1/26. Fetal diagnosis of lethal dysfunction of the right heart in three siblings.A woman, having already delivered one child, underwent fetal echocardiography during three subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/26. Case report and review of the perinatal implications of maternal lithium use.The purpose of this study was to review the use of lithium in pregnancy and its effects on the neonate. This was a case study and review of the published literature.lithium is commonly used in the treatment of psychiatric disorders, specifically bipolar depression. Bipolar disorders that require treatment with lithium demand special consideration when the woman becomes pregnant. Reported neonatal problems with maternal lithium therapy include Ebstein's anomaly, poor respiratory effort and cyanosis, rhythm disturbances, nephrogenic diabetes insipidus, thyroid dysfunction, hypoglycemia, hypotonia and lethargy, hyperbilirubinemia, and large-for-gestational-age infants.lithium can have adverse effects on the fetus and newborn infant, but data suggest normal behavioral patterns in childhood.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
3/26. Severe heart failure from light chain cardiomyopathy (cardiac amyloidosis).Primary (AL)amyloidosis is characterized by clonal production of immunoglobulin with subsequent deposition in several organs. We describe the clinical features of a 66-year old female who was referred to our department for congestive heart failure. One year before, she was examined and found to have diastolic dysfunction of the left ventricle. We could evaluate the diagnosis of primary amyloid cardiomyopathy by echocardiography, doppler echocardiography and laboratory findings and confirmed the diagnosis by biopsy of the rectum mucosa. Clinical outcome was poor, because therapy is poor.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
4/26. congo red-positive cardiac kappa-AL amyloidosis in plasmacytoma -- case report and review of the literature.We report on a 51-year-old female patient who was diagnosed in 2001 as suffering from plasmacytoma. The patient had had complaints of bilateral carpal tunnel syndrome in 1999, treated by a simple dissection without performing histological examination. Congestive heart failure had gradually developed since that time. In 2001 echocardiography revealed a pronounced thickening of the left ventricular wall with systolic and diastolic dysfunction. A kappa-light chain M component and kappa-light chain-restricted bone marrow plasmacytosis were detected. Amyloid deposits staining positive in the kappa-light chain-restriction analysis were observed in a gastric biopsy. Taking into consideration all these findings, a plasmacytoma-associated systemic AL amyloidosis was diagnosed. cyclophosphamide/prednisolone chemotherapy regimen led to complete haematological remission. Cardiac transplantation, combined with autologous peripheral blood stem cell graft, was considered as the next therapeutic step, but the patient died while on the waiting list for transplantation. autopsy detected a highly hypertrophic myocardium with narrowed heart cavities. Microscopic examination revealed dense, pink, acellular, congo red-staining and kappa-immunoperoxidase-positive AL amyloid masses splitting the cardiomyocytes. The present case is remarkable as it demonstrates that carpal tunnel syndrome and congestive heart failure could be symptoms of plasma cell dyscrasia-associated amyloidoses.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
5/26. A rare case of alstrom syndrome presenting with rapidly progressive severe dilated cardiomyopathy diagnosed by echocardiography.alstrom syndrome is an extremely rare, autosomal recessive genetic disorder characterized by a group of signs and symptoms including infantile onset dilated cardiomyopathy, blindness, hearing impairment/loss, and obesity; with diabetes, and hepatic and renal dysfunction later on in life. Since the first description of the syndrome in 1959, there have been fewer than 100 reported cases in the world. We report a case of a 7-month-old girl whose initial presentation of alstrom syndrome was a rapidly progressive severe dilated cardiomyopathy, diagnosed by echocardiography. Unique characteristics of the cardiomyopathy in alstrom syndrome and the important role of echocardiography in alstrom syndrome and other genetically transmitted disorders, especially with known cardiovascular manifestations, is discussed.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
6/26. Cardiac morbidity in advanced chronic myelogenous leukaemia patients treated by successive allogeneic stem cell transplantation with busulphan/cyclophosphamide conditioning after imatinib mesylate administration.Imatinib mesylate is useful for facilitating allogeneic stem cell transplantation (allo-SCT) in advanced-phase chronic myelogenous leukaemia (AP-CML) patients. However, although the side-effects of imatinib are usually minor, cardiac morbidity can develop as a latent adverse effect post SCT when a myeloablative SCT is given to patients taking imatinib. Two AP-CML patients who were treated with imatinib manifested severe cardiac dysfunction after an allo-SCT, whereas cardiac morbidity was not observed in 45 other patients who had not received imatinib. It would appear that exposure to imatinib may have an adverse impact on the heart in AP-CML patients who receive an allo-SCT conditioned with busulphan/cyclophosphamide.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
