Unusual presentation of multiple aneurysms of the ascending aorta

Abstract

At the beginning of the twentieth century, Carrell and Guthrie were the first to use homografts to reconstruct dilated vessels, starting a new era in aorta surgery.1 In 1952, Cooley and DeBakey conducted the first successful ascending aorta intervention without cardiopulmonary bypass in the resection of a sacciform aneurysm by aortorrhaphy.2 In 1956, the same authors performed the first successful replacement of the ascending aorta using cardiopulmonary bypass.3 Since then, aortic surgery has developed quickly because of advances in cardiopulmonary bypass, postoperative care, and surgical techniques that reduce the mortality rates of these procedures.4,5 At present, less invasive techniques, such as endovascular interventions, are performed, especially in thoracic and thoracoabdominal aortic disease, depending on factors such as whether the patients would be at high risk during conventional procedures.6,7 Recent studies and case reports have shown the possibility of performing endovascular procedures in the ascending aorta, which seems to be a promising approach.7 Concerning ascending aorta aneurysms, there are several surgical approaches depending on the level of the disease of the aortic valve, aortic root, sinotubular junction and the ascending aorta. These issues are relevant in the context of congenital diseases such as Marfans syndrome,7 Ehlers-Danlos syndrome,8 congenital aortic valve malformations, and acquired aortic valve diseases.9 It is accepted that apoptosis is the major mechanism for the control of cell density in developing physiological and pathological conditions affecting smooth muscle cells. It was shown that death signals may be triggered outside the cells by cytokine pathways and stress mechanical forces.8 Today, it is known that when there is aortic dilation, there is also cystic medial necrosis. This histological abnormality is characterized by a triad of noninflammatory smooth muscle cell loss, the fragmentation of elastic fibers and the accumulation of basophilic ground substance within cell-depleted areas of the medial layer of the vessel wall, all of which are noninflammatory in nature.9 Medial degeneration does not uniformly involve the ascending aorta.8,9 The convexity of the vessel looks more damaged because of more severe medial necrosis, greater loss of smooth muscle cells and apoptosis and greater elastic fiber fragmentation. These alterations play an important role in the development of aortic aneurysms because they participate in the matrix remodeling process resulting in the synthesis of extracellular proteins such as collagen, elastin, and proteoglycans.8