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Aortic Diseases (also see Chapter 52 )

Aneurysms

Thoracic aortic aneurysms are much less common than abdominal aortic aneurysms. Within the thorax, aortic aneurysms occur most frequently in the ascending aorta, followed by the descending aorta. Aneurysms of the arch only are uncommon. Thoracoabdominal aneurysms are aneurysms of the descending thoracic aorta that extend into the abdominal aorta. Aneurysms of the ascending and descending aorta tend to have different etiologies and natural histories and are managed differently. Aneurysms of the ascending aorta most often result from cystic medial necrosis. This disorder of smooth muscle and elastic fiber in the wall of the aorta is a frequent finding in patients with connective tissue disorders such as Marfan's syndrome. However, many patients with ascending aortic aneurysms have idiopathic cystic medial necrosis of the aorta. Descending aortic aneurysms are most often associated with atherosclerosis. Uncommon causes of thoracic aortic aneurysms include syphilis, mycotic infections, aortic trauma, and confined aortic dissection.

Approximately 40% of patients with thoracic aortic aneurysms are symptomatic at diagnosis. Patients typically have symptoms secondary to some vascular consequence of the aneurysm or secondary to a local compressive/mass effect. Vascular consequences can include aortic incompetence with symptoms of congestive heart failure (CHF), coronary ischemia as a result of aneurysmal compromise of the coronary ostia and proximal coronary arteries, and thromboembolic events. Rarely, thoracic aortic aneurysms can rupture into the heart itself, especially the left atrium. Symptoms may also be induced as a result of compression of local structures, such as the tracheobronchial tree, the superior vena cava, or the esophagus.


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Figure 50-25 A, Influence of aortic size on the cumulative lifetime incidence of natural complications of an aortic aneurysm. On the y axis is plotted the incidence of natural complications (rupture or dissection); on the x axis, aortic size is plotted. This plot is for the ascending aorta. Note the hinge point at 6 cm. B, The same plot for the descending aorta. Note the hinge point at 7 cm.

Patients with symptomatic thoracic aortic aneurysms are always managed surgically. Gott and colleagues noted that nearly half of their marfanoid patients with an aortic root greater than 6.5 cm had aortic dissection.[155] Hence, they recommend prophylactic surgical repair in patients with an aneurysmal size of even less than 6.5 cm. Other studies[156] have examined complications in patients with ascending and descending thoracic aortic aneurysms as a function of aneurysm size ( Fig. 50-25 ) and have recommended criteria for prophylactic surgical intervention for Marfan- and non-Marfan-related aneurysms at each location ( Table 50-6 ).[156]

Dissections

Aortic dissections are not common, but they are frequently catastrophic. The mechanisms underlying the development of aortic dissection are not necessarily agreed on. Most experts believe that aortic dissections begin with an intimal tear that results in the development of an intimal flap. They are observed most frequently (65%) in the ascending aorta. However, autopsy studies do not always
TABLE 50-6 -- Size criteria for surgical intervention for asymptomatic thoracic aortic aneurysm

Non-Marfan's Marfan's (or Familial)
Ascending 5.5 cm 5.0 cm
Descending 6.5 cm 6.0 cm
From Elefteriades JA: Natural history of thoracic aortic aneurysms: Indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg 74:S1877–S1880, 2002.

identify an intimal tear, and it has been suggested that aortic dissections begin in the media of the vessel wall with rupture of the vasa vasorum and subsequent rupture into the vessel lumen ( Fig. 50-26 ). [157] In patients with connective tissue disorders (e.g., Marfan's syndrome), cystic medial degeneration causes weakness of the vessel wall and predisposes to dissection. Patients without connective tissue disorders lack the classic histologic features of medial degeneration. However, the degree of medial degeneration in these patients far exceeds that observed in an appropriately age-matched population. Advanced age and hypertension are the two most important factors associated with medial degeneration and aortic dissection in these patients. Other clinical conditions are also associated with dissection, including bicuspid aortic valves, coarctation, pregnancy, blunt trauma, and postaortotomy.

Aortic dissections are classified by location ( Table 50-7 , Fig. 50-27 ) [157] and duration. The use of more than one classification to characterize location is potentially confusing, but can be simplified in that ascending dissections (DeBakey types I and II, Stanford type A) require emergent surgical intervention whereas descending dissections (DeBakey type III, Stanford type B) are initially treated medically by controlling blood pressure. Acute dissection describes those less than 2 weeks. After 2 weeks, the mortality secondary to dissection levels off, thus rendering the management of chronic dissections very different from that of acute dissections.

Patients with dissections most often have severe pain (sometimes described by patients as ripping or tearing) that may migrate with extension of the dissection. Ascending dissection is associated with anterior chest pain, whereas involvement of the descending aorta is associated with back pain. Patients may also have acute CHF (secondary to acute aortic incompetence), syncope, cerebral vascular accidents, or cardiac arrest.

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