1/79. Long-term follow-up of a true contractile left ventricular diverticulum.The natural history of contractile left ventricular diverticulum in the adult is not known. Serial left ventricular angiography in an adult revealed that a left ventricular diverticulum did not increase in size over a 13-year period, suggesting that the clinical course may be benign.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/79. Sudden death in right ventricular dysplasia with minimal gross abnormalities.Arrhythmogenic right ventricular cardiomyopathy is emerging as a relatively common cause of exercise-induced sudden death in the young. The diagnostic criteria at autopsy are, however, not fully established, leading to both over- and underdiagnosis. We report a young man and a young woman dying suddenly of right ventricular dysplasia during exercise, in whom the gross autopsy findings in the right ventricle were minimal or even absent. However, the histologic features in both right and left ventricles were typical of the disease, and consisted of fibrofatty infiltrates with typical myocyte degeneration of the right ventricle and subepicardial regions of the left ventricle. These cases illustrate that microscopic findings are diagnostic and may be present in the absence of gross findings. Marked fat replacement is not essential for the diagnosis of right ventricular dysplasia, and the right ventricle should be extensively sampled histologically in all cases of sudden unexpected death, especially those that are exercise related.- - - - - - - - - - ranking = 0.55689465379775keywords = sample (Clic here for more details about this article) |
3/79. Unusual recurrence of hydatid cysts of the heart: report of two cases and review of the clinical and surgical aspects of the disease.In cardiac echinococcosis, a hydatid cyst most frequently forms either solely in the heart or in the pericardium, but there are several reports of cysts forming in the liver or lung or in both. In two cases reported here, both patients developed cysts in new sites after one or more previous surgeries for hydatid cyst removal. In Case 1, the patient first underwent spleenectomy and resection of multiple cysts with no evidence of a cyst in the heart; 3 years later, there was no sign of echinococcus in the liver, but a large inframyocardial cyst had damaged the left ventricle. In Case 2, the patient first underwent surgery to remove cysts from the pericardium, 2 years later from the anterior wall of the left ventricle, and, finally, 8 months after this second operation, from the left atrium also with no evidence of cyst formation anywhere else in the heart at the time of surgery. These cases emphasize the need for thorough and frequent reevaluation to detect new hydatid cyst formation in the heart and elsewhere caused by the echinococcus organism.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/79. Atrial blood cyst in an adult with regurgitation of mitral and tricuspid valves.blood cysts of the heart are extremely rare in adults and usually involve valves or the left ventricle. Although two cases of blood cysts in the right atrium in adults have been reported, a cyst combined with a disorder of the valves has never been reported. We report a 52-year-old woman with a blood cyst that generated from the right atrial septum. Furthermore, the patient had regurgitation of both the mitral and tricuspid valves and then underwent surgical excision of the blood cyst, mitral valve plasty and tricuspid valve annuloplasty. We believe that it is possible to diagnose blood cysts with echocardiography, CT and magnetic resonance imaging. echocardiography showed the cyst as a circle without a complete inner free-echo. CT and magnetic resonance imaging showed a mass with a non-enhanced inner structure. Furthermore, the latter showed a cyst that was enhanced by T1- but not T2-weighted images, indicating that the content of the cyst was a persistent substance such as blood. Concerning the generation of blood cysts, we hypothesize that heteroplastic growth arising from primitive pericardial mesothelium causes disorders of valves and blood cysts.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/79. Isolated noncompaction of ventricular myocardium.isolated noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The diagnosis can be made echocardiographically, and the entity may be associated with problems of cardiac rhythm. We describe two illustrative cases, with Wolf-Parkinson-White syndrome in one, and left bundle branch block in the other. We emphasize that children with problems of rhythm and findings suggestive for left ventricular cardiomyopathy, either hypertrophic or dilated; should be investigated to exclude isolated noncompaction of the ventricular myocardium.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/79. Ectopic cardiac calcification associated with hyperparathyroidism in a boy with hypophosphatemic rickets.An adolescent with hypophosphatemic rickets developed cardiac calcifications in the absence of hypercalcemia or elevation of the phosphocalcic product (the product of the total serum calcium and phosphorus concentrations). Cardiac calcifications led to aortic and mitral valve dysfunction, myocardial calcification, and arrhythmia. hyperparathyroidism probably played a significant role in the development of this complication, which emphasizes the necessity for intermittent assessment of parathyroid status in individuals receiving medical therapy for hypophosphatemic rickets.