Abnormal extracellular matrix protein transport associated with increased apoptosis of vascular smooth muscle cells in Marfan syndrome and bicuspid aortic valve thoracic aortic aneurysm

Background - Marfan syndrome (MS) is a genetic disorder caused by a mutation in the fibrillin gene FBN1. Bicuspid aortic valve (BAV) is a congenital heart malformation of unknown cause. Both conditions are associated with ascending aortic aneurysm and premature death. This study examined the relationship among the secretion of extracellular matrix proteins fibrillin, fibronectin, tenascin, and vascular smooth muscle cell (VSMC) apoptosis. The role of matrix metalloproteinase (MMP)- 2 in VSMC apoptosis was studied in MS aneurysm. Methods and Results - Aneurysm tissue was obtained from patients undergoing surgery ( MS: 4 M, 1 F, age 27 - 45 years; BAV: 3 M, 2 F, age 28 - 65 years). Normal aorta from subjects with nonaneurysm disease was also collected ( 4 M, 1 F, age 23 - 93 years). MS and BAV aneurysm histology showed areas of cystic medial necrosis (CMN) without inflammatory infiltrate. Immunohistochemical study of cultured MS and BAV VSMC showed intracellular accumulation and reduction of extracellular distribution of fibrillin, fibronectin, and tenascin. Western blot showed no increase in expression of fibrillin, fibronectin, or tenascin in MS or BAV VSMC and increased expression of MMP-2 in MS VSMCs. There was 4-fold increase in loss of cultured VSMC incubated in serum-free medium for 24 hours in both MS ( 27 +/- 8%) and BAV ( 32 +/- 14%) compared with control ( 7 +/- 5%). Conclusions - In MS and BAV there is alteration in both the amount and quality of secreted proteins and an increased degree of VSMC apoptosis. Up-regulation of MMP-2 might play a role in VSMC apoptosis in MS VSMC. The findings suggest the presence of a fundamental cellular abnormality in BAV thoracic aorta, possibly of genetic origin.

Cost of diseases in Brazil: breast cancer, enteritis, cardiac valve disease and bronchopneumonia

The results from the need to develop methodologies for performing cost analysis in developing countries, principally in the region of Latin America, were studied. It, furthermore, serves to generate knowledge from an economic evaluation in order to support decision-making related to the organization of health systems, particularly in the efficient use of resources which are allocated for the provision of medical services. Two chronic diseases (breast cancer and cardiac valve disease) and two infections (enteritis and bronchopneumonia) were selected for the study. The results recommend the use of a valid methodology for economic cost analysis of any disease to be studied and the use of this information in the decision-making process.

Perfil ecocardiográfico do doente com estenose aórtica degenerativa

Mestrado em Tecnologia de Diagnóstico e Intervenção Cardiovascular - Área de especialização: Ultrassonografia Cardiovascular.

Sensing Cloud Optimization to Solve ED of Units with Valve-Point Effects and Multi-fuels

In this paper a solution to an highly constrained and non-convex economical dispatch (ED) problem with a meta-heuristic technique named Sensing Cloud Optimization (SCO) is presented. The proposed meta-heuristic is based on a cloud of particles whose central point represents the objective function value and the remaining particles act as sensors "to fill" the search space and "guide" the central particle so it moves into the best direction. To demonstrate its performance, a case study with multi-fuel units and valve- point effects is presented.

Mycoplasma pneumoniae and Chlamydia pneumoniae in calcified nodules of aortic stenotic valves

Aortic Valve Stenosis (AVS) has been explained as an atherosclerotic process of the valve as they often exhibit inflammatory changes with infiltration of macrophages, T lymphocytes and lipid infiltration. The present study investigated whether the bacteria Chlamydia pneumoniae (CP) and Mycoplasma pneumoniae (MP), detected previously in atherosclerotic plaques, are also present in AVS. Ten valves surgically removed from patients with AVS were analyzed by immunohistochemistry, in situ hybridization, and electron microscopy. The mean and standard deviation of the percentage areas occupied by CP antigens and MP - DNA were respectively 6.21 +/- 5.41 and 2.27 +/- 2.06 in calcified foci; 2.8 +/- 3.33 and 1.78+/- 3.63 in surrounding fibrotic areas, and 0.21 +/- 0.17 and 0.12 +/- 0.13 in less injured parts of the valve. There was higher amount of CP and MP in the calcified foci and in the surrounded fibrosis than in more preserved valvular regions. In conclusion, the fact that there were greater amounts of CP and MP in calcification foci of AVS favors the hypothesis that AS is not an inevitable degenerative process due to aging, but rather that it may be a response to the presence of these bacteria, similarly to the morphology detected in atherosclerosis damage.

