cardiac strangulation: An atypical complication from epicardial pacemaker leads in a newborn.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified.

Tetralogy of Fallot with critical biventricular dysfunction: is surgical correction achievable?

We describe the case of a 32-month-old patient from a developing country with tetralogy of Fallot associated with a severe biventricular dysfunction. This association is rare but makes the surgical strategy complex and potentially contraindicated. An acute severe hypoxic episode led us to perform palliative rescue intervention involving the placement of an undersized systemic-to-pulmonary shunt.

Thymic-Specific Serine Protease Limits Central Tolerance and Exacerbates Experimental Autoimmune Encephalomyelitis.

The genetic predisposition to multiple sclerosis (MS) is most strongly conveyed by MHC class II haplotypes, possibly by shaping the autoimmune CD4 T cell repertoire. Whether Ag-processing enzymes contribute to MS susceptibility by editing the peptide repertoire presented by these MHC haplotypes is unclear. Thymus-specific serine protease (TSSP) is expressed by thymic epithelial cells and thymic dendritic cells (DCs) and, in these two stromal compartments, TSSP edits the peptide repertoire presented by class II molecules.

Image of a left atrial mass after a cardiopulmonary bypass in a child.

Invagination of an appendage into the left atrium is a rare complication. It occurs spontaneously or after open-heart surgery. In our case, a postoperative transesophageal echocardiogram, after closure of a ventricular septal defect in a 5-month-old infant, revealed a large mass in the left atrium.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

Objectives: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage.

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

Background: Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD).

Aims: To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers.

Assessment of Ventricular Septal Defect Size and Morphology by Three-Dimensional Transthoracic Echocardiography.

Background: Morphologic description of ventricular septal defect (VSD) is mandatory before performing the newly developed transcatheter closure procedure. Inaccurate estimation of defect size has been reported using conventional two-dimensional (2D) transthoracic echocardiography (TTE). The aim of this study was to assess VSD morphology and size using three-dimensional (3D) TTE compared with 2D TTE and surgery.

Mouse and human CD8(+) CD28(low) regulatory T lymphocytes differentiate in the thymus.

Regulatory T (Treg) lymphocytes play a central role in the control of immune responses and so maintain immune tolerance and homeostasis. In mice, expression of the CD8 co-receptor and low levels of the co-stimulatory molecule CD28 characterizes a Treg cell population that exerts potent suppressive function in vitro and efficiently controls experimental immunopathology in vivo. It has remained unclear if CD8(+) CD28(low) Treg cells develop in the thymus or represent a population of chronically activated conventional T cells differentiating into Treg cells in the periphery, as suggested by their CD28(low) phenotype.

3D transthoracic echocardiography to assess pulmonary valve morphology and annulus size in patients with Tetralogy of Fallot.

Background: Accurate evaluation of the pulmonary valve (PV) is crucial before surgical repair of Tetralogy of Fallot (TOF).

Aims: To assess PV and pulmonary annulus (PA) morphology using three-dimensional (3D) transthoracic echocardiography (TTE) in infants referred for surgical repair of TOF. Also, to compare PA measurements obtained by 3D TTE with those from other imaging modalities, including two-dimensional (2D) TTE and computed tomography (CT), with reference to surgical measurements.

The medical history of adults with complex congenital heart disease affects their social development and professional activity.

Background: In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD).

Aims: We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity.

Methods: We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.

Aortic arch aneurysm: short- and mid-term results comparing open arch surgery and the hybrid procedure†.

Objectives: Open arch surgery for aortic arch aneurysm was historically associated with a high risk of postoperative morbi-mortality. Improved operative techniques have now lowered the incidence of these complications but in parallel, hybrid arch procedures have emerged. Nowadays, very little data are available about their mid-term results compared with open surgery.

Safety and efficiency of the new micro-multiplane transoesophageal probe in paediatric cardiology.

Background: Transoesophageal echocardiography (TOE) is feasible in neonates using a miniaturized probe, but is not widely used because of low imaging quality.

Aims: To assess handling and imaging quality of a new release of a micro-TOE probe in children.

Methods: Thirty-eight consecutive children, enrolled during February and May 2013, underwent TOE with the Philips S8-3t probe.

Phase I trial: the use of autologous cultured adipose-derived stroma/stem cells to treat patients with non-revascularizable critical limb ischemia.

Background Aims: Non-revascularizable critical limb ischemia (CLI) is the most severe stage of peripheral arterial disease, with no therapeutic option. Extensive preclinical studies have demonstrated that adipose-derived stroma cell (ASC) transplantation strongly improves revascularization and tissue perfusion in ischemic limbs. This study, named ACellDREAM, is the first phase I trial to evaluate the feasibility and safety of intramuscular injections of autologous ASC in non-revascularizable CLI patients.

Comparison of two- and three-dimensional transthoracic echocardiography for measurement of aortic annulus diameter in children.

Background: Accurate evaluation of aortic root geometry is necessary in congenital aortic valve lesions in children, to guide surgical or angiographical intervention.

Aim: To compare aortic annulus diameters measured by two- and three-dimensional transthoracic echocardiography (2D- and 3D-TTE), to determine the feasibility and reproducibility of 3D imaging and assess the dynamic changes during the cardiac cycle.

Methods: Thirty children without heart disease were prospectively included.

Mechanical occlusion of the inferior vena cava: an early complication after repair of pectus excavatum using the Nuss procedure.

The Nuss procedure is the most widely used surgical procedure to correct pectus excavatum. Although it is a minimally invasive approach, a number of major early complications, such as heart perforation, have been reported. We describe a 15-year-old boy in whom acute occlusion of the inferior vena cava developed after a Nuss repair.

Benefits of extracorporeal membrane oxygenation for major blunt tracheobronchial trauma in the paediatric age group.

Tracheobronchial rupture due to blunt chest trauma is a rare but life-threatening injury among children. The severity of this condition ranges from death before hospital admission to clinical stability resulting in delayed management. Diagnosis is difficult because there is sometimes no evidence of external trauma, in spite of severe chest crush injury and consecutive rupture of airways.