Cases reported "Fibrosis"

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1/39. Right ventricular cardiomyopathy. A rare and underdiagnosed cause of sudden death in young adults.

    We present an example of right ventricular cardiomyopathy, a very uncommon condition which caused sudden death in an adolescent boy. This case report is used to highlight the pathological features of this rare and poorly understood condition, which is often undiagnosed.
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ranking = 1
keywords = right ventricular cardiomyopathy, ventricular cardiomyopathy, cardiomyopathy
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2/39. First brain dead donor heart transplantation under new legislation in japan.

    The first heart transplantation was carried out in japan successfully, after the brain death and organ transplantation law was settled in 1997. The recipient patient was a 47-year-old man with the dilated phase of hypertrophic cardiomyopathy who had been on a Novacor implantable left ventricular assist system for the previous 4 months. Since the donor hospital was about 200 km from the recipient hospital which took approximately 2 hours for transportation, the total ischemic time was 3 hours and 24 minutes. The post-transplant course was smooth, and the patient was discharged on postoperative day 75.
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ranking = 0.0031844482275009
keywords = cardiomyopathy
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3/39. endomyocardial fibrosis (Davies disease) coincidental with systemic lupus erythematosus.

    This is the case of a 27 years-old woman with signs and symptoms of severe untreatable congestive heart failure, anemia, gingival mucosa ulcers, photosensitivity and alopecia. The electrocardiographic, echocardiographic, angiographic and hemodynamic data oriented the diagnosis of restrictive cardiomyopathy, mitral insufficiency secondary to mitral prolapse and bi-atrial dilation. The histologic study of the endomyocardial biopsy, performed during catheterization, showed signs of endomyocardial fibrosis, and immunological analysis was compatible with systemic lupus erythematosus. As far as we know, this is the first case of endomyocardial fibrosis (Davies disease) associated with systemic lupus erythematosus published in the medical literature. The etiology of Davies disease remains unrevealed and its association with systemic lupus erythematosus suggest a probable autoimmune origin.
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ranking = 0.0031844482275009
keywords = cardiomyopathy
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4/39. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.

    Absence of the pulmonary valve occurs usually in association with tetralogy of fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.
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ranking = 0.00081843243015401
keywords = dysplasia
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5/39. Cardiac death after autologous stem cell transplantation (ASCT) for treatment of systemic sclerosis (SSc): no evidence for cyclophosphamide-induced cardiomyopathy.

    In patients with systemic sclerosis (SSc) treatment-related mortality after autologous stem cell transplantation (ASCT) appears to be increased as compared to patients with hematological malignancies. In our phase I/II study on ASCT in autoimmune diseases a patient with SSc died on day 2 after ASCT. Here we report the results of the autopsy which revealed advanced pulmonary and cardiac fibrosis as the most probable cause of death. In spite of detailed technical examination before enrollment, the cardiopulmonary function tests did not reflect the advanced stage of the disease. We conclude that in selected patients with SSc, biopsies should be performed to reduce mortality after ASCT.
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ranking = 0.012737792910004
keywords = cardiomyopathy
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6/39. Congenital fibrosis of the extraocular muscles associated with cortical dysplasia and maldevelopment of the basal ganglia.

    BACKGROUND: Congenital fibrosis of the extraocular muscles (CFEOM) is a rare condition that has been traditionally regarded as a primary eye muscle disease. Recent studies, however, suggest that CFEOM may be the result of a primary neuropathy with secondary myopathic changes. PURPOSE: To describe a previously unrecognized association between congenital fibrosis of the extraocular muscles and structural abnormalities of the brain. DESIGN: Small case series. methods: Detailed clinical examinations and neuroradiologic studies were performed on the three affected family members. In addition, genetic analysis of the family was performed. RESULTS: The three affected family members, mother and two children, have the ocular features of 'classic' congenital fibrosis of the extraocular muscles. All showed dilation of the left lateral ventricle secondary to hypoplasia of the body and tail of the ipsilateral caudate nucleus. There was fusion of an enlarged caudate nucleus head with the underlying putamen. Both children showed widespread bilateral cortical dysplasia. Genetic analysis of the family was inconclusive but consistent with linkage to the CFEOM1 locus on chromosome 12. Chromosomal analysis of the affected individuals did not show evidence of a deletion of chromosome 12 and haplotype analysis was not suggestive of a microdeletion. CONCLUSIONS: Cerebral cortical and basal ganglia maldevelopment can be found in individuals with CFEOM. This suggests that neuroimaging should be considered in the initial diagnostic evaluation of these patients, particularly if there is developmental delay.
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ranking = 0.0040921621507701
keywords = dysplasia
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7/39. The spectrum of intramyocardial small vessel disease associated with sudden death.

    Intramyocardial small vessel abnormalities are not commonly recognized. The best known abnormality is fibromuscular dysplasia involving the sinoatrial or atrioventricular nodal arteries. Small vessel disease has been reported as an isolated cardiac anomaly in individuals with sudden death, and may also be associated with other cardiac conditions including hypertrophic cardiomyopathy and mitral valve prolapse. The nature of the association is unknown, and the mechanism causing sudden death is sometimes obscure. We describe pathological changes of the intramyocardial small vessels of three individuals with sudden death. Abnormalities involved small vessels at different levels. In all the cases, the abnormalities were thought to have caused or contributed to the individual's death. The possible mechanisms of this are discussed.
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ranking = 0.0040028806576549
keywords = cardiomyopathy, dysplasia
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8/39. Segmental odontomaxillary dysplasia: clinical, radiological and histological aspects of four cases.

    Segmental odontomaxillary dysplasia (SOD) is a rare developmental disorder of the maxilla, primarily involving the posterior part of the maxilla. Clinically, the disorder is often diagnosed in early childhood due to a unilateral buccolingual expansion of the posterior alveolar process, gingival enlargement, absence of one or both premolars in the affected region, delayed eruption of the adjacent teeth and malformations of the primary molars. In this report, four patients with SOD are described. The findings were similar to earlier reports, but for the first time an ipsilateral rough erythema on the skin in two of the subjects is reported.
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ranking = 0.0040921621507701
keywords = dysplasia
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9/39. A case of hypereosinophilic syndrome presenting mid-ventricular obstruction.

    A 59-year-old man with hypereosinophilic syndrome (HES) who had been maintained with low-dose prednisolone for 5 years developed the characteristic features of hypertrophic cardiomyopathy. Left ventricular endomyocardial biopsy revealed no eosinophilic infiltration but extensive myocardial fibrosis. Cardiac involvement in HES presents as endocardial fibrosis, resulting in a clinical presentation of restrictive cardiomyopathy. HES heart disease can also present dilated cardiomyopathy, but myocardial hypertrophy has only rarely been noted in conjunction with HES. This report concerns a patient with HES who had clinical and hemodynamic evidence of asymmetric septal hypertrophy with mid-ventricular obstruction.
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ranking = 0.0095533446825028
keywords = cardiomyopathy
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10/39. Sudden death of a case of hypertrophic obstructive cardiomyopathy 19 months after successful percutaneous transluminal septal myocardial ablation.

    A 56-year-old male with hypertrophic obstructive cardiomyopathy complicated with medically refractory paroxysmal atrial fibrillation and congestive heart failure was treated with percutaneous transluminal septal myocardial ablation. The resting left ventricular outflow tract gradient decreased from 70 mmHg to 0 mmHg after the procedure, and clinical symptoms improved dramatically. However, the patient died suddenly 19 months later and autopsy revealed nontransmural myocardial fibrosis with an irregular border in the interventricular septum.
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ranking = 0.015922241137505
keywords = cardiomyopathy
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