What causes aortic dissection?
Aortic dissection is caused by a deterioration of the inner lining of the aorta. There are a number of conditions that predispose a person to develop defects of the inner lining, including high blood pressure, Marfan's disease, Ehlers-Danlos syndrome, connective tissue diseases, and defects of heart development which begin during fetal development. A dissection can also occur accidentally following insertion of a catheter, trauma, or surgery. The main symptom is sudden,
intense pain. The pain can be so intense as to immobilize the patient and cause him to fall to the ground. The pain is frequently felt in both the chest and in the back, between the shoulder blades. The extent of the pain is proportional to the length of the dissection.
The initiating event in an aortic dissection is a tear in the intimal lining of the aorta. Due to the high pressures in the aorta, blood enters the media at the point of the tear. The force of the blood entering the media causes the tear to extend. It may extend proximally (closer to the heart) or distally (away from the heart) or both. The blood will travel through the media, creating a false lumen (the true lumen is the normal conduit of blood in the aorta). Separating the false lumen from the true lumen is a layer of intimal tissue. This tissue is known as the intimal flap. The vast majority of aortic dissections originate with an intimal tear in either the ascending aorta (65%), the aortic arch (10%), or just distal to the ligamentum arteriosum in the descending thoracic aorta (20%). As blood flows down the false lumen, it may cause secondary tears in the intima. Through these secondary tears, the blood can re-enter the true lumen. While it is not always clear why an intimal tear may occur, quite often it involves degeneration of the collagen and elastin that make up the media. This is known as cystic medial necrosis and is most commonly associated with Marfan syndrome and is also associated with Ehlers-Danlos syndrome.
In about 13% of aortic dissections, there is no evidence of an intimal tear. It is believed that in these cases the inciting event is an intramural hematoma (caused by hemorrhage within the media). Since there is no direct connection between the true lumen and the false lumen in these cases, it is difficult to diagnose an aortic dissection by aortography if the etiology is an intramural hematoma. An aortic dissection secondary to an intramural hematoma should be treated the same as one caused by an intimal tear.
Aortic dissection is associated with hypertension (high blood pressure) and many connective tissue disorders. Vasculitis (inflammation of an artery) is rarely associated with aortic dissection. It can also be the result of chest trauma. 72 to 80% of individuals who present with an aortic dissection have a previous history of hypertension.
The highest incidence of aortic dissection is in individuals who are 50 to 70 years old (in their 6th and 7th decade of life). The incidence is twice as high in males as in females (male-to-female ratio is 2:1). Half of dissections in females before age 40 occur during pregnancy (typically in the 3rd trimester or early postpartum period).
A bicuspid aortic valve (a type of congenital heart disease involving the aortic valve) is found in 7-14% of individuals who have an aortic dissection. These individuals are prone to dissection in the ascending aorta. The risk of dissection in individuals with bicuspid aortic valve is not associated with the degree of stenosis of the valve. Marfan syndrome is noted in 5-9% of individuals who suffer from aortic dissection. In this subset, there is an increased incidence in young individuals. Individuals with Marfan syndrome tend to have aneurysms of the aorta and are more prone to proximal dissections of the aorta. Chest trauma leading to aortic dissection can be divided into two groups based on etiology: blunt chest trauma (commonly seen in motor vehicular accidents) and iatrogenic. Iatrogenic causes include trauma during cardiac catheterization or due to an aortic balloon pump.
Aortic dissection may be a late sequalae of cardiac surgery. 18% of individuals who present with an acute aortic dissection have a history of open heart surgery. Individuals who have undergone aortic valve replacement for aortic insufficiency are at particularly high risk. This is because aortic insufficiency causes increased blood flow in the ascending aorta. This can cause dilatation and weakening of the walls of the ascending aorta. |
