Acute aortic syndromes
Abstract: Acute aortic syndromes include aortic dissection, aortic intramural hematoma and penetrating ulcer of the aorta. They are an important differential diagnosis of acute coronary syndrome.
Acute aortic syndromes include aortic dissection, intramural hematoma of the aorta and penetrating ulcer of the aorta. The concept of acute aortic syndrome was introduced by Vilacosta I et al in an editorial which appeared in Heart in 2001.1 The nature and location of pain are important in the diagnosis of acute aortic syndrome. Usually it is an intense tearing type of pain and the location may change as the disease progresses as in aortic dissection. Pain in the front of the chest, neck or throat suggests ascending aortic involvement while pain in the back or over the abdomen suggests involvement of the descending thoracic or abdominal aorta.2 Most of the acute aortic syndromes are associated with significant hypertension, though aortic dissection may have a background of diseases like Marfan syndrome.
Aortic dissection is by far the most well known of the acute aortic syndromes. In the Stanford classification of aortic dissections, type A involves the ascending aorta while type B does not involve the ascending aorta.3 In DeBakey classification, type I involves the ascending aorta, aortic arch and the descending aorta. DeBakey type II aortic dissection involves only the ascending aorta while DeBakey type II is confined to the descending aorta.4
Aortic dissection can extend either anterogradely or retrogradely and involve the side branches. Myocardial infarction can result if the coronary ostium (usually right coronary) is involved. Aortic regurgitation is another aftermath of ascending aortic dissection. Dissection can also lead to hemopericardium with cardiac tamponade. Progression may stop if there is a spontaneous re-entry into the lumen. Similarly, in the long term the thrombosis of the false lumen can lead to good result.
Aortic intramural hematoma
Aortic intramural hematoma is usually the result of rupture of a vasa vasorum in the aortic media. It can lead on to aortic dissection if the hematoma ruptures. Just like an aortic dissection, the aortic intramural hematoma can also extend along the length of the aorta either anterogradely or retrogradely. Ascending aortic intramural hematoma will present with chest pain while descending aortic hematoma may present with back pain or abdominal pain.5 It is not possible to differentiate aortic intramural hematoma from aortic dissection clinically as it needs imaging to confirm and exclude dissection which has similar symptomatology.
Penetrating ulcer of the aorta
Ulceration can occur in atheroclerotic plaques of the aorta. These can lead on to aortic dissection or intramural hematoma.6 These ulcers can deepen and result in aortic perforation with catastrophic results. Penetrating ulcers, like intramural hematoma, need imaging studies for documentation. More and more cases of penetrating ulcers of the aorta are being documented with the widespread use of multi slice computerized tomographic aortograms.
- Vilacosta I, Román JA. Acute aortic syndrome. Heart. 2001 Apr;85(4):365-8.
- Wooley CF, Sparks EH, Boudoulas H. Aortic pain. Prog Cardiovasc Dis. 1998;40:563-89.
- Daily PO, Trueblood HW, Stinson EB, Wuerflein RD, Shumway NE. Management of acute aortic dissections. Ann Thorac Surg. 1970; 10: 237-247.
- DeBakey ME, Beall AC Jr, Cooley DA, Crawford ES, Morris GC Jr, Garrett HE, Howell JF. Dissecting aneurysms of the aorta. Surg Clin North Am. 1966; 46: 1045-1055.
- Maraj R, Rerkpattanapipat P, Jacobs LE, Makornwattana P, Kotler MN. Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol. 2000; 86: 664-668.
- Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H, Mitchell RS, Dake MD. Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. Circulation. 2002; 106: 342-348.