RESUMO
PURPOSE: To describe a novel modification technique to lower the distal radial force of a thoracic stent-graft so as to avert stent-graft-induced new entry tears (SINE) in the fragile aorta of patients with genetic aortic disease and aortic dissection. TECHNIQUE: A commercially available thoracic stent-graft is partially deployed on a back table. The most distal Z-stent is removed, the distal fabric is marked by vascular clips, and the modified stent-graft is reloaded and deployed in the true lumen of an aortic dissection. The technique is demonstrated in 3 patients with aortic dissection related to genetic aortic diseases. CONCLUSION: Creating a low distal radial force stent-graft is easy and can be done in a short time. Endovascular implantation appears feasible and safe.
Assuntos
Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Procedimentos Endovasculares/instrumentação , Síndrome de Loeys-Dietz/cirurgia , Síndrome de Marfan/complicações , Stents , Adulto , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/genética , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Síndrome de Loeys-Dietz/diagnóstico por imagem , Síndrome de Loeys-Dietz/genética , Masculino , Síndrome de Marfan/diagnóstico , Síndrome de Marfan/genética , Pessoa de Meia-Idade , Complicações Pós-Operatórias/prevenção & controle , Desenho de Prótese , Fatores de Risco , Resultado do Tratamento , Adulto JovemRESUMO
Traditional risk stratification model of bicuspid aortic valve (BAV) aortopathy is based on measurement of maximal cross-sectional aortic diameter, definition of proximal aortic shape, and aortic stiffness/elasticity parameters. However, conventional imaging-based criteria are unable to provide reliable information regarding the risk stratification in BAV aortopathy, especially considering the heterogeneous nature of BAV disease. Given those limitations of conventional imaging, there is a growing clinical interest to use circulating biomarkers in the screening process for thoracic aortic aneurysms as well as in the risk-assessment algorithms. We aimed to systematically review currently available biomarkers, which may be of value to predict the natural evolution of aortopathy in individuals with BAV.
Assuntos
Aorta/metabolismo , Aneurisma Aórtico/sangue , Valva Aórtica/anormalidades , Biomarcadores/sangue , Doenças das Valvas Cardíacas/complicações , Aorta/diagnóstico por imagem , Aorta/cirurgia , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/etiologia , Aneurisma Aórtico/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Doença da Válvula Aórtica Bicúspide , Implante de Prótese Vascular , Tomada de Decisão Clínica , Dilatação Patológica , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Humanos , Valor Preditivo dos Testes , Prognóstico , Fatores de RiscoRESUMO
In this overview we aim to address a number of recent insights and developments regarding clinical aspects, etiology, and treatment of Heritable Thoracic Aortic Disease (H-TAD). We will focus on monogenetic disorders related to aortic aneurysms. H-TADs are rare but they provide a unique basis for the study of underlying pathogenetic pathways in the complex disease process of aneurysm formation. The understanding of pathomechanisms may help us to identify medical treatment targets to improve prognosis. Among the monogenetic aneurysm disorders, Marfan syndrome is considered as a paradigm entity and many insights are derived from the study of clinical, genetic and animal models for Marfan syndrome. We will therefore first provide a detailed overview of the various aspects of Marfan syndrome after which we will give an overview of related H-TAD entities.
