The distal aorta in the Marfan syndrome

Mulder, Barbara J.M.

doi:10.1007/bf03086183

Cited by 25 publications

(6 citation statements)

References 34 publications

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“…Нам не удалось обнаружить лите-ратурных данных, подтверждающих или опровергаю-щих данный тезис. Тем не менее, при естественном течении аортальной патологии [17] при синдроме Марфана существует зависимость между возрастом дебюта аортальной патологии и "агрессивностью" роста аневризм и возникновения расслоений аорты.…”

Section: Discussionunclassified

The Condition of Distal Aorta After Proximal Reconstruction for Dissection in Marfan Syndrome

Чернявский¹,

Ляшенко²,

Сирота³

et al. 2016

Russ J Cardiol

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Marfan syndrome is a disease associated with high prevalence of aorta-related morbidity and mortality. Dissections and ruptures of thoracal aorta (ThAo) lead to decreased life duration in persons with Marfan syndrome with mediana at 30 year-old. Aim. To study the changes of distal aorta and clinical outcomes after surgical treatment for proximal aorta dissection in Marfan syndrome. Material and methods. In the SRICP, among patients operated for proximal aorta dissection, 14 were selected, who had histologically proven Marfan syndrome. They were followed-up. Results. In the long-term follow-up after the intervention on thoracoabdominal aorta (TAAo) was required in 2 patients. Reasons for transforming of the distal aorta were the age of the disease onset (p=0,04), acute stage of dissection (p=0,02). Changes in TAAo developed during first months after primary intervention, as in several years. Conclusion.Surgery for proximal dissections in Marfan syndrome patients is related to acceptable levels of morbidity and mortality in early and post-operation periods. Regular follow-up is required of such patients in post-operation period for on-time diagnostics and performing of secondary interventions.Russ J Cardiol 2016, 11 (139): 7-11 http://dx

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Section: Discussionunclassified

The Condition of Distal Aorta After Proximal Reconstruction for Dissection in Marfan Syndrome

Чернявский¹,

Ляшенко²,

Сирота³

et al. 2016

Russ J Cardiol

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“…Although sudden death from aortic dissection is decreasing due to improved aortic monitoring and elective aortic root surgery, some patients need to undertake surgery for distal aorta after aortic root surgery [ 10 ]. After elective aortic root surgery, dilatation of the distal aorta is more common than before [ 11 ].…”

Section: Distal Aortamentioning

confidence: 99%

Cardiac Management

Isekame¹,

Gati²

2016

Diagnosis and Management of Marfan Syndrome

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“…Dependendo do grau de envolvimento das carótidas, alguns pacientes podem ter seqüelas neurológicas e o envolvimento das coronárias pode levar a infarto agudo do miocárdio e morte súbita. O mecanismo pelo qual o paciente evolui a óbito é pela ruptura do saco pericárdico e posterior tamponamento cardíaco (26,27,28) .…”

Section: Sistema Cardiovascularunclassified

Síndrome de Marfan

et al. 2017

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Resumo: O presente estudo consiste numa revisão bibliográfica das bases de dados PubMed e Scielo. Os artigos foram buscados utilizando a expressão Marfan Syndrome. Obteve 239 artigos de livre acesso no PubMed e 06 no Scielo, foram selecionados 37 trabalhos mais relevantes dos últimos cinco anos no tocante aos aspectos genéticos, fisiopatologia, diagnóstico e tratamento, e mais sete de datas anteriores, devido a sua importância histórica. Síndrome de Marfan é uma doença sistêmica, autossômica dominante do tecido conjuntivo causada por mutações nas proteínas da matriz extracelular, especialmente a fibrilina-1. Afeta principalmente os sistemas cardiovascular, esquelético e olhos. As manifestações mais freqüentes são: aneurisma de aorta torácica, luxação do cristalino ocular e crescimento exagerado de ossos longos. Há um novo entendimento sobre o papel da regulação do fator de crescimento tumoral β. Esta revisão destaca fisiopatologia, manifestações clínicas, tratamento padrão, bem como opções terapêuticas inovadoras. Considera importante o acompanhamento multidisciplinar, objetivando o diagnóstico precoce, reduzindo a morbimortalidade dos indivíduos afetados.Palavras-chave: Síndrome de Marfan; FBN1; Diagnóstico; Tratamento; TGF-β. Marfan SyndromeAbstract: This study is a review of the PubMed and Scielo database. Articles were searched using the terms Marfan Syndrome. We obtained 239 freely accessible articles on PubMed and 06 on Scielo, we selected 37 most relevant papers of the last five years, related to genetic aspects, pathophisiology, diagnostic and management, and 07 oldest because of their historic importance. Marfan syndrome is a systemic autosomal dominant connective tissue disease caused by mutations in the extracellular matrix proteins, especially the fibrillin-1. Affects primarily the cardiovascular, skeletal and eye. The most frequent manifestations are: thoracic aortic aneurysm, dislocation of the ocular lens and overgrowth of long bones. There is a new understanding of the role of regulation of tumor growth factor β. This review emphasizes pathophysiology, clinical manifestations, standard treatment and innovative therapeutic options. Considers the importance of a multidisciplinary follow, aiming at early diagnosis, reducing the morbidity of affected individuals.

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The distal aorta in the Marfan syndrome

Cited by 25 publications

References 34 publications

The Condition of Distal Aorta After Proximal Reconstruction for Dissection in Marfan Syndrome

The Condition of Distal Aorta After Proximal Reconstruction for Dissection in Marfan Syndrome

Cardiac Management

Síndrome de Marfan

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