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/79. Novel heteroplasmic mtDNA mutation in a family with heterogeneous clinical presentations.The protean manifestations of a novel maternally inherited point mutation of the mitochondrial genome are reported. The proband showed isolated, spastic paraparesis. A brother, who had suffered from a multisystem progressive disorder, ultimately died of cardiomyopathy. Another brother is healthy. The proband's mother showed truncal ataxia, dysarthria, severe hearing loss, mental regression, ptosis, ophthalmoparesis, distal cyclones, and diabetes mellitus. A muscle biopsy performed in the proband failed to show the morphological abnormalities typical of mitochondrial disorders; the activities of respiratory chain complexes were normal. However, complex I and IV activities were low in the muscle homogenate of the affected mother and brother. sequence analysis of mtDNA showed a heteroplasmic mutation of the tRNA(Ile) gene (G4284A). The mutation load was approximately 55%, 80%, and 90% in the muscle mtDNA of the proband, his mother, and his affected brother, respectively. Mutation was undetected in the healthy brother, as well as in 100 control samples. Several cybrid clones containing homoplasmic mutant mtDNA from the proband showed significant reductions of complex IV activity and maximum oxygen consumption rate, compared with homoplasmic wild-type clones derived from the same subject.- - - - - - - - - - ranking = 0.55689465379775keywords = sample (Clic here for more details about this article) |
8/79. Cardiomyopathy in association with selenium deficiency: a case report.A 46-year-old man developed "dilated cardiomyopathy" probably caused by selenium deficiency while on total parenteral nutrition (TPN). This development emphasizes the role of considering selenium deficiency as a reversible cause of unexplained cardiomyopathy in impaired nutritional state.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/79. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.The current dietary treatment of long-chain fatty acid oxidation defects (high carbohydrate with medium-even-chain triglycerides and reduced amounts of long-chain fats) fails, in many cases, to prevent cardiomyopathy, rhabdomyolysis, and muscle weakness. We hypothesized that the apparent defect in energy production results from a depletion of the catalytic intermediates of the citric acid cycle via leakage through cell membranes (cataplerosis). We further hypothesized that replacing dietary medium-even-chain fatty acids (precursors of acetyl-CoA) by medium-odd-chain fatty acids (precursors of acetyl-CoA and anaplerotic propionyl-CoA) would restore energy production and improve cardiac and skeletal muscle function. We fed subjects with long-chain defects a controlled diet in which the fat component was switched from medium-even-chain triglycerides to triheptanoin. In three patients with very-long-chain acyl-coa dehydrogenase deficiency, this treatment led rapidly to clinical improvement that included the permanent disappearance of chronic cardiomyopathy, rhabdomyolysis, and muscle weakness (for more than 2 years in one child), and of rhabdomyolysis and weakness in the others. There was no evidence of propionyl overload in these patients. The treatment has been well tolerated for up to 26 months and opens new avenues for the management of patients with mitochondrial fat oxidation disorders.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
10/79. Systemic lupus erythematosus presented as non-inflammatory necrotizing vasculopathy-induced ischemic glomerulopathy and small vessels-related ischemic cardiomyopathy.The clinical significance of lupus non-inflammatory necrotizing vasculopathy (NINV) is not well established. For example, since lupus renal NINV is usually reported to coexist with proliferative and active glomerulonephritis, it is difficult to demonstrate the role of NINV on renal pathophysiology. Here we report a 16-year-old SLE boy with renal NINV presenting as ischemic glomerulopathy and small vessels-related ischemic heart failure. The renal biopsy demonstrated mild proliferative glomerulonephritis and NINV initially, and one month later repeated renal biopsy showed NINV with ischemic glomerulopathy. These findings established that NINV, but not proliferative glomerulonephritis, was responsive for his acute renal failure (ARF). Another interesting question is about the pathophysiology of his myocardial dysfunction. This patient presented typical angina and congestive heart failure (CHF). Echocardiograms and ventriculography revealed dilatation of four chambers and low ejection fraction. Serial electrocardiograms demonstrated evolutionary ischemic changes. coronary angiography revealed no abnormality of large vessels. These findings suggested small vascular lesions-induced myocardial ischemia was the underlying mechanism of dilated cardiomyopathy. As myocardial biopsy was not done in our case, we could only speculate, but not prove, that the NINV observed in renal biopsy may also involve in cardiac microvascular beds. Nevertheless, this interesting case emphasized the role of obliterative small vascular lesions in the pathophysiology of ARF and myocardial dysfunction. The patient was treated with high-dose corticosteroid, plasma infusion and hemodialysis. His cardiac function improved gradually, however the renal function did not recover.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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