1/74. The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition complexes.Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
2/74. Two-dimensional color doppler echocardiographic imaging of a Gerbode defect: a case report.A Gerbode defect is a ventricular septal defect that communicates directly between the left ventricle and the right atrium. The pathology may be due to a congenital defect, can result from trauma, or can occur after endocarditis or aortic valve replacement. We report the case of a 20-year-old man who has a defect between the left ventricle and the right atrium (Gerbode defect) that was diagnosed with two-dimensional color Doppler echocardiography.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
3/74. Unbalanced atrioventricular septal defect with parachute valve.A 5-month-old male patient presented with right-dominant unbalanced atrioventricular septal defect and left-sided parachute valve, and underwent successful biventricular repair. Because of the presence of a small left ventricle, left atrium, and a single left papillary muscle, an additional orifice was created in the left-sided atrioventricular valve with artificial partitioning of the right-sided atrioventricular valve. There was no evidence of mitral stenosis or regurgitation on follow-up echocardiography.- - - - - - - - - - ranking = 7keywords = valve (Clic here for more details about this article) |
4/74. Complete surgical repair of aortic atresia with a normal left ventricle.Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair.- - - - - - - - - - ranking = 32.677105130343keywords = atresia, valve (Clic here for more details about this article) |
5/74. pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection--prenatal echocardiographic diagnosis.The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.- - - - - - - - - - ranking = 187.54794667977keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
6/74. Prosthetic mitral valve replacement after atrioventricular septal defect repair: a technique for small children.An 11-month-old girl was transferred for consideration of cardiac transplantation. She had previously undergone repair of complete atrioventricular septal defect at another institution. Her postoperative course was notable for severe mitral regurgitation, pulmonary hypertension, and heart failure. At reoperation, the left atrioventricular valve was considered irreparable with a very small (11 mm) annulus. Using a technique to enlarge the mitral annulus, a 17-mm prosthetic valve was placed. Her postoperative course was unremarkable and she is doing very well at 3 years follow-up.- - - - - - - - - - ranking = 6keywords = valve (Clic here for more details about this article) |
7/74. Mitral atresia with premature closure of foramen ovale. A rare hemodynamic cause for failure of Blalock-Taussig anastomosis to relieve inadequate pulmonary blood flow.The clinical, hemodynamic, and surgical findings encountered in the management of a hypoxic male infant with a rare and complex variety of cyanotic congenital heart disease associated with inadequate pulmonary blood flow are described. A poor clinical response to creation of a Blalock-Taussig anastomosis led to the discovery of mitral atresia complicated by premature closure of the foramen ovale and partially relieved by the presence of a levoatriocardinal vein. The subsequent creation of an atrial septal defect enhanced the function of the subclavian artery to pulmonary artery anastomosis and provided palliative relief of hypoxia. Some of the clinical and laboratory findings indicating the presence of additional lesions complicating the picture of a tetralogy of fallot and requiring additional surgical considerations are discussed. The experience indicates that hemodynamic as well as surgical causes may explain the failure of a systemic artery to pulmonary artery anastomosis to function adequately and should be sought.- - - - - - - - - - ranking = 31.677105130343keywords = atresia (Clic here for more details about this article) |
8/74. Invasive intrauterine treatment of pulmonary atresia/intact ventricular septum with heart failure.The mortality and morbidity of children with pulmonary atresia/intact ventricular septum (PA/IVS) are linked to the degree of right ventricular (RV) hypoplasia. Opening up the pulmonary valve (PV) in fetal life could result in improved growth of the RV making it amenable to biventricular repair postnatally. Successful valvulotomy of the PV was performed in a fetus with heart failure at 28 weeks. Following the procedure there was significant growth of the tricuspid valve and RV. The neonate was delivered at 38 weeks with a RV suitable for biventricular repair. In utero pulmonary valvulotomy is feasible and may change the natural history of the condition in affected fetuses with PA/IVS.- - - - - - - - - - ranking = 837.32622460609keywords = pulmonary atresia, pulmonary valve, atresia, valve (Clic here for more details about this article) |
9/74. williams syndrome associated with complete atrioventricular septal defect.williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the syndrome. Complete atrioventricular septal defect has rarely been associated with williams syndrome and only one necropsy case has been reported in the literature. The long term follow up of williams syndrome associated with complete atrioventricular septal defect is reported. During a 10 year follow up period, the pressure gradient in the ascending aorta did not increase despite narrowing of the ascending aorta as identified on an aortogram.- - - - - - - - - - ranking = 2keywords = valve (Clic here for more details about this article) |
10/74. Surgical repair of a common atrium in an adult.We report a rare successful surgical repair of a common atrium (CA) with mild tricuspid valve (TV) regurgitation due to valvular annulus enlargement in a 39-year-old man, who had a complete atrial septum defect (ASD) without the characteristic of an endocardial cushion defect. The left-to-right shunt ratio was 85 percent and the Qp/Qs was 6.7 due to the CA. Left ventriculogram revealed no evidence of typical goose-neck deformity and no mitral valve regurgitation. The operation consisted of making a new atrial septum with an autologous pericardial patch and tricuspid annuloplasty (DeVega) using extracorporeal circulation. There was no evidence of a cleft on the anterior leaflet of the mitral valve or the septal leaflet of the TV. The postoperative echocardiogram showed no residual shunt flow through a new atrial septum and no TV regurgitation, and atrioventricular (AV) dissociation did not occur. We consider this procedure to be widely applicable in consideration of the favorable results obtained after surgical treatment.- - - - - - - - - - ranking = 3keywords = valve (Clic here for more details about this article) |
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