Extrathoracic heart in northern Cameroon: a case report

Tantchou et al report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon. Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. Ectopia cordis is frequently associated with other congenital defects involving multiple organ systems. We report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon

The Shisong Cardiac Center in Cameroon: An Example of a Long-Term Collaboration/Cooperation Toward Autonomy

Congenital heart diseases (CHD) are present in nearly 1% of live births; according to WHO, there are 1. 5 million newborns affected by CHD per year and more than 4 million children waiting for cardiac surgery treatment worldwide. The majority of these children (~90%) could be treated, saved and subsequently have a good quality of life but unfortunately, in developing countries with a suboptimal care or no access to care, they are destined to die. Cameroon, one of the 40 poorest countries in the world, is a typical example of this dramatic scenario and this is why we started a collaboration project with a local religious partner (Tertiary Sisters of Saint Francis) in 2001 with the aim of establishing the first cardiac surgery center in this country. There are various well-known organizational models to start a cooperation project in pediatric cardiac surgery in a developing country. In our case, the project included a long-term collaboration with a stable local partner, a big financial investment and a long period of development (10 years or more). It is probably the most difficult model but it is the only one with the greatest guarantee of success in terms of sustainability and autonomy. The aim of this study is to analyze the constructive and problematic aspects of the 17-year collaboration in this project, and to assess possible solutions regarding its critical issues. Although much has been done during this 17-year we are aware that there is still a lot that needs to be done

Transcatheter Closure of Perimembranous Ventricular Septal Defects

n/

Right ventricular restoration during pulmonary valve implantation in adults with congenital heart disease☆

n/

Transcatheter closure of atrial septal defect in young children Results and follow-up

AbstractObjectivesThis study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children.BackgroundResults of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children.MethodsBetween December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age ≤5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter).ResultsThe mean age at closure was 3.6 ± 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 ± 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly.ConclusionsIn the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children

Thymic Epithelium Abnormalities in DiGeorge and Down Syndrome Patients Contribute to Dysregulation in T Cell Development

The thymus plays a fundamental role in establishing and maintaining central and peripheral tolerance and defects in thymic architecture or AIRE expression result in the development of autoreactive lymphocytes. Patients with partial DiGeorge Syndrome (pDGS) and Down Syndrome (DS) present alterations in size and architecture of the thymus and higher risk to develop autoimmunity. We sought to evaluate thymic architecture and thymocyte development in DGS and DS patients and to determine the extent to which thymic defects result in immune dysregulation and T cell homeostasis perturbation in these patients. Thymi from pediatric patients and age-matched controls were obtained to evaluate cortex and medullary compartments, AIRE expression and thymocyte development. In the same patients we also characterized immunophenotype of peripheral T cells. Phenotypic and functional characterization of thymic and peripheral regulatory T (Treg) cells was finally assessed. Histologic analysis revealed peculiar alterations in thymic medulla size and maturation in DGS and DS patients. Perturbed distribution of thymocytes and altered thymic output was also observed. DGS patients showed lower mature CD4+ and CD8+ T cell frequency, associated with reduced proportion and function of Tregs both in thymus and peripheral blood. DS patients showed increased frequency of single positive (SP) thymocytes and thymic Treg cells. However, Tregs isolated both from thymus and peripheral blood of DS patients showed reduced suppressive ability. Our results provide novel insights on thymic defects associated with DGS and DS and their impact on peripheral immune dysregulation. Indeed, thymic abnormalities and defect in thymocyte development, in particular in Treg cell number and function could contribute in the pathogenesis of the immunodysregulation present in pDGS and in DS patients

The Right Ventricle in Adults with Tetralogy of Fallot

XVIII, 194 p.online resource