Isolated Cleft of the Anterior Mitral Valve Leaflet

Isolated anterior mitral leaflet cleft (not associated with atrio-ventricular septal defect) is a rare cause of congenital mitral regurgitation, and the treatment consists of direct suturing of the cleft. We present a clinical case with this entity.

A Papillary Fibroelastoma of the Tricuspid Valve

Primary cardiac tumours are rare in children. Of these, papillary fibroelastomas are unusual but benign, usually being found in adults. There are only sporadic cases reported in children. We diagnosed such a papillary fibroelastoma involving the tricuspid valve in an asymptomatic child during a routine cardiac investigation.

Mitral Valve Myxomas: an Unusual Entity

Primary tumours of the heart are uncommon entities, cardiac myxomas being the most frequent. However, mitral valve myxomas are exceptionally rare. In the last 12 years, there have been 25 myxomas diagnosed at our institution, with only two of them originating from the mitral valve. Both patients were female, the first, 25, and the second, 72 years old. The younger patient was very symptomatic with a large mass, 4 cm long, which involved both leaflets causing significant obstruction to the left ventricular inflow. The second one had a smaller mass located at the atrial side of the posterior leaflet that only produced some flow divergence. Neither of them had constitutional nor embolic symptoms. Both patients were submitted to emergent surgical resection that in the first case involved the mitral valve and replacement with mechanical prosthesis. The macroscopic appearance of these tumours suggested a malignant aetiology which may represent somewhat different features of the myxomas when originating from the cardiac valves. Both patients are well reflecting the good prognosis of this illness after resection, although the younger patient was re-operated because of prosthetic valve obstruction and suspicion of recurrence that was not confirmed. Because of the illustrative images and different presentations, we found it interesting to report and discuss them together.

Prosthetic Mitral Valve Thrombosis in Pregnancy: From Thrombolysis to Anticoagulation

INTRODUCTION: Pregnant women with mechanical prosthetic heart valves are at increased risk for valve thrombosis. Management decisions for this life-threatening complication are complex. Open-heart surgery has a very high risk of maternal mortality and fetal loss. Bleeding and embolic risks associated with thrombolytic agents, the limited efficacy of thrombolysis in certain subgroups, and a lack of experience in the setting of pregnancy raise important concerns. CASE REPORT: We report a case of mitral prosthetic valve thrombosis in early pregnancy, which was successfully treated with streptokinase. Ten years later, the same patient had an uneventful pregnancy, throughout which acenocoumarol was maintained. CONCLUSION: With this case we review the prevention (with oral anticoagulant therapy) and treatment of prosthetic valve thrombosis during pregnancy, which is important for both obstetrician and cardiologist.

Percutaneous Treatment of Mitral Valve Regurgitation: Initial Experience with the MitraClip Device

INTRODUCTION: Mitral regurgitation (MR) is the most common valvular disease and has recently become the target of a number of percutaneous approaches. The MitraClip is virtually the only device for which there is considerable experience, with more than 20,000 procedures performed worldwide. OBJECTIVE: To describe our initial experience of the percutaneous treatment of MR with the MitraClip device. METHODS: We describe the first six MitraClip cases performed in this institution (mean age 58.5 ± 13.1 years), with functional MR grade 4+ and New York Heart Association (NYHA) heart failure class III or IV (n=3), with a mean follow-up of 290 ± 145 days. RESULTS: Procedural success (MR ≤ 2+) was 100%. Total procedure time was 115.8 ± 23.7 min, with no in-hospital adverse events and discharge between the fourth and eighth day, and consistent improvement in the six-minute walk test (329.8 ± 98.42 vs. 385.33 ± 106.95 m) and in NYHA class (three patients improved by two NYHA classes). During follow-up there were two deaths, in two of the four patients who had been initially considered for heart transplantation. CONCLUSION: In patients with functional MR the MitraClip procedure is safe, with both a high implantation and immediate in-hospital success rate. A longer follow-up suggests that the clinical benefit decreases or disappears completely in patients with more advanced heart disease, namely those denied transplantation or on the heart transplant waiting list.

