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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.
For the International Classification of Diseases (ICD-11), the International Paediatric and Congenital Cardiac Code (IPCCC) was developed to standardize the nomenclature of congenital heart disease. Under this system, English is now the official language, and persistent truncus arteriosus should properly be termed common arterial trunk .
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 ]
As the arrangement of connective tissue is disturbed, the vessel wall is no longer able to hold its original conformation and begins to unravel due to the continued hypertension. High blood pressure mold and force the artery to now take on an elongated, tortuous course to better withstand the higher pressures. [citation needed]
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]
Acute aortic syndrome (AAS) describes a range of severe, painful, potentially life-threatening abnormalities of the aorta. [1] These include aortic dissection, intramural thrombus, and penetrating atherosclerotic aortic ulcer. [2] AAS can be caused by a lesion on the wall of the aorta that involves the tunica media, often in the descending ...
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.