1/104. Right lateral transthoracic approach mimicking standard transesophageal echocardiographic views in a patient with giant left atrium.We describe the case of a patient with long-standing severe mitral periprosthetic regurgitation and a giant left atrium. The patient was referred for surgery. On the third postoperative day, after resuture of the dehiscence of the valve sewing ring, the patient complained of dyspnea. Transthoracic ultrasound examination was performed to eliminate pleural effusion. The severe right lateral displacement of an aneurysmatic left atrial cavity contacting with the thoracic wall allowed us to obtain excellent images of the posterior cardiac anatomy by a right lateral thoracic view. The new transthoracic approach made it possible to safely assess the atrial side of the mitral prosthesis, eliminating mitral regurgitation after surgery without transesophageal echocardiographic examination.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
2/104. Neonatal marfan syndrome: a case report.A case of neonatal marfan syndrome is presented. The patient was noted to have cardiomegaly and tricuspid regurgitation on antenatal ultrasound scan. She was born with long, slender fingers and toes, an aged appearance and non-paralytic hypotonia. Echocardiogram revealed a dilated right atrium, right ventricle, dysplastic tricuspid valve and severe tricuspid regurgitation. She subsequently died of severe heart failure. Post-mortem examination showed the pathological features of lobar emphysema and cystic medial necrosis of the aorta. These features supported the diagnosis of neonatal marfan syndrome. Nucleotide sequencing showed substitution of G by A at codon 1032 in exon 25 located in the long arm of chromosome 15. This resulted in the substitution of a cysteine by a tyrosine. A de novo mutation is suggested by the absence of affected family members.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
3/104. pulmonary atresia with intact ventricular septum--a report of two cases with review of literature.Two perinatal autopsy cases are reported where a rare congenital anomaly, namely pulmonary atresia with intact ventricular septum in association with tricuspid stenosis and a hypoplastic right ventricle was encountered.- - - - - - - - - - ranking = 16.10780006011keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
4/104. Pathological and neuropathological findings in two males with fragile-X syndrome.The present paper addresses post mortem pathological and neuropathological findings in two males with fragile-X syndrome, aged 67 and 87 years. Both subjects died from sudden, unexpected cardiovascular causes, and both showed abnormalities of the mitral valve, ventricular hypertrophy and cardiomegaly. Both cases demonstrated macrocephaly characteristic of the classical Martin-Bell phenotype in FRAXA. There was increased brain weight in both cases: macroscopically, both cerebral and cerebellar hemispheres appeared normal, but dilated lateral ventricles were seen; and microscopic examination of the brain in case 2 showed normal hexalaminar architecture and no gross neuronal dropout. The hippocampus showed mild CA4 pyramidal cell loss and associated gliosis. The cerebellum showed focal Purkinje cell loss and corresponding Bergmann gliosis. Whilst there is a need to delineate the microscopic features of fragile-X syndrome from those of the ageing process, there is an urgent need for more systematic neuropathological studies of fragile-X syndrome; the increased brain weight and Purkinje cell loss in autism and fragile-X syndrome reopens the debate on these two conditions. The case for further research into the cardiac anomalies in fragile-X syndrome is also strengthened by the findings. Finally, the present report confirms the role of interstitial cell hyperplasia as the major cause of megalo-testes in this condition.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
5/104. Autotransplantation procedure for giant left atrium repair.BACKGROUND: Giant left atrium has been associated with bronchopulmonary and left ventricular compression [Kawazoe 1983]. CASE REPORT: We present a patient with severe congestive heart failure (CHF), respiratory insufficiency and a giant left atrium (GLA) following two previous mitral valve procedures and tricuspid valve annuloplasty in the distant past. Mitral prosthetic function and ventricular systolic function were felt to be normal leading to a tentative diagnosis of diastolic restriction from left ventricular compression and pericardial constriction. A pericardial decortication procedure through left thoracotomy was initially done but proved ineffective. Subsequently, full evidence of hemodynamic failure due to the giant left atrium and its respiratory complication was recognized and the patient underwent cardiac autotransplantation procedure [Kosak 1987], with the aim to reduce the left atrial dimensions to normal. CONCLUSIONS: Calcification of posterior left atrial wall prevented a completely satisfactory reduction of atrial size and the severity of ventricular adhesions from the previous pericardial procedure resulted in very long cardiopulmonary bypass time with severe bleeding complications. This case provides ample evidence that GLA can cause respiratory failure and needs to be surgically corrected.