Which condition is an autosomal dominant connective tissue disorder associated with aortic dissection?

Marfan's syndrome is recognized as an autosomal dominant connective tissue disorder that significantly increases the risk of aortic dissection. This condition is characterized by a range of symptoms affecting the skeletal system, cardiovascular system, and eyes. The primary issue in Marfan's syndrome is a mutation in the fibrillin-1 gene, which is crucial for the formation of elastic fibers in connective tissues. Because of this mutation, individuals with Marfan's syndrome can experience abnormalities in blood vessels, particularly the aorta, leading to its dilation and potential dissection.

The cardiovascular complications in Marfan's syndrome, particularly involving the aorta, are a critical concern, as they can lead to life-threatening situations if not monitored and managed appropriately. Regular echocardiographic evaluations are essential for individuals with this syndrome to detect aortic dilation early.

Other options do not share the defining features of an autosomal dominant connective tissue disorder characterized by aortic dissection. For example, neurofibromatosis type 2 primarily affects the nervous system and does not have the same connective tissue implications or aortic complications as Marfan's syndrome. Huntington's chorea is a neurodegenerative disorder, and sickle cell anemia is an inherited blood disorder related to hemoglobin, neither