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Double Outlet Right Ventricle (double + outlet_right_ventricle)
Selected AbstractsDisturbed morphogenesis of cardiac outflow tract and increased rate of aortic arch anomalies in the offspring of diabetic ratsBIRTH DEFECTS RESEARCH, Issue 12 2004Daniėl G.M. Molin Abstract BACKGROUND Maternal diabetes (MD) is a risk factor for offspring to develop cardiovascular anomalies; this is of growing clinical concern since the number of women in childbearing age with compromised glucose homeostasis is increasing. Hyperglycemia abrogates cardiovascular development in vitro; however, a link to cardiovascular defects in diabetic offspring remains to be investigated. METHODS We have studied cardiovascular development in offspring of MD rats by examining serial histological sections of GD 12.0,18.0 offspring. Development of pharyngeal arch artery malformations was analyzed and related to intracardiac anomalies. RESULTS Pharyngeal arch artery and intracardiac defects were present in 27 of 37 MD GD 13.0,18.0 offspring. Early sixth arch arteries showed abrogated arteriogenesis, whereas fourth arch artery defects developed as a result of abnormal remodeling. Morphometrical analysis showed increased apoptosis in regressing artery segments and reduced apoptosis in persisting artery segments. Double outlet right ventricle with infundibular stenosis (tetralogy of Fallot) was predominantly found in combination with sixth artery defects and pulmonary atresia. As confirmed by morphometric analysis and three-dimensional (3D)-reconstructions, outflow tract defects coincided with endocardial cushion hypoplasia. Cases with teratology of Fallot additionally showed a shorter outflow tract. No relation with apoptosis or disturbed neural crest cell migration was found. CONCLUSIONS Our data uniquely demonstrate mechanistic differences involved in the development of sixth and fourth artery anomalies. Whereas increased apoptosis induces fourth artery anomalies, pulmonary outflow obstruction abrogates sixth artery differentiation independent of apoptosis. The model presented allows analysis of diabetic conditions on cardiovascular development in vivo, essential for elucidating this teratology. Birth Defects Research (Part A), 2004. © 2004 Wiley-Liss, Inc. [source] Resternotomy for a Retrosternal Cardiac Pseudoaneurysm in a 1.5-Year- Old Child: A Case ReportJOURNAL OF CARDIAC SURGERY, Issue 5 2005Ghassan M. Baslaim M.D. The wall of the pseudoaneurysm consists of fibrous tissue and lacks the structural elements found in a normal cardiac wall, and it is contained by the pericardial adhesions or the epicardial wall. Early surgery is recommended even for asymptomatic patients due to the propensity for rupture and fatal outcome. We report our experience with the surgical approach of a child with a cardiac pseudoaneurysm who had undergone a biventricular repair of a double outlet right ventricle with non-committed ventricular septal defect in the form of intraventricular tunneling. [source] Angioarchitecture of the venous and capillary system in heart defects induced by retinoic acid in mice,BIRTH DEFECTS RESEARCH, Issue 7 2009Anna Ratajska Abstract BACKGROUND: Corrosion casting and immunohistochemical staining with anti-alpha smooth muscle actin and anti-CD34 was utilized to demonstrate the capillary plexus and venous system in control and malformed mouse hearts. METHODS: Outflow tract malformations (e.g., double outlet right ventricle, transposition of the great arteries, and common truncus arteriosus) were induced in progeny of pregnant mice by retinoic acid administration at day 8.5 of pregnancy. RESULTS: Although control hearts exhibited areas in which capillaries tended to be oriented in parallel arrays, the orientation of capillaries in the respective areas of malformed hearts was chaotic and disorganized. The major branch of a conal vein in control hearts runs usually from the left side of the conus to its right side at the root of the pulmonary trunk and opens to the right atrium below the right auricle; thus, it has a curved course. On the other hand, a conal vein in malformed hearts courses from the left side or from the anterior side of the conus and tends to traverse straight upwards along the dextroposed aorta or along the aortopulmonary groove with its proximal part located outside of the heart. Other cardiac veins in outflow tract malformations are positioned in the same locations as in control hearts. CONCLUSIONS: We postulate that the changed location of the conal vein and disorganized capillary plexus result from malformed morphogenesis of the outflow tract and/or a disturbed regulation of angiogenic growth factor release from the adjacent environment. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc. [source] Endovascular stent implantation in the pulmonary arteries of infants and children without the use of a long vascular sheathCATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 4 2002Robert H. Pass MD Abstract Endovascular stent implantation for pulmonary artery stenosis requires the use of a long, large-bore vascular sheath to insure precise implantation without embolization or malposition. A long vascular sheath may be difficult to position and usage may be associated with vascular compromise and/or hemodynamic embarrassment, especially in infants and small children. We report a new technique for pulmonary artery endovascular stent implantation without the use of a long sheath. From December 2000 to May 2001, 10 patients underwent implantation of 13 Palmaz Corinthian premounted biliary transhepatic stents for pulmonary artery stenosis. Median age was 0.8 years (range, 0.5,18.5) and median weight was 11.8 kg (range, 4.6,65). Patient diagnoses were tetralogy of Fallot (five), double outlet right ventricle (three), branch peripheral pulmonary artery stenosis (two), single ventricle s/p cavopulmonary shunt (one), and truncus arteriosus (one). All Palmaz Corinthian stents were delivered uncovered on Cordis Opta LP balloon catheters via short sheaths (6,7 Fr); super-stiff guidewires were not always necessary. These stents, with a maximal expanded diameter of 12 mm, were placed for peripheral pulmonary artery stenosis as a definitive procedure or at the pulmonary artery bifurcation in patients who were expected to undergo future open heart surgery. The stents were initially implanted on 4, 6, or 8 mm balloon catheters and further expanded if needed. Stents were placed in the right pulmonary artery alone in three patients, left pulmonary artery alone in four patients, and side-by-side stents were implanted simultaneously in three patients. All thirteen stents were implanted successfully in the desired location without stent malposition or embolization. Mean angiographic diameter increased from 2.5 ± 1.5 to 5.7 ± 1.4 mm (P < 0.01) and peak systolic ejection gradients decreased from 44 ± 22 to 14 ± 11.6 mm Hg (P < 0.01). The uncovered delivery of the premounted Palmaz Corinthian stent allowed for precise and safe endovascular stent implantation without the hemodynamic and technical problems associated with long vascular sheath usage. This technique is useful for the palliation of proximal pulmonary artery stenosis and is effective definitive treatment for peripheral pulmonary artery stenosis in small infants and children. Cathet Cardiovasc Intervent 2002;55:505,509. © 2002 Wiley-Liss, Inc. [source] | ||||||||||