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The treatment of arterial tortuosity syndrome entails possible surgery for aortic aneurysms, as well as regular clinical surveillance including regular follow-up echocardiograms. [7] The prognosis and lifespan of this condition are unclear. Early reports of mortality were high, [10] but more recent data suggests about 12% mortality. [5] [11]
Aortic unfolding is an abnormality visible on a chest X-ray, that shows widening of the mediastinum which may mimic the appearance of a thoracic aortic aneurysm. [ 1 ] With aging, the ascending portion of the thoracic aorta increases in length by approximately 12% per decade, whereas the diameter increases by just 3% per decade.
A thoracic aortic aneurysm is an aortic aneurysm that presents primarily in the thorax. A thoracic aortic aneurysm is the "ballooning" of the upper aspect of the aorta, above the diaphragm. Untreated or unrecognized they can be fatal due to dissection or "popping" of the aneurysm leading to nearly instant death.
Loeys–Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder. It has features similar to Marfan syndrome and Ehlers–Danlos syndrome. [3] [4] [5] The disorder is marked by aneurysms in the aorta, often in children, and the aorta may also undergo sudden dissection in the weakened layers of the wall of the aorta.
The aortic wall dilatation at the commissural level causes the cusps to effectively shorten and prevent them from converging during systole, which results in aortic valve incompetence. The arch is typically spared from the aneurysmal process, though it may involve the entire ascending aorta. The ectatic aorta may experience dissections.
The aorta normally has three small pouches that sit directly above the aortic valve (the sinuses of Valsalva), and an aneurysm of one of these sinuses is a thin-walled swelling. Aneurysms may affect the right (65–85%), non-coronary (10–30%), or rarely the left (< 5%) coronary sinus. [ 1 ]
The aberrant right subclavian artery frequently arises from a dilated segment of the proximal descending aorta, the so-called Diverticulum of Kommerell (which was named for the German radiologist Burkhard Friedrich Kommerell (1901–1990), who discovered it in 1936). [5] [6] It is alternatively known as a lusorian artery. [1] [3]
The aortic wall is made up of three different components the inner layer (intima), the muscle layer (media), and the outer layer (adventitia). A traumatic injury to the thoracic aorta can cause disruption of any of these parts. Therefore, aortic injury is on a scale from injury to a part of the inner layer to a complete tear of all three layers ...