Cases reported "Cardiomyopathy, Dilated"

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1/8. Iterative atrial tachycardia originating from the coronary sinus musculature.

    A case of iterative atrial tachycardia leading to dilated cardiomyopathy is reported. During electrophysiologic study, the tachycardia showed a markedly irregular cycle length associated with changes in atrial activation breakthrough as demonstrated by coronary sinus (CS) recordings and frequently degenerated into self-terminating atrial fibrillation. Left atrial transseptal mapping demonstrated the earliest endocardial atrial activation close to the posterolateral mitral annulus, but this was invariably later than that recorded within the CS, where low-energy radiofrequency applications eliminated the tachycardia. No acute vessel damage was observed at postablation CS angiography. In accordance with previously published experimental data, we hypothesized that the muscular sleeves surrounding the CS might be involved in the genesis of this tachycardia. During 6-month follow-up, the patient remained asymptomatic without tachycardia recurrences and with complete recovery of left ventricular function, confirming the reversible nature of the tachycardia-induced cardiomyopathy.
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2/8. warfarin-induced skin necrosis associated with acquired protein c deficiency.

    A 36-year-old woman developed skin necrosis of the inner thighs following the re-introduction of warfarin after a laparoscopic cholecystectomy. She had a history of liver disease and cardiomyopathy and was on warfarin for 10 years. warfarin-induced skin necrosis secondary to protein c deficiency was diagnosed. Although warfarin was ceased immediately, the prothrombin time measurements remained prolonged and warfarin levels remained therapeutic. Our patient, who had attached great significance to warfarin therapy, had continued the ingestion of warfarin despite our advice. She required three surgical debridements. Protein C levels, as measured 1 year later, were within normal limits, confirming the transient nature of the acquired deficiency during the acute event. This is the second reported case of warfarin necrosis associated with acquired protein c deficiency.
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3/8. Changes in right ventricular dimensions and performance after passive cardiac containment.

    BACKGROUND: Previous studies have shown that the cardiac support device (CSD) improves left ventricular structure and function in patients with heart failure by preventing further cardiac enlargement. The aim of this study was to identify effects on the right ventricle (RV). methods: Ten male patients with idiopathic dilated cardiomyopathy underwent electron-beam computed tomographic (CT) examination within 1 month before, and 6 to 9 months after CSD implantation. The RV end-diastolic and end-systolic volumes (EDV, ESV) and diameters (EDD, ESD), stroke volume (SV), ejection fraction (EF), total and forward RV output (RVO, fRVO), and tricuspid regurgitation fraction (TRF) were calculated. RESULTS: The EDV measurements decreased from 182.1 /- 49.6 to 137.5 /- 37.0 mL, ESV from 114.8 /- 47.0 to 68.3 /- 23.8 mL, EDD from 48.2 /- 6.6 to 41.6 /- 7.1 mm, and ESD from 39.6 /- 6.9 to 32.7 /- 6.5 mm (p < 0.05 for each). Ejection fraction increased from 38.5 /- 8.9 to 52.0% /- 7.7% and fRVO from 4.0 /- 0.8 to 4.6 /- 1.1 L/min (each with p < 0.05). TRF decreased from 18.2 /- 14.1 to 10.4% /- 13.5%, whereas SV and RVO remained nearly unchanged. Postoperatively, RV volumes, EF, and fRVO were not different from 15 age- and gender-matched normal control patients. CONCLUSIONS: Implantation of a CSD leads to a decrease in RV size and improved RV performance. These data together with the results of previous studies demonstrating improved left ventricular structure and function confirm the biventricular nature of recovery with the CSD.
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4/8. Familial dilated cardiomyopathy.

    Idiopathic dilated cardiomyopathy is generally considered a sporadic, nongenetic disorder, and reports of familial cases are often regarded as rare occurrences. Results of the present investigation of 6 families with this disorder suggest that familial forms of dilated cardiomyopathy occur more frequently than previously suspected. The familial nature of the dilated cardiomyopathy was not readily apparent in 3 of these families until thorough family investigations had been performed. The clinical symptoms and age of onset were variable from one family to another and within families. Based on these observations, it is recommended that all persons diagnosed with dilated cardiomyopathy have a thorough review of their family history. If there are any cases of unexplained heart disease, sudden unexpected death or syncopal episodes, further investigations of relatives should be performed. echocardiography is a convenient noninvasive tool for investigating relatives. early diagnosis of affected relatives is important for 2 reasons--treatment of significant arrhythmias may prevent sudden unexpected death, and genetic counseling can be provided.
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5/8. Peripartum cardiomyopathy: two case reports and a review.

