A thoracic aortic diameter ≥ 50% larger than normal is considered an aneurysm (normal diameter varies by location). Most thoracic aortic aneurysms do not cause symptoms, although some patients have chest or back pain; other symptoms and signs are usually the result of complications (eg, dissection, compression of adjacent structures, thromboembolism, rupture). Risk of rupture is proportional to the size of the aneurysm. Diagnosis is made by ultrasonography or CT angiography. Treatment is endovascular stent grafting or surgery.
Thoracic aortic aneurysms (TAAs) account for one fourth of aortic aneurysms. Men and women are affected equally. About 40% of TAAs occur in the ascending thoracic aorta (between the aortic valve and brachiocephalic, or innominate, artery), 10% occur in the aortic arch (including the brachiocephalic, carotid, and subclavian arteries), 35% occur in the descending thoracic aorta (distal to the left subclavian artery), and 15% occur in the upper abdomen (as thoracoabdominal aneurysms).
TAAs may dissect (see Aortic Dissection), compress or erode into adjacent structures, and lead to thromboembolism, leak, or rupture. Aneurysms of the ascending aorta sometimes affect the aortic root, causing aortic valve regurgitation or occlusion of the coronary arteria ostia, causing angina, MI, or syncope.
Most TAAs result from atherosclerosis. Risk factors for both include prolonged hypertension, dyslipidemia, and smoking; additional risk factors for TAAs include presence of aneurysms elsewhere and older age (peak incidence at age 65 to 70).
Congenital connective tissue disorders (eg, Marfan syndrome, Ehlers-Danlos syndrome) cause cystic medial necrosis, a degenerative change that leads to TAAs complicated by aortic dissection (see Aortic Dissection) and by widening of the proximal aorta and aortic valve (annuloaortic ectasia), which causes aortic regurgitation. Marfan syndrome causes 50% of cases of annuloaortic ectasia, but cystic medial necrosis and its complications can occur in young people even if no congenital connective tissue disorder is present.
Infected (mycotic) TAAs result from hematogenous spread of systemic or local infections (eg, sepsis, pneumonia), lymphangitic spread (eg, in TB), or direct extension (eg, in osteomyelitis or pericarditis). Bacterial endocarditis and tertiary syphilis are uncommon causes. TAAs occur in some connective tissue disorders (eg, temporal arteritis, Takayasu arteritis, granulomatosis with polyangiitis [Wegener granulomatosis]).
Blunt chest trauma causes pseudoaneurysms (extramural hematomas due to blood that has leaked through the torn aortic wall).
Symptoms and Signs
Most TAAs are asymptomatic until complications (eg, thromboembolism, rupture, aortic regurgitation, dissection) develop. However, compression of adjacent structures can cause chest or back pain, cough, wheezing, dysphagia, hoarseness (due to left recurrent laryngeal or vagus nerve compression), chest pain (due to coronary artery compression), and superior vena cava syndrome. Erosion of aneurysms into the lungs causes hemoptysis or pneumonitis; erosion into the esophagus (aortoesophageal fistula) causes massive hematemesis. Dissection manifests with tearing pain, often radiating to the back. Thromboembolism may cause stroke, abdominal pain (due to mesenteric embolism), or extremity pain. Patients who do not immediately die of a ruptured TAA present with severe chest or back pain and hypotension or shock; exsanguination most commonly occurs into the pleural or pericardial space.
Additional signs include Horner syndrome due to compression of sympathetic ganglia, palpable downward pull of the trachea with each cardiac contraction (tracheal tug), and tracheal deviation. Visible or palpable chest wall pulsations, occasionally more prominent than the left ventricular apical impulse, are unusual but may occur.
Syphilitic aneurysms of the aortic root classically lead to aortic regurgitation and inflammatory stenosis of the coronary artery ostia, which may manifest as chest pain due to myocardial ischemia. Syphilitic aneurysms do not dissect.
TAAs are usually first suspected when a chest x-ray incidentally shows a widened mediastinum or enlargement of the aortic knob; however, chest x-ray has poor sensitivity for TAA and is not a reliable screening tool (eg, in patients with chest pain). These findings or symptoms and signs suggesting an aneurysm should be followed up with a 3-dimensional imaging test. CTA can delineate aneurysm size and proximal or distant extent, detect leakage, and identify coincident pathology. MRA may provide similar detail. Transthoracic echocardiography (TTE) can delineate size and extent and detect leakage of aneurysms of the ascending but not descending aorta; TEE can fully visualize the thoracic aorta and thus is especially useful for detecting aortic dissection. Contrast angiography provides the best image of the arterial lumen but no information on extraluminal structures, is invasive, and has a significant risk of renal and extremity atheroembolism and contrast nephropathy. Choice of imaging test is based on availability and local experience; however, if rupture is suspected, TEE or CTA, depending on availability, is done immediately.
Aortic root dilation or unexplained ascending aorta aneurysms warrant serologic testing for syphilis. If a mycotic aneurysm is suspected, bacterial and fungal blood cultures are done.
TAAs enlarge an average of 3 to 5 mm/yr; risk factors for rapid enlargement include larger size of aneurysm, location in the descending aorta, and presence of mural thrombi. Median diameter at aneurysm rupture is 6 cm for ascending aneurysms and 7 cm for descending aneurysms, but rupture of smaller aneurysms may occur in patients with Marfan syndrome. Survival rate of patients with untreated large TAAs is 65% at 1 yr and 20% at 5 yr.
Treatment is endovascular stent grafting when anatomically possible and open surgical repair for more complex aneurysms. Immediate control of hypertension is essential.
Ruptured TAAs, if untreated, are universally fatal; they require immediate intervention, as do leaking aneurysms and those that cause acute dissection or acute valvular regurgitation. Surgery involves a median sternotomy (for ascending and aortic arch aneurysms) or left thoracotomy (for descending and thoracoabdominal aneurysms) and subsequent excision of the aneurysm and replacement with a synthetic graft. Transcatheter-placed endovascular stent grafts (endografts) for descending TAAs are being used more frequently as a less invasive alternative to open surgery. With emergency surgery, 1-mo mortality rate is about 40 to 50%. In patients who survive, incidence of serious complications (eg, renal failure, respiratory failure, severe neurologic damage) is high.
Elective surgery is indicated for large aneurysms (diameter > 5 to 6 cm in the ascending aorta, > 6 to 7 cm in the descending aorta, and, for patients with Marfan syndrome, > 5 cm in any location) and also for aneurysms that rapidly enlarge (> 1 cm/yr). Elective surgery is also indicated for symptomatic, traumatic, or syphilitic aneurysms. For syphilitic aneurysms, benzathine penicillin 2.4 million units once/wk IM is given for 3 wk afterward. For patients allergic to penicillin, tetracycline or erythromycin 500 mg po qid for 30 days is acceptable.
Although surgical repair of an intact TAA improves outcome, mortality rate may still exceed 5 to 10% at 30 days and is 40 to 50% at 10 yr. Risk of death increases greatly if aneurysms are complicated (eg, in the aortic arch or thoracoabdominal aorta) or if patients have CAD, are older, are symptomatic, or have preexisting renal insufficiency. Perioperative complications (eg, stroke, spinal injury, renal failure) occur in about 10 to 20%.
Asymptomatic aneurysms that do not meet criteria for elective surgical or endovascular repair are treated with aggressive BP control using a β-blocker and other antihypertensives if necessary. Smoking cessation is essential. Patients require frequent follow-ups to check for symptoms and serial CT every 6 to 12 mo.
Last full review/revision May 2014 by John W. Hallett, Jr., MD
Content last modified May 2014