The megaaortic syndrome: Progression of ascending aortic aneurysm or a disease of distinct origin?

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• 作者：Ulrike Baranyi^a ; ¹ ; Christian Stern^a ; ¹ ; Birgitta Winter^a ; ¹ ; Adrian T&uuml ; rkcan^a ; ¹ ; Bernhard Scharinger^a ; ¹ ; Marie-Elisabeth Stelzm&uuml ; ller^b ; ¹ ; Thomas Aschacher^b ; ¹ ; Martin Andreas^b ; ¹ ; Marek Ehrlich^b ; ¹ ; G&uuml ; nther Laufer^b ; ¹ ; David Bernhard^c ; ¹ ; Barbara Messner^a ; ¹ ; ^{Barbara.messner@meduniwien.ac.at}
• 关键词：Thoracic aortic aneurysms ; Atherosclerosis ; Megaaortic syndrome ; MMP-9
• 刊名：International Journal of Cardiology
• 出版年：2017
• 出版时间：15 January 2017
• 年：2017
• 卷：227
• 期：Complete
• 页码：717-726
• 全文大小：2215 K
• 卷排序：227

文摘

Thoracic aortic aneurysm (TAA) is an often asymptomatic disease with fatal outcome, such as dissection or rupture. The megaaortic syndrome (MAS) is an extensive dilatation of the whole aorta with low incidence but high lethal outcome with unknown pathophysiology so far.Methods and resultsWe compared aortic tissue of patients with sporadic TAAs and MAS of the ascending aorta with non-aneurysmal control tissues. Specimens of MAS patients showed a significantly reduced thickness of the media but an increased thickness of the intima compared to control tissue and TAAs with moderate dilatation. Advanced media degeneration however was detectable in both, TAAs with enhanced luminal diameter and MAS specimens, accompanied by reduced medial smooth muscle cell-density. Further specimens of MAS were characterized by massive atherosclerotic lesions in contrast to specimens of sporadic TAA patients. Infiltrations of macrophages in atherosclerotic lesions but also in the media adjacent to the adventitia were significantly elevated in tissue of TAAs with dilatation ≤ 6 cm. Of note, atherosclerotic plaque-associated macrophages as well as those in the external media produce huge amounts of MMP-9 which is possibly involved in media degeneration and tissue destruction.ConclusionsTaken together these results demonstrate that the pathology of MAS shows similarities with that of TAAs but pathological differences in the ascending aorta, suggesting that MAS might be a disease of different origin.