Dura mater mitral and tricuspid bioprostheses: 30 years of follow-up

PURPOSE: The dura mater bioprosthesis was developed in the Department of Cardiopneumology of the Hospital das Clínicas of the University of São Paulo Medical School in 1971. Here, we present the clinical results of the dura mater bioprosthesis over 30 years of follow-up. METHODS: We studied 70 consecutive patients who underwent mitral or tricuspid valve replacement with a dura mater bioprosthesis between January 1971 and August 1972. RESULTS: The early mortality was 10% (7 patients). The follow-up was 87% complete (9 patients were lost to follow-up). Two patients were alive and asymptomatic 30 years after valve replacement; 33 patients underwent reoperations due to valve dysfunction, and 19 died during the follow-up period. At 30 years, the actuarial survival was 49.2 ± 8.6%; freedom from rupture, 27.0 ± 10.2%; freedom from calcification, 78.8 ± 8.6%; and freedom from reoperation, 18.8 ± 7.5%. CONCLUSIONS: The dura mater bioprosthesis played an important role in the treatment of patients with mitral and tricuspid valve disease. The low rate of thromboembolism and the long period of follow-up without evidence of valve dysfunction, which occurred for several of our patients, are important characteristics of these bioprosthesis.

Flow cytometry analyses of calcification propensity

Dissertação de mestrado integrado em Engenharia Biomédica (área de especialização em Engenharia Clínica)

Diagnóstico pela ecocardiografia transesofágica e evolução de 35 pacientes portadores de flail mitral valve

OBJETIVO: Descrever os achados ao ecocardiograma transesofágico e evolução clínica de portadores de flail mitral valve. MÉTODOS: No período de janeiro/93 a março/97, 1675 pacientes foram submetidos, em nossa instituição, a ecocardiograma transesofágico, sendo que em 35 casos foi feito o diagnóstico de flail mitral valve e, posteriormente, obtida sua evolução clínica. RESULTADOS: A idade dos pacientes variou 12 a 87 anos (média 65±15) e 27 (77%) eram do sexo masculino. O folheto posterior foi o mais acometido (25 pacientes, 71%). O mecanismo do flail foi ruptura de cordoalha tendínea em todos os casos, exceto um, que apresentava importante alongamento e redundância de cordoalha. A etiologia foi prolapso e/ou degeneração mixomatosa em 15 pacientes, degenerativa em 9, isquêmica em 5, reumática em 4 e endocardite em 3. Regurgitação mitral de grau importante ocorreu em 25 (71%) pacientes e moderada em 10 (29%). O tempo médio de acompanhamento foi de 375±395 dias (1 a 1380). Foram submetidos a tratamento clínico 19 pacientes e a tratamento cirúrgico 16, sendo que em todos foi confirmado o diagnóstico transesofágico. O número total de óbitos (hospitalar e pós-hospitalar) foi alto (34%), tanto em pacientes submetidos a tratamento clínico quanto cirúrgico. Entre os sobreviventes, 17 estão em classe funcional (CF) I e 6 em CF II da NYHA. CONCLUSÃO: O diagnóstico de flail mitral valve ao ecocardiograma transesofágico é acurado, permitindo a definição de sua etiologia e mecanismo. A alta mortalidade à época do diagnóstico, provavelmente, se relaciona à gravidade da doença subjacente. Embora os pacientes não operados estejam evoluindo bem, a baixa CF observada nestes pacientes pode ser atribuída ao curto período de seguimento.

Mitral valve disease with rheumatic appearance in the presence of left ventricular endomyocardial fibrosis

This is a report of a nine-year-old boy with both mitral stenosis and regurgitation and extensive endomyocardial fibrosis of the left ventricle. Focus is given to the singularity of the fibrotic process, with an emphasis on the etiopathogenic aspects.