- - - - - - - - - - ranking = 2keywords = valve (Clic here for more details about this article) |
6/104. A novel technique for giant left atrium reduction.We herein describe a safe and reproducible technique for left atrial volume reduction in patients with a giant left atrium. In a 56-year-old patient undergoing redo mitral valve replacement, the left atrium measured 18 x 20 x 17 cm occupying the middle-lower segment of the right hemithorax with compression of the adjacent organs. The left atrial volume was reduced by triangular resections of the atrial wall and the mitral valve was replaced using a mechanical prosthesis. The postoperative course was uneventful and the left atrial diameter was 11.2 cm at the latest control.- - - - - - - - - - ranking = 2keywords = valve (Clic here for more details about this article) |
7/104. Left atrial reduction and mitral valve replacement in a 5-year-old girl with severe mitral regurgitation and giant left atrium.Giant left atrium associated with mitral valve disease has been implicated in the morbidity following mitral valve repair or replacement. Various methods including left atrial plication have been described to reduce the size of left atrium. Herein we describe our technique of left atrial reduction in a 5-year-old girl with severe mitral regurgitation and giant left atrium. She underwent mitral valve replacement and circumferential left atrial reduction with successful outcome.- - - - - - - - - - ranking = 7keywords = valve (Clic here for more details about this article) |
8/104. Giant left atrium secondary to tight mitral stenosis leading to acquired lutembacher syndrome: a case report with emphasis on role of echocardiography in assessment of lutembacher syndrome.lutembacher syndrome is an unusual clinical entity of congenital secundum atrial septal defect in combination with rheumatic mitral stenosis. Although this classic form is seldom seen by the adult cardiologist, spontaneous lutembacher syndrome as discussed later or the iatrogenic variant is not infrequently encountered. The pathophysiologic, clinical, and hemodynamic differences of mitral valve disease in the presence of atrial septal defect compared with isolated mitral stenosis are highlighted in this case review. Special emphasis has also been given to echocardiographic evaluation of this syndrome complex, particularly in the setting of percutaneous mitral valvuloplasty, which produces the iatrogenic form of lutembacher syndrome.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
9/104. Congenitally unguarded tricuspid valve orifice with a giant right atrium and a massive clot in an asymptomatic adult.Congenitally unguarded tricuspid valve orifice, a variant of tricuspid valve dysplasia, is a rare malformation with protean manifestations. This report describes an asymptomatic adult who, on echocardiographic examination ordered in view of an abnormal 12-lead surface electrocardiogram and plain chest X-ray, was found to have an unguarded tricuspid valve orifice with a giant right atrium (12 x 10 cm), intense spontaneous echo contrast and a large right atrial clot.- - - - - - - - - - ranking = 7keywords = valve (Clic here for more details about this article) |
10/104. Asymptomatic giant right atrial aneurysm.A 5-year-old asymptomatic boy was found incidentally, on a chest radiograph, to have gross cardiomegaly; further evaluation by echocardiography showed a giant right atrial aneurysm. The patient underwent successful surgical reduction of the right atrium, closure of an atrial septal defect, and tricuspid valve repair These measures were taken to prevent thrombus formation in the right atrium, prevent paradoxical embolism, and lower the risk of atrial arrhythmias. The morphologic features of the resected atrial tissue showed paper-thin wall with a central aneurysm and focal endocardial fibrosis consistent with a diagnosis of idiopathic dilatation of the right atrium.- - - - - - - - - - ranking = 1keywords = valve (Clic here for more details about this article) |
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