1/14. A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/14. 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 = 0.4keywords = vascular disease (Clic here for more details about this article) |
3/14. Postoperative management of children with biliary atresia and heart failure.The postoperative course in two children with extrahepatic biliary atresia and cardiovascular disease was reviewed and the correlation between biliary drainage and cardiac function was analyzed. Both patients obtained satisfactory biliary drainage after Kasai's hepatic portoenterostomy. One patient developed heart failure postoperatively due to severe viral myocarditis. This child's total serum bilirubin concentration remained elevated for eight months, despite adequate bilirubin excretion, until her cardiac function returned to normal. Another patient died of cardiac failure due to congenital heart disease 83 days after Kasai's operation, but his postoperative biliary drainage was satisfactory as long as cardiac function remained compensated. In both cases, fluid intake was restricted severely (30 to 70 ml/kg body weight/day), as titrated by echocardiographic assessment of cardiac function, but biliary excretion was satisfactory as long as the cardiac fractional shortening ratio was greater than 30% and the ejection fraction was greater than 55%. This suggests that cardiac decompensation affects postoperative biliary excretion in patients with biliary atresia; however, with careful medical management satisfactory biliary drainage can be achieved even in patients with severe heart diseases.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
4/14. Aortopulmonary window coexisting with tetralogy of fallot: echocardiographic diagnosis.Aortopulmonary window coexisting with tetralogy of fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
5/14. Thoracic cage deformities in the early diagnosis of the marfan syndrome and cardiovascular disease.The marfan syndrome is frequently complicated by cardiovascular abnormalities. Of these, aortic dissection and aortic valve regurgitation are the most life-threatening. The most noticeable abnormalities of the marfan syndrome--the skeletal abnormalities--may be subtle and limited. Presented here are five reports of cases of the marfan syndrome. All patients had potentially lethal cardiovascular complications. Either the syndrome had not been previously diagnosed or the patient had not been adequately monitored despite the the presence of thoracic cage deformities present from youth. The purpose of this report is to heighten recognition of the association of thoracic cage deformities with the marfan syndrome to permit early diagnosis of the associated cardiovascular complications. Surgical management of these complications can favorably alter the natural history of the marfan syndrome.- - - - - - - - - - ranking = 0.8keywords = vascular disease (Clic here for more details about this article) |
6/14. magnetic resonance imaging in pediatric vascular disease.magnetic resonance imaging (MRI) was used to study eight children with known vascular disease proved by angiography. Congenital and acquired large vessel disease was equally well demonstrated noninvasively by both MRI and angiography, and in one patient MRI provided superior information about pulmonary artery patency.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/14. cardiovascular diseases due to viruses.In acute myocardiopathy and pericardiopathy, Coxsackie virus B infections are increasingly recognized as a cause of primary myocardial disease and may cause chronic cardiovascular disease. With other viruses, such as cytomegaloviruses and rubella virus, transplacental infection during pregnancy may occur, and this can cause the congenital rubella syndrome, which involves heart abnormalities. Other viruses are now under study. myocarditis was observed in a newborn infant infected with Coxsackie virus B3, which was isolated by tissue culture methods. Experimental infection of Coxsackie virus A and B was studied in suckling mice and the histopathologic changes in heart muscle were observed. Laboratory findings of viral infection are very useful for clinical diagnosis, however care needs to be taken with respect to the obtaining of specimens, diagnostic procedures, and the assessment of results.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/14. Pulmonary vascular disease in children with congenital cardiovascular malformations. Etiologic considerations.A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation.- - - - - - - - - - ranking = 0.8keywords = vascular disease (Clic here for more details about this article) |
9/14. Sudden death and partial absence of the right ventricular myocardium: a report of three cases and a review of the literature.Three patients with congenital partial absence of the right ventricular myocardium were studied. These cases are unique in that all three patients died suddenly and none had clinical evidence of cardiovascular disease. Two of the three patients were active in sports, and both died suddenly while playing basketball. At the time of autopsy, the only significant abnormality was cardiomegaly, with right atrial and ventricular dilation and partial absence of the right ventricular myocardium.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
10/14. Surgical treatment of infantile lobar emphysema in cardiovascular disease with left-to-right shunts.Serious symptoms associated with the lobar emphysema in congenital heart diseases with left-to-right shunt disappeared after radical operation for cardiac lesion. The study of seven autopsied cases revealed that the lobar emphysema resulted from check valve mechanism created by compression of the bronchi by distended pulmonary artery. Eight cases of our experience of lobar emphysema disappeared shortly after the operation for congenital heart disease with left-to-right shunt seems to indicate infantile lobar emphysema in congenital heart diseases with left-to-right shunt should not be treated by resection but rather by radical operation for cardiac lesion as soon as possible even in infancy.- - - - - - - - - - ranking = 0.8keywords = vascular disease (Clic here for more details about this article) |
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