ABSTRACT
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.
Subject(s)
Aortic Aneurysm, Thoracic/surgery , Blood Vessel Prosthesis , Endovascular Procedures/instrumentation , Loeys-Dietz Syndrome/surgery , Marfan Syndrome/complications , Stents , Adult , Aortic Aneurysm, Thoracic/diagnostic imaging , Aortic Aneurysm, Thoracic/genetics , Endovascular Procedures/adverse effects , Female , Humans , Loeys-Dietz Syndrome/diagnostic imaging , Loeys-Dietz Syndrome/genetics , Male , Marfan Syndrome/diagnosis , Marfan Syndrome/genetics , Middle Aged , Postoperative Complications/prevention & control , Prosthesis Design , Risk Factors , Treatment Outcome , Young AdultABSTRACT
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.
Subject(s)
Aorta/metabolism , Aortic Aneurysm/blood , Aortic Valve/abnormalities , Biomarkers/blood , Heart Valve Diseases/complications , Aorta/diagnostic imaging , Aorta/surgery , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/etiology , Aortic Aneurysm/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Bicuspid Aortic Valve Disease , Blood Vessel Prosthesis Implantation , Clinical Decision-Making , Dilatation, Pathologic , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/surgery , Heart Valve Prosthesis Implantation , Humans , Predictive Value of Tests , Prognosis , Risk FactorsABSTRACT
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.