7/26. Severe alcoholic cardiomyopathy reversed with abstention from alcohol.In a chronic alcoholic with a severe congestive cardiomyopathy the angiographic and hemodynamic evidence of left ventricular dysfunction was completely reversed after 1 year of abstinence. At the time of initial presentation two of the traditional hallmarks of a poor prognosis were present, namely, a long exposure to alcohol before the onset of symptoms and severe diffuse left ventricular dilation and hypokinesis (ejection fraction 14.9 percent). Yet, today, 18 months later he is asymptomatic, receiving no medications and has normal left ventricular function. A reexamination of prognostic factors in alcoholic cardiomyopathy may be indicated.- - - - - - - - - - ranking = 100.10759009089keywords = ventricular dysfunction, left ventricular dysfunction, dysfunction (Clic here for more details about this article) |
8/26. Structural and molecular pathology of the heart in Carvajal syndrome.BACKGROUND: Carvajal syndrome is a familial cardiocutaneous syndrome consisting of woolly hair, palmoplantar keratoderma, and heart disease. It is caused by a recessive deletion mutation in desmoplakin, an intracellular protein that links desmosomal adhesion molecules to intermediate filaments of the cytoskeleton. The pathology of Carvajal syndrome has not been described. methods: Here, we report the first description of the structural and molecular pathology of the heart in Carvajal syndrome. We characterized gross and microscopic pathology and identified changes in expression and distribution of intercalated disk and intermediate filament proteins in ventricular myocardium. RESULTS: We identified a unique cardiomyopathy characterized by ventricular hypertrophy and dilatation, focal ventricular aneurysms, and distinct ultrastructural abnormalities of intercalated disks, but no evidence of fibrofatty infiltration or replacement of myocardium. We also observed markedly decreased amounts of specific immunoreactive signal for desmoplakin, plakoglobin, and the gap junction protein, connexin43, at intercalated disks. The intermediate filament protein, desmin, which is known to bind desmoplakin, showed a normal intracellular pattern of distribution but failed to localize at intercalated disks. CONCLUSIONS: The desmoplakin mutation in Carvajal syndrome produces a cardiomyopathy with unique pathologic features. Altered protein-protein interactions at intercalated disks likely cause both contractile and electrical dysfunction in Carvajal syndrome.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
9/26. Improvement of cheyne-stokes respiration, central sleep apnea and congestive heart failure by noninvasive bilevel positive pressure and medical treatment.A 57-year-old man was admitted with dyspnea. Clinical evaluation revealed atrial fibrillation and congestive heart failure (CHF). Standard medical therapy of CHF failed to completely improve the dyspnea and polysomnography revealed cheyne-stokes respiration with central sleep apnea (CSR-CSA). He was equipped with noninvasive positive pressure ventilation (NPPV) with bilevel positive airway pressure (BiPAP). The combined therapy of medical treatment of the CHF and administration of NPPV with BiPAP reduced the CSR-CSA. This regimen resulted in marked improvement of cardiac function, evaluated by echocardiography, and reduction of plasma concentration of brain natriuretic peptide. After the patient recovered from CHF and was discharged from hospital, he continued to use NPPV with BiPAP at home. In patients with CHF, it is important to be aware of sleep-related breathing disorders because treatment will not only improve the hypoxemia, but also the cardiac dysfunction.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
10/26. Disproportionate hypertrophy of the interventricular septum and its regression in Cushing's syndrome. Report of three cases.Three patients of Cushing's syndrome with severe disproportionate hypertrophy of the interventricular septum are reported. All three underwent adrenalectomy and the diagnosis of Cushing's syndrome was confirmed by the presence of adrenal adenoma. All three showed hypertension before the adrenalectomy and two remained hypertensive following adrenalectomy. Before the operations the electrocardiograms revealed inverted T waves in broad leads, and mechanocardiogram systolic and diastolic dysfunction. After the adrenalectomies, abnormal findings on electrocardiograms were normalized and septal hypertrophy was completely regressed. It appears that not only high aortic pressure but also excessive plasma cortisol may be an etiologic factor of the left ventricular hypertrophy in Cushing's syndrome.- - - - - - - - - - ranking = 0.25keywords = dysfunction (Clic here for more details about this article) |
| | Next -> |