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Overview of acute aortic syndromes

Authors
Warren J Manning, MD
James H Black, III, MD
Section Editors
Gabriel S Aldea, MD
Edward Verrier, MD
Deputy Editor
Kathryn A Collins, MD, PhD, FACS

INTRODUCTION

Acute aortic syndromes include a spectrum of life-threatening aortic conditions. By convention, acute disease is distinguished from chronic disease at an arbitrary time point of two weeks from initial clinical presentation (<24 hours hyperacute, 1 to 14 days acute, 14 to 90 days subacute, and >90 days chronic) and is typically manifest with symptoms. Acute aortic dissection is the most familiar and is defined by a separation of the layers of the aortic wall by an inciting intimal injury. Intimal tear without hematoma, penetrating aortic ulcer, aortic intramural hematoma, and periaortic hematoma are variants of the classically described aortic dissection (figure 1) [1-3]. The changes associated with these are more localized, though they are similar to those of aortic dissection, with penetration and weakening of the aortic wall that predisposes to aortic rupture, which may manifest as a contained periaortic hematoma or free rupture into the chest or abdomen [2]. Each of these conditions can affect the thoracic aorta, abdominal aorta, and, in some cases, both.

An overview of the clinical features, classification, diagnosis, and approach to treatment of acute aortic syndromes including acute aortic dissection, which is also discussed in detail elsewhere, aortic intramural hematoma, and penetrating aortic ulcer are reviewed here. (See "Clinical features and diagnosis of acute aortic dissection" and "Management of acute aortic dissection".)

DEFINITION AND PATHOPHYSIOLOGY

Acute aortic dissection — Acute aortic dissection is defined as a separation of the layers of the aortic wall due to an intimal tear (figure 1). For spontaneous dissection, it is uncertain whether the initiating event is a primary rupture of the intima with secondary dissection of the media or primary hemorrhage within the media and subsequent rupture of the overlying intima [4]. The initial intimal tear can occur in the ascending aorta or descending aorta and occasionally can originate in the abdominal aorta. Blood at high pressure passes through the tear and separates the intima from the media and/or adventitia, creating a true lumen and a false lumen. The dissection can propagate proximally or distally from the initial tear to involve the aortic valve, coronary arteries, or branches of the thoracic or abdominal aorta [5]. Such propagation is responsible for many of the associated clinical features of aortic dissection (acute chest or back pain, neurologic symptoms). A higher mean pressure in the false lumen can cause dynamic or static compression and occlusion of the true lumen with malperfusion of the branches of the aorta, resulting in end-organ ischemia (coronary, cerebral, spinal, extremity, visceral) [6]. Aortic regurgitation, coronary ischemia, and cardiac tamponade can occur if the dissection progresses proximally to involve the aortic valve or the pericardial sac. In addition, multiple communications may form between the true lumen and the false lumen. (See "Clinical features and diagnosis of acute aortic dissection", section on 'Clinical features'.)

Abdominal aortic dissection can occur as an extension to a thoracic aortic dissection with the intimal flap located in the proximal or descending thoracic aorta, or it can occur in isolation [7-13]. Isolated abdominal aortic dissection is reported occasionally and can be due to spontaneous, iatrogenic, or traumatic mechanisms [14]. The infrarenal abdominal aorta is more commonly involved than the suprarenal aorta. In one review of 52 reported cases, the entry site for spontaneous isolated abdominal aortic dissections (SIAADs) most commonly occurred between the renal arteries and inferior mesenteric artery [15]. A concomitant abdominal aortic aneurysm was identified in 40 percent of patients and indicated the need for repair. (See "Management of asymptomatic abdominal aortic aneurysm".)

Intimal tear without hematoma — Intimal tear without hematoma (figure 1) is an uncommon variant of aortic dissection that is characterized by a stellate or linear intimal tear associated with exposure of the underlying aortic media or adventitia. There is no separation of the medial layers or progression [3].

                   

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