Aortic dissection risk factors

Aortic dissection Microchapters
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Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Raviteja Guddeti, M.B.B.S. [3]

Overview

Aging, atherosclerosis, diabetes, hypertension and trauma are common risk factors for aortic dissection. Uncommon risk factors include bicuspid aortic valve, cocaine, coarctation of the aorta, cystic medial necrosis, Ehlers-Danlos syndrome, giant cell arteritis, heart surgery, Marfan’s syndrome, pseudoxanthoma elasticum, Turner's syndrome, tertiary syphilis and the third trimester of pregnancy.

Risk Factors

• Aging. The highest incidence of aortic dissection is in individuals who are 50 to 70 years old.
• Atherosclerosis and its associated risk factors like diabetes
• Bicuspid aortic valve is present in approximately 7%-14% of patients. 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.
• Chest trauma. Chest trauma leading to aortic dissection can be divided into two groups based on etiology: blunt chest trauma (commonly seen in car accidents) and iatrogenic. Iatrogenic causes include trauma during cardiac catheterization or due to an intra-aortic balloon pump.
• Coarctation of the aorta
• Cocaine abuse
• Cystic medial necrosis
• Deceleration trauma most commonly causes aortic rupture, not dissection
• Diabetes
• Ehlers-Danlos syndrome
• Familial hypercholesterolemia
• Giant cell arteritis
• Heart surgery particularly aortic valve replacement; 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.
• Hypertension is seen in 71-86% of patients. It occurs most frequently in those with type III dissection.
• Male gender. The incidence is twice as high in males as in females (male-to-female ratio is 2:1).
• Marfan’s syndrome is present in 5%-9% of patients. In this subset, there is an increased incidence in young individuals. Individuals with Marfan syndrome patients are more prone to proximal dissections of the aorta.
• Preexisting aortic aneurysm
• Preexisting aortic valve disease
• Prior aortic aneurysm repair
• Prior aortic dissection
• Prior aortic dissection repair
• Pseudoxanthoma elasticum
• Tertiary syphilis
• Tetralogy of Fallot
• Third trimester of pregnancy. Half of dissections in females before age 40 occur during pregnancy (typically in the 3rd trimester or early postpartum period).
• Turner's syndrome. Turner syndrome increases the risk of aortic dissection as a result of aortic root dilatation.
• Vasculitis (inflammation of an artery) is rarely associated with aortic dissection.

2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease (DO NOT EDIT)

Estimation of Pretest Risk of Thoracic Aortic Dissection (DO NOT EDIT)

Class I

"1. Providers should routinely evaluate any patient presenting with complaints that may represent acute thoracic aortic dissection to establish a pretest risk of disease that can then be used to guide diagnostic decisions. This process should include specific questions about medical history, family history, and pain features as well as a focused examination to identify findings that are associated with aortic dissection, including:

a. High-risk conditions and historical features (Level of Evidence: B): Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Turner syndrome, or other connective tissue disease. Patients with mutations in genes known to predispose to thoracic aortic aneurysms and dissection, such as FBN1, TGFBR1, TGFBR2, ACTA2, and MYH11. Family history of aortic dissection or thoracic aortic aneurysm. Known aortic valve disease. Recent aortic manipulation (surgical or catheter-based). Known thoracic aortic aneurysm.

b. High-risk chest, back, or abdominal pain features (Level of Evidence: B): Pain that is abrupt or instantaneous in onset. Pain that is severe in intensity. Pain that has a ripping, tearing, stabbing, or sharp quality.

c. High-risk examination features (Level of Evidence: B): Pulse deficit. Systolic blood pressure limb differential greater than 20 mm Hg. Focal neurologic deficit. Murmur of aortic regurgitation (new)."

"2. Patients presenting with sudden onset of severe chest, back, and/or abdominal pain, particularly those less than 40 years of age, should be questioned about a history and examined for physical features of Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Turner syndrome, or other connective tissue disorders associated with thoracic aortic disease. (Level of Evidence: B)"

"3. Patients presenting with sudden onset of severe chest, back, and/or abdominal pain should be questioned about a history of aortic pathology in immediate family members as there is a strong familial component to acute thoracic aortic disease. (Level of Evidence: B)"

"4. Patients presenting with sudden onset of severe chest, back, and/or abdominal pain should be questioned about recent aortic manipulation (surgical or catheter-based) or a known history of aortic valvular disease, as these factors predispose to acute aortic dissection. (Level of Evidence: C)"

"5. In patients with suspected or confirmed aortic dissection who have experienced a syncopal episode, a focused examination should be performed to identify associated neurologic injury or the presence of pericardial tamponade. (Level of Evidence: C)"

"6. All patients presenting with acute neurologic complaints should be questioned about the presence of chest, back, and/or abdominal pain and checked for peripheral pulse deficits as patients with dissection related neurologic pathology are less likely to report thoracic pain than the typical aortic dissection patient. (Level of Evidence: C)"