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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]
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.
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.
Abdominal aortic aneurysm (AAA) is a localized enlargement of the abdominal aorta such that the diameter is greater than 3 cm or more than 50% larger than normal. [1] An AAA usually causes no symptoms, except during rupture. [ 1 ]
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.
ICD-10 is the 10th revision of the International Classification of Diseases (ICD), a medical classification list by the World Health Organization (WHO). It contains codes for diseases, signs and symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or diseases. [1]
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.
[1] [2] This disorder is the cause of 20% of thoracic aortic aneurysms [3] [4] Some families affected by this condition have shown mild versions of some symptoms that are associated with Marfan syndrome and Loeys-Dietz syndrome , these signs include tall stature, joint hypermobility , cutaneous stretch marks , and either pectus excavatum or ...