Aortic dissection begins as an intimal tear which then allows for blood to pass through the weakened media of the aortic wall. The most common cause of aortic dissection is hypertension but it can also be caused by connective tissue diseases such as Marfan’s syndrome and Ehlers-Danlos syndrome.
Marfan’s syndrome is caused by a gene mutation in FBN1 on chromosome 15 leading to a defect in fibrillin (a glycoprotein that forms a sheath around elastin). It causes cystic medial necrosis of the media which is due to fragmentation of elastic laminae with an accumulation of myxoid material in aortic media leading to aortic dissection.
Other cardiac pathology associations include aortic valve incompetence and mitral valve prolapse. Other findings of the syndrome are tall stature with long extremities, hypermobile joints, pectus excavatum, arachnodactyly and upward/temporal subluxation of lenses.
Aortic dissection begins as an intimal tear which then allows for blood to pass through the weakened media of the aortic wall. The most common cause of aortic dissection is hypertension but it can also be caused by connective tissue diseases such as Marfan’s syndrome and Ehlers-Danlos syndrome.
Marfan’s syndrome is caused by a gene mutation in FBN1 on chromosome 15 leading to a defect in fibrillin (a glycoprotein that forms a sheath around elastin). It causes cystic medial necrosis of the media which is due to fragmentation of elastic laminae with an accumulation of myxoid material in aortic media leading to aortic dissection.
Other cardiac pathology associations include aortic valve incompetence and mitral valve prolapse. Other findings of the syndrome are tall stature with long extremities, hypermobile joints, pectus excavatum, arachnodactyly and upward/temporal subluxation of lenses.