    Peripartum cardiomyopathy is a myocardial disease of a dilatative (congestive) nature which typically presents in the last month of pregnancy or within the first 5 postpartum months. In the first case described, heart failure was noted prior to delivery, and complete recovery was achieved with early and aggressive management. Cardiomyopathy developed postpartum in the second case, with a fatal outcome. early diagnosis and prompt treatment are of paramount importance. mortality is still high. Recent pathologic findings, methods of diagnosis and theories on etiology are discussed.
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6/8. Dominantly inherited dilated cardiomyopathy.

    We describe a family in which there is segregating an autosomal dominant gene determining a cardiomyopathy. The pathodynamics is that of pump failure associated with dilatation of the heart, generally having an overt clinical onset from the fourth through seventh decades. Dysrhythmia is a frequent concomitant feature. There may be an associated skeletal myopathy, either producing a very mild proximal weakness or proving detectable only upon biopsy. This family is similar to other reported cases of familial dominant "idiopathic" dilated cardiomyopathy, but the nature of the heterogeneity within this category remains to be elucidated. The roles of echocardiography, cardiac biopsy, and skeletal muscle biopsy in the presymptomatic detection of the heterozygote are noted.
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7/8. A case of right ventricular dilated cardiomyopathy.

    This paper presents a sudden death case of 16-year-old girl. She is assumed to have died of ventricular arrhythmia clinically. On autopsy, the right cardiac ventricle was markedly dilated due to extensive infiltration of fibroadipose tissue and resultant depletion of the myocytes, but there was no full thickness disappearance of the contractile element in any part. Considerable parts of remaining myocytes showed degenerative changes similar to those of dilated cardiomyopathy. The left ventricle was moderately dilated. It showed similar significant degenerative changes to the right ventricle, but the degree was less extensive than the right. These findings do not fill the diagnostic requirements of neither Uhl's anomaly nor parchment heart, which reveal almost total depletion of the right ventricular myocytes. However, the findings were consistent with arrhythmogenic right ventricular dysplasia, in case the concept of the entity is extended, we concluded that it would be better to adopt a term--cardiomyopathy--to this case rather than dysplasia. Because, there were considerable histological evidence of myocardial degeneration suggesting primary myocardial disease rather than congenital defect in nature. We diagnosed, therefore, this case as right ventricular dilated cardiomyopathy, comprising a type of dilated cardiomyopathy whose myocardial lesions predominate in the right compared to the left ventricle.
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8/8. Detection of enteroviral rna in end-stage dilated cardiomyopathy in children and adolescents.

    medical records and archival myocardial specimens of 33 children and adolescents with end-stage idiopathic dilated cardiomyopathy (IDCM) were collected to evaluate retrospectively the potential role of enteroviral persistence in the pathogenesis of IDCM. The clinical history and laboratory assessment of each patient were reviewed carefully in order to obtain information on the nature and etiology of infections in the past and at the time of diagnosis of cardiomyopathy. Sixty-four formaldehyde-fixed, paraffin-embedded myocardial specimens, obtained from endomyocardial biopsies (n = 5), explanted hearts (n = 10), or autopsies (n = 49), were studied by the polymerase chain reaction (PCR) and by in situ hybridization to detect enteroviral rna in the specimens. Control specimens included 34 formaldehyde-fixed, paraffin-embedded myocardial specimens from children with other cardiomyopathies, metabolic diseases, structural heart defects, or various noncardiac malignancies. The presence of cellular rna in the specimens was confirmed by amplification of glyceraldehyde-3-phosphate dehydrogenase (GAPDH) mRNA or beta-actin mRNA as positive controls. Only one specimen from the 32 IDCM patients with appropriate myocardial specimens was positive for enteroviral rna by PCR. sequence analysis of the amplified viral segment showed a significant degree of homology between the viral sequence and echovirus 1. One specimen from the control patients also appeared positive by PCR, but sequence analysis of the amplified viral segment revealed it as rhinovirus 16. The results do not indicate any significant role for enteroviral persistence in end-stage childhood IDCM, although they need to be confirmed using a prospective study with fresh frozen specimens. However, mechanisms other than viral persistence may be more important in the progression of IDCM to end-stage heart failure in this age group.
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