Herz
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Non-invasive tomographic imaging modalities have recently contributed to identifying aortic intramural hematoma, a variant form of classic dissection, which is characterized by the absence of an intimal tear and thus the absence of direct flow communication, and which represents an important disease entity in acute aortic syndrome. Clinical investigations have revealed that intramural hematoma has characteristic clinical features and that the natural remodeling process of hematoma is different from that of classic aortic dissection. These findings suggest that intramural hematoma is not just a precursor to aortic dissection, but may be a unique disease entity with a more favorable prognosis compared to aortic dissection. ⋯ Due to favorable outcomes with medical treatment in Asian patients with type A intramural hematoma, a tailored or individualized approach based on risk stratification using initial clinical information and including imaging studies and timely surgical repair has been suggested in hemodynamically stable patients; however, these results need to be confirmed in other patient populations. The concept of a "micro-tear" which cannot be easily visualized using conventional imaging modalities has been raised: "echo-free space" on transesophageal echocardiography and "focal contrast enhancement" on ultra-fast computerized tomography have drawn many physicians' attention, and the possibility of a pathophysiologic link between classic aortic dissection and intramural hematoma has been discussed. Further investigations are needed to test whether intramural hematoma begins with an initial intimal tear and a different aortic status--characterized by a more rigid and non-compliant aorta associated with old age or long-standing hypertension--and results in an apparently absent intimal flap.
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Marfan syndrome is a hereditary disease with a prevalence of 2-3 in 10,000 births, leading to a fibrillin connective tissue disorder with manifestations in the skeleton, eye, skin, dura mater and in particular the cardiovascular system. Since other syndromes demonstrate similar vascular manifestations, but therapy may differ significantly, diagnosis should be established using the revised Ghent nosology in combination with genotypic analysis in specialized Marfan centres. The formation of aortic root aneurysms with the subsequent risk of acute aortic dissection type A (AADA) or aortic rupture limits life expectancy in patients with Marfan syndrome. ⋯ Although results of prospective randomised long-term studies comparing surgical techniques are lacking, the David operation has become the surgical method of choice for aortic root aneurysms, not only at the Heidelberg Marfan Centre. Replacement of the aneurysmal dilated aortic arch is performed under moderate hypothermic circulatory arrest combined with antegrade cerebral perfusion using a heart-lung machine, which we also use in thoracic or thoracoabdominal aneurysms. Close post-operative follow-up in a Marfan centre is pivotal for the early detection of pathological changes on the diseased aorta.
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The working group "Aortic Surgery and Interventional Vascular Surgery" of the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) set up the German registry for acute aortic dissection type A (GERAADA) in July 2006. This web-based database was developed to record data of patients who had undergone surgery for aortic dissection type A (AADA). The aim of GERAADA is to learn from analyzing the data of AADA patients how to improve the perioperative management and surgical treatment of patients with AADA and to identify possible parameters affecting patient risk and outcome. ⋯ A European Registry of Aortic Diseases ("EuRADa") is being established this year under the leadership of the "Vascular Domain" of the European Association for Cardio-Thoracic Surgery (EACTS). This database will collect parameters on all aortic diseases, dissection types A and B, aneurysms, perforating ulcer (PAU), intramural wall hematoma (IMH), traumatic aortic ruptures, and all potential treatment strategies (medical treatment, open surgical and endovascular).