1/218. Complete atrioventricular septal defect and Ebstein's anomaly.A case child with complete atrioventricular septal defect (AVSD) and Ebstein's anomaly underwent surgical treatment at 3 months of age. She died on the third postoperative day. Postmortem examination showed complete AVSD, downward displacement of the right atrioventricular valve, left ventricular outflow tract obstruction, and hypertensive pulmonary vascular disease. association of complete AVSD and Ebstein's anomaly is a rare cardiac anomaly for which no attempt at surgical repair has previously been made. This report deals with our experience and also with the morphological features of this anomaly.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
2/218. Autosomal dominant secundum atrial septal defect with various cardiac and noncardiac defects: a new midline disorder.We report on a Lebanese family in which 12 persons had an atrial septal defect and various cardiac and noncardiac anomalies. Cardiac anomalies are left axis deviation of QRS, right bundle branch block, atrial fibrillation, wolff-parkinson-white syndrome, nodal atrioventricular rhythm, aortic stenosis, pulmonic valve stenosis, mitral stenosis (lutembacher syndrome), and low implantation of the tricuspid valve (Ebstein disease). Noncardiac abnormalities consisted specially of the presence of hypertelorism, cleft lip, and pectus excavatum. This combination appears to constitute a hitherto undescribed autosomal dominant midline disorder of the heart and upper half of the body with almost full penetrance and variable expressivity. The mutation does not map to any known locus involved in atrial septal defect or conduction block.- - - - - - - - - - ranking = 2keywords = valve (Clic here for more details about this article) |
3/218. Atrioventricular septal defect with separate right and left atrioventricular valvar orifices in a patient with foetal hydantoin syndrome.The teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
4/218. An unusual tethering of the bridging leaflets in atrioventricular septal defect producing a communication from left atrium to right ventricle.We describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
5/218. Chiari network entanglement and herniation into the left atrium by an atrial septal defect occluder device.The Chiari network is a fenestrated membrane consisting of threads and strands in the right atrium. First described in 1897 by anatomist Hans Chiari, it is a congenital remnant of embryonic development resulting from incomplete resorption of the right valve of the sinus venosus. Found in 2% to 3% of the population, it is generally not of clinical importance. Rarely, however, the network may be associated with serious complications such as thrombus formation, embolus entrapment, arrhythmia, tumor development, and catheter entrapment. We report the entanglement of an Amplatzer septal occluder device catheter in a prominent Chiari network that was herniated into the left atrium. Transesophageal echocardiographic recognition of this before deployment and guidance during disentanglement is described below.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
6/218. Transcatheter closure of a patent foramen ovale following mitral valve replacement.We report the successful closure of a postoperative patent foramen ovale in a patient who underwent coronary artery bypass grafting and mitral valve replacement for severe mitral insufficiency. The postoperative course was complicated by severe hypoxemia due to a large patent foramen ovale. The patient underwent transcatheter closure with the Das Angel Wings transcatheter occluder (Microvena Corporation, White Bear Lake, MN) with immediate improvement.- - - - - - - - - - ranking = 5keywords = valve (Clic here for more details about this article) |
7/218. Divided right atrium. Diagnosis by echocardiography, and considerations on the functional role of the Eustachian valve.A child presented at birth with severe cyanosis. echocardiography showed hypoplasia of the right heart with a right-to-left shunt at atrial level. A conservative approach was adopted initially, and the situation improved over a few months, with reversal of the atrial shunt. Surgery was successfully performed at 4 years of age after further echocardiography revealed a congenitally large Eustachian valve and an atrial septal defect.- - - - - - - - - - ranking = 5keywords = valve (Clic here for more details about this article) |
8/218. Atrial septal defect as an uncommon cardiovascular malformation with Turner's syndrome.Cardiovascular malformations are frequently observed in Turner's syndrome. bicuspid aortic valve and coarctation of aorta are commonly associated with Turner's syndrome while an atrial septal defect is unusual. Here report a rare case of atrial septal defect with Turner's syndrome.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
9/218. Bivalvation for common atrioventricular valve regurgitation in cyanotic heart disease.We describe the case of a 15-year-old boy with complex heart disease, in whom we successfully performed bivalvation for common atrioventricular valve regurgitation and bilateral, bidirectional cavopulmonary shunts. Postoperative Doppler echocardiography revealed significant reduction of atrioventricular valve regurgitation.- - - - - - - - - - ranking = 6keywords = valve (Clic here for more details about this article) |
10/218. family cluster of atrial septal defect.Atrial septal defect is one of the most common congenital cardiac defects, occurring in 9% to 15% of live births. This defect has been reported to have a genetic etiology in some cases, although the full extent of genetic involvement in atrial septal defect is not known. Because symptoms are often lacking in childhood and manifest primarily in adulthood and because physical findings may be lacking, it is important to take a family history in patients in whom the defect is suspected. When evaluating children, a family history of atrial septal defect should raise suspicion of increased risk so that early diagnosis and treatment can be made. The author reports a cluster of 11 diagnosed atrial septal defects within one 32-member family group, 6 of whom required surgery to repair the defects. It is significant that most of the individuals were lacking in objective findings on physical examination, notably a murmur, as well as in subjective complaints. Diagnoses were made by use of two-dimensional echocardiography with color-enhanced Doppler ultrasonography in all but two cases--one diagnosed with transesophageal echocardiography and one, serendipitously, during cardiac catheterization. echocardiography also revealed mitral valve prolapse, thin atrial septal wall, with aneurysm in some cases, and regurgitation of mitral or tricuspid valves in several individuals. Cardiac enlargement, usually of the right atrium, was seen in most of the individuals.- - - - - - - - - - ranking = 2keywords = valve (Clic here for more details about this article) |
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