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PubMedDate not supplied

Unusual combination of congenital heart defects in an infant with Noonan syndrome.

Congenital heart disease occurs in 35-50% of patients diagnosed with Noonan syndrome. We present an infant with an unusual combination of congenital heart defects not previously reported, including partial atrioventricular septal defect, polyvalvular dysplasia, and progressive hypertrophic cardiomyopathy. We discuss the possible interaction between these lesions that may have led to the patient's rapid demise.

Angiography↗
PubMed2005

Understanding heart development and congenital heart defects through developmental biology: a segmental approach.

ABSTRACT The heart is the first organ to form and function during development. In the pregastrula chick embryo, cells contributing to the heart are found in the postero-lateral epiblast. During the pregastrula stages, interaction between the posterior epiblast and hypoblast is required for the anterior lateral plate mesoderm (ALM) to form, from which the heart will later develop. This tissue interaction is replaced by an Activin-like signal in culture. During gastrulation, the ALM is committed to the heart lineage by endoderm-secreted BMP and subsequently differentiates into cardiomyocyte. The right and left precardiac mesoderms migrate toward the ventral midline to form the beating primitive heart tube. Then, the heart tube generates a right-side bend, and the d-loop and presumptive heart segments begin to appear segmentally: outflow tract (OT), right ventricle, left ventricle, atrioventricular (AV) canal, atrium and sinus venosus. T-box transcription factors are involved in the formation of the heart segments: Tbx5 identifies the left ventricle and Tbx20 the right ventricle. After the formation of the heart segments, endothelial cells in the OT and AV regions transform into mesenchyme and generate valvuloseptal endocardial cushion tissue. This phenomenon is called endocardial EMT (epithelial-mesenchymal transformation) and is regulated mainly by BMP and TGFbeta. Finally, heart septa that have developed in the OT, ventricle, AV canal and atrium come into alignment and fuse, resulting in the completion of the four-chambered heart. Altered development seen in the cardiogenetic process is involved in the pathogenesis of congenital heart defects. Therefore, understanding the molecular nature regulating the 'nodal point' during heart development is important in order to understand the etiology of congenital heart defects, as well as normal heart development.

Animals↗
PubMed1993

Use of polytetrafluoroethylene surgical membrane as a pericardial substitute in the correction of congenital heart defects.

Patients who undergo repair of congenital heart defects have a high probability of eventually needing a repeat operation. In such cases, use of a pericardial substitute can preclude adhesion formation, thereby helping to avert the complications of a repeat sternotomy. This report describes our use of the GORE-TEX polytetrafluoroethylene surgical membrane in 321 patients who underwent repair of congenital heart disease at our hospital between May of 1989 and December of 1992 (these cases account for 37.1% of all such repairs performed during this period). The patients' ages ranged from 2 days to 31 years (mean, 5.9 years). Eighty-eight patients (27.4%) had at least 1 previous median sternotomy, and 234 (73.0%) had a moderate to high probability of reoperation. Seventy-eight patients (24.3%) received a homograft, which was positioned extracardially in 63 cases. During a maximum follow-up period of 43 months (mean, 26 +/- 5.6 months), 11 patients died, for an overall mortality of 3.4%. In no case was death attributable to membrane-related complications. Postoperative complications were encountered in 12 patients (3.7%), 10 of whom required an early repeat thoracotomy. One patient had mediastinitis, for an incidence of 0.3% (in contrast to 0.2% in 864 patients who underwent similar repair without the use of a surgical membrane). No complications were encountered in the 27 patients in whom the membrane was implanted during delayed sternal closure. Seventeen patients (5.5% of the survivors) underwent a repeat sternotomy, for further correction of congenital heart defects, a mean of 20 months after the original repair. At reoperation, major complications were encountered. Although the number of reoperations was low, we conclude that use of the polytetrafluoroethylene surgical membrane for pericardial closure in children is a safe procedure that helps prevent complications at reoperation.

Adolescent↗
PubMed1993

Polymorphisms and linkage disequilibrium in the COL6A1 and COL6A2 gene cluster: novel DNA polymorphisms in the region of a candidate gene for congenital heart defects in Down's syndrome.

The COL6A1 and COL6A2 (collagen VI) gene cluster on chromosome 21 is a candidate region for defects leading to congenital heart anomalies in Down's syndrome. We report a variable number of tandem repeats (VNTR) and a restriction fragment length polymorphism (RFLP) in this gene region, detected using a COL6A1 cDNA probe. Linkage disequilibrium relationships were studied among the RFLPs of this gene cluster. The RFLP reported here shows no significant linkage disequilibrium with any others in the region. It has a polymorphism information content value of 0.27, raising the informativity of the locus.

Chi-Square Distribution↗
PubMed1995

Congenital cardiovascular disease and cardiac surgery in childhood: Part 1. Cyanotic congenital heart defects.

The management of congenital heart disease is in a state of evolution, with earlier surgical and/or catheter interventions dominating clinical decision-making. Recent advances in interventional cardiac catheterization techniques, as well as continuing advances in the surgical management of complex congenital defects, continue to be the focus of attention of cardiologists and surgeons. The majority of the papers reviewed document mid- and long-term results of specific operative procedures for and address the appropriate role of cardiac catheterization techniques in the management of transposition of the great vessels, tetralogy of Fallot, total anomalous pulmonary venous connection, truncus arteriosus, pulmonary atresia with intact ventricular septum, and the univentricular heart. Early definitive intervention has become the standard of care for almost all defects reviewed.

Adolescent↗