Valva aórtica bicúspide: fundamentos teóricos e clínicos para substituição simultânea da aorta ascendente

The bicuspid aortic valve (AV) is the most common cardiac congenital anomaly and has been found to be a significant risk factor for developing calcific AV disease. However, the mechanisms of disease development remain unclear. In this study we quantified the structure of human normal and bicuspid leaflets in the early disease stage. From these individual leaflet maps average fiber structure maps were generated using a novel spline based technique. Interestingly, we found statistically different and consistent regional structures between the normal and bicuspid valves. The regularity in the observed microstructure was a surprising finding, especially for the pathological BAV leaflets and is an essential cornerstone of any predictive mathematical models of valve disease. In contrast, we determined that isolated valve interstitial cells from BAV leaflets show the same in vitro calcification pathways as those from the normal AV leaflets. This result suggests the VICs are not intrinsically different when isolated, and that external features, such as abnormal microstructure and altered flow may be the primary contributors in the accelerated calcification experienced by BAV patients.

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“…It should be noted that BAV is part of a larger spectrum of AV structural anomalies that range from single to quad leaflet forms. 5,16,17,23 …”

Section: Introductionmentioning

confidence: 99%

Architectural Trends in the Human Normal and Bicuspid Aortic Valve Leaflet and Its Relevance to Valve Disease

et al. 2014

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“…Indeed, in patients with BAV without dilatation of the ascending aorta, even successful valve replacement is not able to prevent long-term dilatation of the ascending aorta. Transthoracic echocardiography (TTE) is the first standard examination during the followup of these patients [18]. Currently, in patients with previous BAV and aortic valve replacement, transthoracic echocardiographic followup is performed twice a year and it is integrated with angio-CT in the case of initial signs of aortic disease or of suspicious echocardiographic findings.…”

Section: Discussionmentioning

confidence: 99%

The Role of ECG-Gated CT in Patients with Bicuspid Aortic Valve Replacement: New Perspectives in Short- and Long-Term Followup

et al. 2013

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The aim of our study was to compare the results of the TTE (transthoracic echocardiography) with the results obtained by the ECG-gated 64 slices CT during the followup of patients with bicuspid aortic valve (BAV), after aortic valve replacement; in particular we evaluated the aortic root and the ascending aorta looking for a new algorithm in the followup of these patients. From January 1999 to December 2009 our attention was focused on 67 patients with isolated surgical substitution of aortic valve; after dismissal they were strictly observed. During the period between May and September 2010, these patients underwent their last evaluation, and clinical exams, ECG, TTE, and an ECG-gated-MDCT were performed. At followup TTE results showed an aortic root of 36.7 ± 4 mm and an ascending aorta of 39.6 ± 4.8 mm. ECG- gated CT showed an aortic root of 37.9 ± 5.5 mm and an ascending aorta of 43.1 ± 5.2. The comparison between preoperative and postoperative TTE shows a significant long-term dilatation of the ascending aorta while the aortic root diameter seems to be stable. ECG-gated CT confirms the stability of the aortic root diameter (38.2 ± 5.3 mm versus 37.9 ± 5.5 mm; <0.0001) and the increasing diameter value of the ascending aorta (40.2 ± 3.9 mm versus 43.1 ± 5.2 mm; P = 0.0156). Due to the different findings between CT and TTE studies, ECG-gated CT should no longer be considered as a complementary exam in the followup of patients with BAV, but as a fundamental role since it is a real necessity.

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“…It has been noted that the fibrillin-1 content was remarkably reduced in the aorta of BAV patients, compared with that of patients with a tricuspid aortic valve. 12 - 14 …”

Section: Etiologymentioning

confidence: 99%

The bicuspid aortic valve and related disorders

Yuan

Hua

2010

Sao Paulo Med. J.

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Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the population, with strong male predominance. Individuals may have a normally functioning BAV, and may be unaware of its presence and the potential risk of complications. However, they may easily develop aortic valve disorders: either stenotic or regurgitant, or both. Today, BAV is recognized as a syndrome incorporating aortic valve disorders and aortic wall abnormalities, including aortic dilation, dissection or rupture. Congenital or hereditary diseases such as ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, Turner's syndrome, Marfan's syndrome etc., may frequently be associated with BAV. Infective endocarditis and occasionally thrombus formation may develop during the lives of BAV patients. Elevated cholesterol or C-reactive protein may be seen in laboratory findings of these patients. Beta-blockers and statins are the possibilities for medical treatment, and aortic valve repair/replacement and ascending aorta replacement are indicated for patients with a severely diseased aortic valve and aorta. Rigorous follow-up throughout life is mandatory after BAV has been diagnosed. The aim of the present article was to describe the implications of BAV and its associated disorders, and to discuss diagnostic and treatment strategies. RESUMOA válvula aórtica bicúspide (VAB) é a malformação cardíaca congênita, mais comum afetando 1-2% da população, com forte predominância no sexo masculino. Indivíduos afetados podem exibir funcionamento normal da VAB, ignorando sua presença e o potencial risco de complicações associadas.Mas podem facilmente desenvolver disfunções de valva aórtica, com estenose, regurgitação, ou ambas. Hoje em dia, a VAB é reconhecida como uma síndrome incorporando alterações de valva aórtica e anormalidades da parede aórtica, inclusive dilação da aorta, dissecção ou ruptura. Doenças congênitas ou hereditárias, como defeito do septo ventricular, canal arterial patente, coartação de aorta, síndrome de Turner, síndrome de Marfan etc., podem estar frequentemente associadas com VAB. A endocardite infecciosa e ocasionalmente formação de trombo podem se desenvolver durante a vida nos pacientes com VAB. Elevação do colesterol ou proteína C-reativa pode ser observada nos resultados laboratoriais desses pacientes.Betabloqueador e estatinas são as opções de tratamento médico e reparação/substituição da valva aórtica e substituição da aorta ascendente são indicadas para pacientes com doença grave da valva aórtica e artéria aorta. Acompanhamento rigoroso por toda a vida é obrigatório quando do diagnóstico da VAB. O objetivo do presente artigo é descrever as implicações do VAB assim como suas desordens associadas e discutir as estratégias de diagnóstico e tratamento.

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Valva aórtica bicúspide: fundamentos teóricos e clínicos para substituição simultânea da aorta ascendente

Cited by 4 publications

References 46 publications

Architectural Trends in the Human Normal and Bicuspid Aortic Valve Leaflet and Its Relevance to Valve Disease

Architectural Trends in the Human Normal and Bicuspid Aortic Valve Leaflet and Its Relevance to Valve Disease

The Role of ECG-Gated CT in Patients with Bicuspid Aortic Valve Replacement: New Perspectives in Short- and Long-Term Followup

The bicuspid aortic valve and related disorders

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