1/8. Evolution of left ventricular diseasein the fetus. Case report.A fetal case is described that showed a rapid progression from the features of initial left ventricular fibroelastosis at 20 weeks of gestation to a more marked dilation at 22 weeks and finally to a hypoplastic left ventricle with aortic stenosis at 24 weeks of gestation. This case confirms the evolutive character of left ventricular disease during fetal life.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/8. Electron beam computed tomography appearance of endocardial fibroelastosis EBCT appearance of endocardial fibroelastosis.Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/8. Incidental disclosure of asymptomatic coronary embolic occlusion related to mitral valve papillary fibroelastoma: an unusual finding and a review of the literature.Cardiac papillary fibroelastoma (CPF) located on mitral and aortic valves are known to produce systemic embolism mainly represented by strokes, whereas myocardial infarction and sudden death usually result from specific locations around LVOT. Coronary artery embolic occlusion originating from a mitral location has not yet been reported. The case is reported of a 42-year-old man referred for surgical treatment of a mitral valve papillary fibroelastoma disclosed after transitory and completely regressive left hemicorporeal deficiency and previous myocardial infarct. Due to the left chamber location, surgery was scheduled and complete removal of the mass achieved. These findings emphasize the potential life-threatening complications of CPF and, independent of risk factors, the need to perform systematic coronary angiography before surgical excision is considered.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/8. Balloon dilatation of the aortic valve in the fetus: a report of two cases.Because they had irreversible damage to the left ventricular myocardium none of 12 patients with critical aortic stenosis diagnosed prenatally survived after postnatal treatment. This experience prompted three attempts at intrauterine balloon dilatation of the aortic valve in two fetuses with this condition. On each attempt the balloon catheter was successfully delivered to the left ventricle. In the first fetus the aortic valve was not crossed and the fetus died the next day. In the second fetus the balloon was correctly positioned across the aortic valve and inflated in the valve ring. After delivery, a further balloon angioplasty was performed; this relieved the stenosis but the patient died five weeks later from persisting left ventricular dysfunction related to endocardial fibroelastosis. Balloon angioplasty is feasible in fetal life but the prognosis depends on the ability of the relief of stenosis to limit, prevent, or allow regression of left ventricular damage before delivery.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/8. mucopolysaccharidosis i presenting with endocardial fibroelastosis of infancy.We describe two female infants with Hurler syndrome (mucopolysaccharidosis i) whose deaths are attributed to cardiac failure with associated, autopsy-confirmed endocardial fibroelastosis. One infant had confirmed alpha-L-iduronidase deficiency in cultured dermal fibroblasts, and the other infant had histologic evidence of tissue mucopolysaccharide accumulation at autopsy and a sibling with confirmed alpha-L-iduronidase deficiency and the Hurler syndrome phenotype. Clear cells ("Hurler" cells) were identified within the myocardium and endocardium of both infants. We propose that the ventricular mural accumulation of mucopolysaccharides induced extensive proliferation of elastic or collagen fibers within the endocardium. Cardiac failure may precede recognition of clinical and roentgenographic features of Hurler syndrome. Our findings and a literature review suggest that certain heritable storage disorders, including mucopolysaccharidosis i, should be considered when infants have clinical electrocardiographic and echocardiographic findings consistent with endocardial fibroelastosis or have autopsy-documented endocardial fibroelastosis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/8. Contracted form of primary endocardial fibroelastosis in a young adult without congestive heart failure.A case of a 24-year-old man with the contracted form of primary endocardial fibroelastosis diagnosed by left ventricular endomyocardial biopsy showing a markedly thickened endocardium with fibroelastic proliferation is reported. He had no evident symptoms of congestive heart failure except for shortness of breath on moderate exertion. Echocardiogram showed thickened and dense echoes from the left side of the septum and from the posterior left ventricular endocardium. Hemodynamic and angiographic studies revealed marked elevation of right and left ventricular end-diastolic pressures with dip and plateau pressure contours, moderate pulmonary hypertension, left atrial enlargement and mild mitral regurgitation. Further elevation of right and left ventricular diastolic pressures and pulmonary artery pressure was observed at the second evaluation after 5 years. Our patient suggests that primary endocardial fibroelastosis should be included in the differential diagnosis of adult patients with obscure types of cardiac disease.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/8. endocardial fibroelastosis: myocardial and vascular alterations associated with viral-like nuclear particles.Although clinical, immunologic, and experimental evidence exists implicating in utero viral infection of the myocardium in the development of primary endocardial fibroelastosis, the infectious etiology of this condition remains somewhat controversial. To date, specific features of viral myocarditis and morphological demonstration of viral particles have not been described in EFE. The present case is the first in which extensive light microscopic and ultrastructural analysis of the myocardium revealed abnormalities consistent with a primary viral myocarditis associated with typical EFE. These alterations consisted of chronic myocardial inflammation, extensive interstitial fibrosis, severe degenerative changes in myocardial cells, and a marked proliferation of endothelial cells in large nad small intramyocardial vessels leading to vascular occlusions. In support of the infectious etiology of this disease, similar features were noted in skeletal muscle. Most interestingly, viral-like particles were observed in many myocardial and endothelial nuclei. Although we are not absolutely certain of the viral nature of these particles, their appearance suggests viral assoicated material. We propose that the presence of these particles in this case in association with the other morphological alterations in support for the viral etiology of EFE. The prominent vascular occlusion observed in the myocardium may be an important clue to the pathogenesis of fibroelastosis as either a primary or secondary disease.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/8. heart transplantation in an 8-month-old girl. 10th anniversary report.In 1984, Dr. Denton A. Cooley led a surgical team that implanted a cardiac allograft in an 8-month-old girl who had end-stage cardiac disease secondary to endocardial fibroelastosis. At that time, experience with cardiac transplantation in infants was limited, and the long-term effects of the procedure were cause for concern. Ten years later, our patient is a healthy 4th-grade student who enjoys a remarkably normal life. She has grown and developed quite satisfactorily, and her heart has enlarged in proportion to her overall somatic growth. Long-term immunosuppression has produced no adverse effects, and the child's medical problems have differed little from those of her peers. This landmark case has yielded preliminary answers to a number of important questions about cardiac transplantation in infants and has confirmed our original opinion that the procedure is well warranted in selected patients.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |