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Basic Information ⬇

AUTHORS: Cristina Font, MD and Philip Stockwell, MD

Definition

Acute aortic syndromes are a spectrum of aortic pathologies that include aortic dissection, intramural hematomas (IMHs), and penetrating atherosclerotic ulcers.1 Aortic dissections are the most common and occur when blood passes through an intimal tear, separating the intima from the medial layers and creating a false lumen.1 IMH and penetrating aortic ulcer are variants of the classically described aortic dissection. Fig. 1 illustrates acute aortic syndromes.

Figure 1 Acute aortic syndromes.

A, Classic aortic dissection. There is a tear in the intima with blood entering the media and a dissecting cleavage plane propagating for variable distances anterograde (and occasionally retrograde) throughout the aortic wall. B, Aortic intramural hematoma (IMH). A spontaneous hemorrhage of the vasa vasorum leads to bleeding within the media in the absence of an intimal tear or intimal flap. C, Penetrating atherosclerotic aortic ulcer (PAU). An ulcerated aortic plaque ruptures into the media, leading to an outpouching or ulceration in the aortic wall. This may be associated with IMH formation; pseudoaneurysm; or a focal, thick-walled aortic dissection. (From Zipes DP: Braunwald’s heart disease: a textbook of cardiovascular medicine, ed 11, Philadelphia, 2019, Elsevier.)

Classification

Aortic dissection is generally classified according to anatomic location (Fig. 2). Table 1 summarizes classification schemes of acute aortic dissection.

  • Stanford (more commonly used classification system): Type A ascending aorta (proximal), type B descending aorta (distal)
  • DeBakey: Type I ascending and descending aorta, type II ascending aorta, type III descending aorta
  • The Society of Thoracic Surgery and Society of Vascular Surgery have recently proposed a classification system of dissection subtypes to more precisely define the location of disease as extending between two anatomic zones2
  • Aortic dissection can also be classified by acuity of presentation: Hyperacute (<24 h), acute (2 to 7 days), subacute (8 to 30 days), and chronic (>30 days). The overall survival rate is inversely related to time of presentation, with the highest survival rate in the hyperacute group and the lowest survival rate in the chronic group

TABLE 1 Classification Schemes of Acute Aortic Dissection

DeBakey Classification
Type IOriginates in the ascending aorta and extends at least to the aortic arch and often to the descending aorta (and beyond)
Type IIOriginates in the ascending aorta and confined to this segment
Type IIIOriginates in the descending aorta, usually just distal to the left subclavian artery, and extends distally
Stanford Classification
Type ADissections involving the ascending aorta (with or without extension into the descending aorta)
Type BDissections not involving the ascending aorta

From Mann DL et al: Braunwald’s heart disease, ed 10, Philadelphia, 2015, Elsevier.

Figure 2 Classification schemes of acute aortic dissection.

From Mann DL et al: Braunwald’s heart disease, ed 10, Philadelphia, 2015, Elsevier.

Synonyms

  • Aortic dissection
  • Dissecting aortic aneurysm
  • AAS
ICD-10CM CODES
I71.00Dissection of unspecified site of aorta
I71.01Dissection of thoracic aorta
I71.02Dissection of abdominal aorta
I71.03Dissection of thoracoabdominal aorta
Epidemiology & Demographics
Incidence

2.6 to 3.5 per 100,000 person-yr; more frequent in winter as compared to other seasons, although no clear reason has been identified.3,4

Predominant Sex & Age

Males (65%) females (35%), ages 60 to 80 yr; mean = 63 yr1,3,5

Risk Factors: (Table 2)

  • Hypertension (found in up to 77% of patients with aortic dissection)3,5
  • Atherosclerosis (found in up to 27% of patients with aortic dissection)3,5
  • Preexisting aortic aneurysm (found in up to 16% of patients with aortic dissection)3,5
  • Age (60 to 80 yr)
  • Family history of aortic aneurysms/dissection
  • History of cardiac surgery, aortic valve replacement, intraaortic catheterization1,3,5
  • Disorders of collagen (Marfan syndrome, Ehlers-Danlos syndrome)3,5
  • Vascular inflammation (giant cell arteritis, Takayasu arteritis, rheumatoid arthritis, syphilitic aortitis)
  • Aortic coarctation, bicuspid aortic valve
  • Turner syndrome
  • Cocaine abuse (usually within 12 h of last use of cocaine)1,3,5
  • Trauma (∼20% road accident fatalities found to have ruptured aorta on autopsy)1
  • Pregnancy and delivery
  • Fluoroquinolone use1
  • Table 3 summarizes genetically triggered conditions associated with aortic dissection

TABLE 3 Genetically Triggered Conditions Associated with Aortic Dissection

Marfan syndrome (MFS)Autosomal dominant disorder of connective tissue caused by FBN1 mutation; incidence of 1 in ≈5000 individuals; multisystem manifestations, including ectopia lentis; mitral valve prolapse, aortic root aneurysm, aortic dissection; skeletal features (pectus deformities, scoliosis, arachnodactyly, hyperflexibility, tall stature, elongated fingers and toes); dural ectasia; spontaneous pneumothorax
Loeys-Dietz syndrome (LDS)Autosomal dominant disorder caused by mutations in TGFBR1 and TGFBR2, associated with aneurysms and dissections involving the aorta and branch vessels, often at relatively small diameters and young age; manifestations include craniofacial features (hypertelorism, craniosynostosis, cleft palate, bifid or broad uvula), bluish sclera, arterial tortuosity, velvety and hyperlucent skin, easily visible veins, clubfeet, skeletal abnormalities; phenotypes may vary, including those with more pronounced craniofacial features and those with more cutaneous features; ectopia lentis has not been described in LDS; mutations in TGFB2 lead to a syndrome with an overlap in clinical features of LDS and MFS
Familial thoracic aortic aneurysm (FTAA) syndromesAutosomal dominant disorders with variable expression and penetrance leading to thoracic aortic aneurysms (TAAs) and dissections at variable ages in families; ACTA2 mutations occur in 10%-15% of cases of FTAA and are associated with bicuspid aortic valve (BAV) disease, cerebral aneurysms, livedo reticularis, iris flocculi, PDA, moyamoya, and premature coronary artery disease; gene mutations causing familial thoracic aortic aneurysm and dissection (TAAD) include ACTA2, TGFBR1, TGFBR2, FBN1, MYH11, MYLK, TGFB2, SMAD3
Vascular Ehlers-Danlos syndrome (vEDS)Autosomal dominant disorder of collagen synthesis caused by a gene mutation in COL3A1 leading to rupture and dissection of the aorta (usually the descending and abdominal aorta) and branch vessels; manifestations include flexible digits, hyperlucent skin with visible veins, varicose veins, typical facial appearance, and spontaneous rupture of the uterus or bowel
Bicuspid aortic valve (BAV)Congenital condition affecting ≈1% of the population, familial in ≈9% of cases; often associated with dilation of the ascending aorta and carries increased risk for aortic dissection; gene mutations include NOTCH1 and loci at 15q, 18q, 5q, and 13q; may be associated with FTAA
Turner syndrome (TS)Genetic disorder affecting 1 in 2000 live-born girls and caused by complete or partial loss of the second sex chromosome (XO, Xp); women with TS often have BAV and aortic coarctation; associated with ascending aortic dilation for body size and increased risk for aortic dissection, especially when associated with BAV, hypertension, and coarctation
Aneurysms-osteoarthritis syndromeAutosomal dominant genetic disorder resulting from mutations in the SMAD3 gene and associated with premature osteoarthritis, osteochondritis dissecans, skeletal features, aortic aneurysms, branch vessel aneurysms, and arterial tortuosity; overlap with LDS phenotype

From Mann DL et al: Braunwald’s heart disease, ed 10, Philadelphia, 2015, Elsevier.

TABLE 2 Risk Factors for Aortic Dissection

  • Hypertension
  • Heritable or genetic thoracic aortic disease and syndromes
    • Marfan syndrome
    • Loeys-Dietz syndrome
    • Familial thoracic aortic aneurysm syndromes
    • Vascular Ehlers-Danlos syndrome
    • Turner syndrome
  • Congenital diseases/syndromes
    • Bicuspid aortic valve
    • Coarctation of the aorta
    • Tetralogy of Fallot
  • Atherosclerosis
    • Penetrating atherosclerotic ulcer
  • Trauma, blunt or iatrogenic
    • Catheter/guidewire
    • Intra-aortic balloon pump
    • Aortic/vascular surgery
    • Motor vehicle accident
    • Coronary artery bypass grafting/aortic valve replacement/TAVR
    • Thoracic endovascular aneurysm repair (TEVAR)
  • Cocaine/methamphetamine use
  • Inflammatory/infectious diseases
    • Giant cell arteritis
    • Takayasu arteritis
    • Behçet syndrome
    • Aortitis
    • Syphilis
  • Pregnancy (with underlying aortopathy)
  • Weightlifting (with underlying aortopathy)

TAVR, Transcatheter aortic valve replacement.

From Zipes DP: Braunwald’s heart disease: a textbook of cardiovascular medicine, ed 11, Philadelphia, 2019, Elsevier.

Physical Findings & Clinical Presentation

  • Sudden onset of severe sharp, tearing, or ripping chest, back, or abdominal pain.1,3,5 However, painless dissection occurs in ∼6.3% of cases2
  • Anterior chest pain (79% type A, 63% type B)3
  • Back pain, abdominal pain (43% type A, 64% type B)3
  • Syncope (19% type A, 3% type B), generally secondary to cardiac tamponade or stroke3
  • Congestive heart failure (CHF)1
  • May present with hypertension (28% for type A, 66% in type B dissection),5 although 25% present with hypotension (systolic blood pressure <100 mm Hg), which can indicate bleeding, cardiac tamponade, or severe aortic regurgitation.3 Patients presenting with very high systolic blood pressure (>180 mm Hg for type A, >200 mm Hg for type B dissection), as well as in those with systolic blood pressure ≤100 mm Hg, have significantly higher in-hospital mortality rates
  • Pulse and blood pressure differentials (>20 mm Hg between arms) in 19% to 31% of cases caused by partial compression of subclavian arteries6
  • Aortic regurgitation in 40% to 75% of cases of proximal dissection,1 often with diastolic decrescendo murmur
  • Myocardial ischemia caused by coronary artery occlusion, most commonly involving the right coronary artery
  • Stroke in 5% to 10% of patients (secondary to dissection into or decreased blood flow to the carotids)1
  • Mesenteric ischemia occurs in 3% to 5% of cases, with external compression, flap prolapse, or involvement of arterial ostia1
  • Horner syndrome (ptosis, miosis, anhidrosis)
  • Vocal cord paralysis or hoarse voice (caused by compression of the left recurrent laryngeal nerve)1
Etiology

Genetics, in addition to other risk factors (Tables 4, 5, 6, and 7), contribute to the development of aortic aneurysms and dissection.

TABLE 7 Crawford Classification of Thoracoabdominal Aneurysms

IDescending thoracic aorta to suprarenal aorta
IIProximal descending thoracic aorta to infrarenal aorta (below the diaphragm)
IIIMid descending thoracic aorta to infrarenal aorta
IVSupravisceral aorta to infrarenal aorta

From Soto JA, Lucey BC: Emergency radiology: the requisites, ed 2, Philadelphia, 2017, Elsevier.

TABLE 6 Descending Thoracic Aortic Aneurysm

DegenerativeResulting from atherosclerosis
Focal pseudoaneurysm resulting from a penetrating aortic ulcer
ArteritisGiant cell
Takayasu
Behçet disease
Inherited/connective tissueMarfan syndrome
Ductus aneurysm
Loeys-Dietz syndrome
InfectiousMycotic aneurysms
TraumaticPosttraumatic pseudoaneurysms
Chronic aortic transection

From Soto JA, Lucey BC: Emergency radiology: the requisites, ed 2, Philadelphia, 2017, Elsevier.

TABLE 5 Aortic Arch Aneurysms

DegenerativeResulting from Atherosclerosis
ArteritisGiant cell
Takayasu
Behçet disease
InfectiousSyphilis

From Soto JA, Lucey BC: Emergency radiology: the requisites, ed 2, Philadelphia, 2017, Elsevier.

TABLE 4 Ascending Thoracic Aortic Aneurysms

DegenerativeAssociated with Atherosclerosis
Inheritable/connective tissueMarfan syndrome
Ehlers-Danlos syndrome
Loeys-Dietz syndrome
Turner syndrome
Osteogenesis imperfecta
Rheumatoid arthritis
Bicuspid aortic valve
Aneurysm of sinus of Valsalva
ArteritisGiant cell
Takayasu
Behçet disease
Relapsing polychondritis
InfectiousSyphilis
Mycotic aneurysms

From Soto JA, Lucey BC: Emergency radiology: the requisites, ed 2, Philadelphia, 2017, Elsevier.

Diagnosis ⬆ ⬇

Differential Diagnosis

  • Known as the great imitator: Pulmonary embolism, acute coronary syndrome, aortic stenosis/insufficiency, nondissecting aneurysm, pericarditis, cholecystitis, peptic ulcer disease, pancreatitis, musculoskeletal pain.3
  • Consider aortic dissection in patients with unexplained stroke, chest pain, syncope, acute-onset CHF, abdominal pain, back pain, and malperfusion of extremities or internal organs.3 Acute aortic syndromes may be associated with nonspecific signs and symptoms; a high clinical index of suspicion is necessary to detect the disease early in its course as nearly two thirds of patients under medical care are not diagnosed before death. Box E1 summarizes a differential diagnosis of aortic dissection.
  • In an emergency situation, a rapid yet comprehensive workup is crucial to reduce diagnostic time delay. This should include clinical assessment, laboratory data (D-dimer and troponin), chest x-ray, ECG, and aortic imaging in the appropriate patient.1,3,5
  • The Aortic Dissection Detection Risk Score (ADD-RS) can be a useful tool in diagnosing aortic dissection in the emergency room setting.6 ADD-RS is based on the presence of one or more of the clinical risk markers outlined in Table 8. A high ADD-RS effectively stratifies the risk for acute aortic dissection (score 0 is low risk, score 1 is intermediate risk, and score ≥2 is high risk).6 Score 0 has 95.6% certainty in ruling out aortic dissection.
  • The addition of D-dimer to ADD-RS may further improve diagnostic performance of each of these when used alone for ruling out acute aortic dissection or other acute aortic syndromes.6 Among low- to intermediate-risk patients (ADD-RS <2), a negative D-dimer (<500 mg/dl) can rule out aortic dissection with 99.7% certainty.6 Among high-risk patients (ADD-RS ≥2), D-dimer is not discriminatory and requires conclusive imaging.6

TABLE 8 Aortic Dissection Detection Risk Score (ADD-RS)

Clinical Risk Markers∗Score
High-risk condition such as Marfan syndrome, family history of aortic disease, known aortic valve disease, known thoracic aortic aneurysm, or previous aortic manipulation, including cardiac surgery1
Pain in the chest, back, or abdomen described as abrupt, of severe intensity, or a ripping/tearing sensation1
Physical examination findings of perfusion deficit, including pulse deficit, systolic blood pressure difference, or focal neurologic deficit, or with aortic diastolic murmur and hypotension/shock1

∗Presence of ≥1 marker within each of these groups is given a score of 1 with a maximum cumulative score of 3 if all three are present.

Modified from Erbel R et al: 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC), Eur Heart J 35:2873-2926, 2014; Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease, Circulation 121:e266-369, 2010; and Hiratzka et al: Surgery for aortic dilatation in patients with bicuspid aortic valves: a statement of clarification from the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines, J Am Coll Cardiol 67:724-731, 2016.

Box E1 Differential Diagnosis of Aortic Dissection

  • Cardiovascular
    • Acute coronary syndrome with or without ST-segment elevation
    • Shock
    • Acute pulmonary edema
    • Acute valvular dysfunction
    • Pericarditis
    • Acute extremity ischemia
  • Pulmonary
    • Pulmonary embolus
    • Pneumothorax
  • Gastrointestinal
    • Pancreatitis
    • Peptic ulcer disease (including perforation)
    • Esophageal spasm/reflux
    • Ischemic bowel
  • Neurologic
    • Stroke/transient ischemic attack
    • Spinal cord compression
  • Renal
    • Renal colic

From Cameron P et al: Textbook of adult emergency medicine, ed 5, Australia, 2019, Elsevier.

Workup

ECG: Helpful to rule out myocardial infarction, although dissection can lead to coronary ischemia.1,3,5

Laboratory Tests

  • D-Dimer has a high negative predictive value in dissection, but lacks specificity in the setting of acute aortic dissection.1,3 However, a negative D-dimer does not rule out IMH or penetrating aortic ulcer.1,3
  • Soluble ST2, a novel biomarker, may have superior diagnostic performance over D-dimer in discriminating acute aortic dissection from other acute chest pain syndromes.7 At a cutoff level of 34.6 ng/ml, the sensitivity and specificity for acute aortic dissection were 99.1% and 84.9%, respectively.7
  • Several other biomarkers with different diagnostic windows can be used in the diagnosis of aortic dissection1,8:
    1. Smooth muscle myosin heavy chain protein (released from damaged medial smooth muscle) can be used to detect proximal aortic dissections (91% sensitivity and 93% specificity). Myosin heavy chains will peak within 3 h of dissection and clear within 24 h of aortic injury.
    2. CK-BB isoenzyme also peaks within 6 h of dissection.
    3. Calponin, a smooth muscle troponin counterpart, increases in aortic dissection with a wider diagnostic window when compared to smooth muscle myosin heavy chain and CK-BB.
    4. C-reactive protein, fibrinogen and soluble elastin fragments are under investigation.
Imaging Studies

