Minipuberty and Sexual Dimorphism in the Infant Human Thymus.

AIRE expression in thymus is downregulated by estrogen after puberty, what probably renders women more susceptible to autoimmune disorders. Here we investigated the effects of minipuberty on male and female infant human thymic tissue in order to verify if this initial transient increase in sex hormones - along the first six months of life - could affect thymic transcriptional network regulation and AIRE expression. Gene co-expression network analysis for differentially expressed genes and miRNA-target analysis revealed sex differences in thymic tissue during minipuberty, but such differences were not detected in the thymic tissue of infants aged 7-18 months, i.

Double Root Switch Repair for Taussig-Bing Anomaly.

The double root switch was used to repair a Taussig-Bing anomaly in a two-month-old child. The surgery was performed with translocation of the aortic root to the left ventricular outflow tract and the pulmonary root to the right ventricular outflow tract, after closure of the ventricular septal defect with a patch that ultimately directed flow from the left ventricle to the aorta. This surgical approach has the theoretical advantage of reducing the likelihood of subneopulmonary obstruction, left ventricular outflow obstruction, neoaortic dilatation, and regurgitation and therefore has the potential to minimize the mortality and morbidity related to reintervention and reoperation.

Modular transcriptional repertoire and MicroRNA target analyses characterize genomic dysregulation in the thymus of Down syndrome infants.

Trisomy 21-driven transcriptional alterations in human thymus were characterized through gene coexpression network (GCN) and miRNA-target analyses. We used whole thymic tissue--obtained at heart surgery from Down syndrome (DS) and karyotipically normal subjects (CT)--and a network-based approach for GCN analysis that allows the identification of modular transcriptional repertoires (communities) and the interactions between all the system's constituents through community detection. Changes in the degree of connections observed for hierarchically important hubs/genes in CT and DS networks corresponded to community changes.

Mixed type total anomalous pulmonary venous connection: early results and surgical techniques.

Introduction: The mixed total anomalous pulmonary connection is a rare type of congenital cardiopathy. The occurrence of multiple connections of the pulmonary veins has important implication in the diagnosis and surgical planning. These types of total anomalous pulmonary venous connection (TAPVC) and the infracardiac have higher surgical mortality.

Double root switch: a complete anatomical correction for transposition of the great arteries.

We report the cases of two neonates with transposition of the great arteries, whose surgical correction consisted of translocation of the aortic root with the aortic valve and coronary arteries to the left ventricle and translocation of the pulmonary root with the pulmonary valve to the right ventricle. We believe that the aortic translocation may reduce the likelihood of dilatation of the neoaorta, aortic regurgitation, and changes in the endothelium of coronary arteries which have occasionally been seen following arterial switch operations.

Balloon atrial septostomy guided by echocardiography in a neonatal intensive care unit.

Background: The bedside two-dimensional echocardiography (2-D ECHO) has been successfully used to guide the balloon atrial septostomy, speeding up the procedure and preventing the risks of transportation to the hemodynamics laboratory.

Objective: To assess the results of the bedside balloon atrial septostomy in cyanotic neonates.

Methods: Between January/1997 and July/2008, 102 atrioseptostomies by balloon catheter guided by echocardiography were carried out and saturation levels, defect diameter, oxygen saturation, clinical-laboratory response and complications related to the procedure were evaluated.

New technique: aortic and pulmonary translocation with preservation of pulmonary valve.

We applied successfully, a new surgical technique, in two children with transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, that utilized aortic translocation with the aortic valve and the coronary arteries to the left ventricle, after correction of left outflow tract obstruction and correction of the ventricular septal defect, associated to pulmonary root translocation to the right ventricle, conserving integrally the pulmonary valve.

New technique: Norwood operation with regional cerebral and coronary perfusion.

Objective: To assess the immediate result of the modified Norwood procedure with new technique of anterograde regional cerebral perfusion (ARCeP) and retrograde regional coronary perfusion (RRCoP) in substitution of profound hypothermia and circulatory arrest in children with hypoplastic left heart syndrome (HLHS) with extremely hypoplastic ascending aorta (HA).

Methods: In the period of December of 2006 to February 2008 the modified Norwood procedure with tube between the right ventricle and pulmonary arteries or Sano shunt was performed in eight children with HLHS and with ascending aorta diameter less than 3 mm, (four male and four female) with median age of 9.2 days (ranging from 1 to 29 days) and median weigh of 3.

Biointegration and growth of porcine valved pulmonary conduits in a sheep model.

As there is currently no suitable valved pulmonary conduit for small children, porcine conduits treated by the L-Hydro process were implanted into 9 newborn lambs to investigate growth potential. Of the 8 survivors, 7 were kept alive for 12 months after implantation. The diameter of the conduit and gradient across the valve were evaluated at surgery and at 3 and 9 months postoperatively using bidirectional echocardiographic and angiographic methods.

[The role of echocardiography as an isolated method for indicating surgery in patients with congenital heart disease].

Objective: To determine the diagnostic accuracy of echocardiography for indicating surgery without catheterization in patients with congenital heart disease through a prospective analysis and comparison of the echocardiographic diagnosis with the intraoperative findings, or invasive study, or both.

Methods: From February 2000 to January 2001, 493 patients with congenital heart diseases indicated for surgery were followed up. They underwent echocardiography with color-flow mapping for a therapeutic decision.