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Today, cardiovascular symptoms of Marfan syndrome are still the most significant issues in diagnosis and management of the disease, but adequate prophylactic monitoring and prophylactic therapy offers something approaching a normal lifespan, and more manifestations of the disease are being discovered as more patients live longer. [75]
Congenital contractural arachnodactyly (CCA), also known as Beals–Hecht syndrome, is a rare autosomal dominant congenital connective tissue disorder. [1] As with Marfan syndrome, people with CCA typically have an arm span that is greater than their height and very long fingers and toes. [2]
The Bentall procedure is a type of cardiac surgery involving composite graft replacement of the aortic valve, aortic root, and ascending aorta, with re-implantation of the coronary arteries into the graft. This operation is used to treat combined disease of the aortic valve and ascending aorta, including lesions associated with Marfan syndrome.
People with symptoms suggestive of thoracic aortic dissection should be routinely evaluated "to establish a pretest risk of disease that can then be used to guide diagnostic decisions." People diagnosed with Marfan syndrome should immediately have an echocardiogram to measure the aorta and followed up six months later to check for aortic ...
An aortic aneurysm is an enlargement (dilatation) of the aorta to greater than 1.5 times normal size. [1] Typically, there are no symptoms except when the aneurysm dissects or ruptures, which causes sudden, severe pain in the abdomen and lower back. [2] [3] The etiology remains an area of active research.
[13] [20] Marfan Syndrome is found in 5–9% of individuals who had an aortic dissection. In this subset, the incidence in young individuals is increased. Individuals with Marfan syndrome tend to have aneurysms of the aorta and are more prone to proximal dissections of the aorta. [22]
What is Marfan syndrome? This genetic disorder of the connective tissue impacts heart health. Causes long bones. People have long arms, legs.
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] Occasionally, abdominal, back, or leg pain may occur. [2] Large aneurysms can sometimes be felt by pushing on the ...