Cases reported "Cardiomyopathies"

Filter by keywords:



Filtering documents. Please wait...

1/672. Hereditary dysrhythmic congestive cardiomyopathy.

    A patient with hereditary congestive cardiomyopathy, who presented with recurrent episodes of life-threatening ventricular arrhythmias most often precipitated by exercise, is described. The condition is marked by either a progressive course, in which case congestive cardiac failure may set in towards the end, or by unexpected sudden death. The family tree could be traced for 10 generations. The information about the tenth generation firmly established that 4 members, 2 of whom had died, were affected. Other evidence suggests that the condition was the cause of death in 3 members of the eighth generation.
- - - - - - - - - -
ranking = 1
keywords = cardiomyopathy
(Clic here for more details about this article)

2/672. Thrombolysis with resolution of pulmonary hypertension in a heart transplant candidate.

    We report a patient with idiopathic cardiomyopathy and high pulmonary resistance due to pulmonary emboli of unknown age. Successful thrombolytic therapy returned his pulmonary resistance to normal, allowing orthotopic cardiac transplantation. This case underscores the need to aggressively diagnose and treat pulmonary emboli in potential transplant candidates.
- - - - - - - - - -
ranking = 0.2
keywords = cardiomyopathy
(Clic here for more details about this article)

3/672. 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 = 33.446347264035
keywords = ventricular dysplasia, right ventricular dysplasia, right ventricular cardiomyopathy, ventricular cardiomyopathy, cardiomyopathy, dysplasia
(Clic here for more details about this article)

4/672. Progression of cardiomyopathy and neuropathy after liver transplantation in a patient with familial amyloidotic polyneuropathy caused by tyrosine-77 transthyretin variant.

    Familial amyloidotic polyneuropathy is an inherited form of amyloidosis associated with a mutant form of a protein called transthyretin. The methionine-30 variant is the most frequent mutation observed. This disorder is caused by deposition of this protein as amyloid in several organs, such as the heart, kidneys, and peripheral nervous system. The disease is always progressive and fatal, and patients die 7 to 10 years after the onset of symptoms. liver transplantation is at present the only choice for these patients because it provides improvement of symptoms and/or stops progression of the disease in most patients. We report the case of a patient who showed clear progression of cardiomyopathy and neuropathy after liver transplantation.
- - - - - - - - - -
ranking = 1
keywords = cardiomyopathy
(Clic here for more details about this article)

5/672. Very long chain acyl coenzyme a dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation.

    A 5-month-old Korean boy who presented with lethargy and cardiomyopathy was diagnosed with very long chain acyl coenzyme a dehydrogenase (VLCAD) deficiency by organic acid, fatty acid, acylcarnitine, and molecular genetic analysis. The patient was a compound heterozygote for mutations in the VLCAD gene. One allele contains a 3-bp deletion in exon 6, deleting glutamic acid in codon 130 (E130del ); this allele is of paternal origin. The patient's maternally derived allele is a novel mutation, C1843T in exon 20, which creates a premature termination codon (R615stop ). Although molecular genetic characterization of VLCAD deficiency is limited to a few patients, heterogeneity of mutations is already apparent. However, the E130del is a relatively frequent mutant allele, which has been noted in 2 previously identified patients. The 2 mutant alleles in our patient appear to be responsible for his severe and fatal clinical manifestations.
- - - - - - - - - -
ranking = 0.2
keywords = cardiomyopathy
(Clic here for more details about this article)

6/672. Severe hypertensive sequelae in a child with Seckel syndrome (bird-like dwarfism).

    We report a 19-year-old male with Seckel syndrome (bird-like dwarfism) who presents with malignant hypertension associated with hypertensive nephrosclerosis, dilated cardiomyopathy, and a ruptured cerebral artery aneurysm. Although end-organ injury due to chronic hypertension occurs frequently in adults, no previous reports of renal insufficiency due to hypertension exist in children or adolescents. We speculate that this patient may have been particularly prone to hypertensive end-organ injury due to his extreme short stature.
- - - - - - - - - -
ranking = 0.2
keywords = cardiomyopathy
(Clic here for more details about this article)

7/672. Deletion of chromosome 18 with cardiomyopathy.

    A female child is described with deletion of chromosome 18 and cardiomyopathy. The clinical features and treatment of the case are described, and the literature of chromosome 18 reviewed.
- - - - - - - - - -
ranking = 1
keywords = cardiomyopathy
(Clic here for more details about this article)

8/672. biopsy-proven cardiomyopathy in heterozygous Fabry's disease.

    A 23-year-old woman with heterozygous Fabry's disease who had acroparesthesia was admitted to hospital for precise examination of the disease before childbearing. She had no cardiac-related symptoms and no abnormality on physical examination. The alpha-galactosidase A activity in her leukocytes was present, but lower than normal. However, the endomyocardial biopsy showed specific changes for Fabry's disease. As Fabry's disease is a rare X-linked recessive inborn error of glycosphingolipid metabolism, heterozygous females are usually asymptomatic, but rarely can be affected as severely as hemizygous males. This is an isolated case of heterozygous Fabry's disease in a female in whom cardiac involvement was detected by endomyocardial biopsy, although she had no cardiac abnormality on physiological examinations. In conclusion, endomyocardial biopsy is useful for evaluation of the cardiac involvement of Fabry's disease even in an asymptomatic case.
- - - - - - - - - -
ranking = 0.8
keywords = cardiomyopathy
(Clic here for more details about this article)

9/672. Histiocytoid cardiomyopathy: three new cases and a review of the literature.

    Histiocytoid cardiomyopathy (HC), a rare arrhythmogenic disorder, presents as difficult-to-control arrhythmias or sudden death in infants and children, particularly girls. Three cases are described with autopsy findings. In two cases, yellow-tan nodules were grossly visible in the myocardium; in the third case, no gross lesions were identified. Microscopic examination in all three cases revealed multiple, scattered clusters of histiocytoid myocytes which on ultrastructural examination were filled with abnormal mitochondria, scattered lipid droplets, and scanty myofibrils. These pathologic findings are similar to those previously described. The pathogenesis of this entity remains controversial. It was recently proposed that this disorder is X-linked dominant with the associated gene located in the region of Xp22.
- - - - - - - - - -
ranking = 1.740813340591
keywords = arrhythmogenic, cardiomyopathy
(Clic here for more details about this article)

10/672. Intraoperative use of automated external defibrillator.

    We report on the anesthetic management of a patient with peripartum cardiomyopathy and frequent episodes of ventricular tachycardia, who underwent surgery for tracheal stenosis. Prior to this surgery, the patient had been implanted with an automatic implantable cardioverter-defibrillator (AICD), placed abdominally. In the operating room, the AICD was deactivated, and an automated external defibrillator (AED) was placed. Intraoperatively, the AED identified and treated the patient's ventricular tachycardia. Advantages of the AED in this hospital setting included rapid response to the cardiomyopathy, safe, hands-free operation, and minimal disruption of the surgical procedure. safety concerns when using the AED are also detailed.
- - - - - - - - - -
ranking = 0.4
keywords = cardiomyopathy
(Clic here for more details about this article)
| Next ->


Leave a message about 'Cardiomyopathies'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.