  • Multidetector CT (Fig. E3) is considered the gold standard, but its use may be limited in patients with renal failure as it involves the use of intravenous contrast.1,3,5
  • Transesophageal echocardiography (TEE), multidetector CT, and MRI are all highly sensitive (98% to 100%) and specific (95% to 98%).9 Test of choice depends on clinical circumstances and hospital availability.
  • TEE is study of choice in unstable patients with type A dissection but is operator dependent.1
  • MRI has high sensitivity and specificity but limited availability; not suitable for unstable patients; contraindicated with noncompatible pacemakers, metal devices; uses gadolinium-based contrast which is less nephrotoxic than iodinated agents in patients without severe renal dysfunction.1
  • With medium or high pretest probability, a second diagnostic test should be done if the first is negative.
  • Coronary computed tomographic angiography (CTA) may be an alternative and useful diagnostic study when evaluating for pulmonary embolism, acute coronary syndrome, and aortic dissection.1
  • Aortography rarely done, as less sensitive than TEE, CT, or MRI.1
  • Chest radiograph may show widened mediastinum (52% in type A dissections and 39% in type B dissections) and displacement of aortic intimal calcium.4 It is normal in 29% to 36% of patients with aortic dissection.
  • Although the diagnostic sensitivity of transthoracic echocardiography is suboptimal (31% to 55%), it is useful in assessing potential high-risk features or complications, such as pericardial effusion and cardiac tamponade, and in making other potential diagnoses. A negative transthoracic echocardiography, however, does not exclude aortic dissection.1

Figure E3 Contrast-Enhanced Computed Tomography Scan Demonstrating Acute Type A Aortic Dissection with Enlargement of the Ascending Aorta and Intimal Flaps (Arrows) in the Ascending and Descending Aorta

Both the True Lumen (Tl) and the False Lumen are Opacified with Contrast Material in This Example.

From Mann DL et al: Braunwald’s heart disease, ed 10, Philadelphia, 2015, Elsevier.

Treatment ⬆ ⬇

TABLE 10 Indications for Thoracic Endovascular Aortic Repair for Type B Aortic Dissection∗

  • Rupture
  • Impending rupture
  • Malperfusion
  • Hemorrhagic pleural effusion
  • Refractory pain
  • Refractory hypertension
  • Aneurysmal dilation (>55 mm) Rapid increase in diameter Recurrent symptoms

∗Or open surgical repair if anatomy is unsuitable for TEVAR.

From Zipes DP: Braunwald’s heart disease: a textbook of cardiovascular medicine, ed 11, Philadelphia, 2019, Elsevier.

TABLE 9 Size Threshold for Prophylactic Aortic Root or Ascending Aortic Aneurysm Resection for Various Conditions

ConditionSize Threshold∗
Degenerative aneurysm≥5.5 cm
Bicuspid aortic valve≥5.5 cm
Bicuspid aortic valve with risk factors or low surgical risk†≥5.0 cm
Bicuspid aortic valve requiring aortic valve replacement>4.5 cm
Marfan syndrome≥5.0 cm
Marfan syndrome with risk factors‡>4.5 cm
Loeys-Dietz syndrome§4.0-4.5 cm
Familial thoracic aortic aneurysm syndromes4.5-5.0 cm
Turner syndrome>2.5 cm/m2

∗Lower thresholds for intervention may be considered according to body surface area in patients of small stature or in the case of rapid growth of the aorta. Age, body size, rapid growth, family history, risk of surgery, and patient and physician wishes may influence aortic size threshold.

† Family history of aortic dissection or aortic growth rate of ≥0.5 cm/yr or if the patient is at low surgical risk (<4%) and the surgery is performed by an experienced aortic surgical team in a center with established expertise in these procedures. Other risk factors for aortic dissection include coarctation of the aorta, hypertension, and the root phenotype of bicuspid aortic valve.

‡ Family history of aortic dissection or rapid aortic growth (>3 mm/yr), or severe aortic or mitral regurgitation. If pregnancy desired, consider prophylactic aortic surgery for aortic diameter of 4.0-4.5 cm.

§ It is reasonable to consider surgical repair of the aorta in adults with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation with aortic diameter of 4.2 cm or more by transesophageal echocardiogram or 4.4-4.6 or more by CT or MRI. Aortic surgery at smaller diameters may be recommended when there are severe craniofacial features, rapid growth, or a family history of aortic dissection.

Surgical thresholds vary depending on the specific gene mutation involved. TAA caused by ACTA2, SMAD3, and MYLK may lead to aortic dissection at relatively small aortic diameters.

From Zipes DP: Braunwald’s heart disease: a textbook of cardiovascular medicine, ed 11, Philadelphia, 2019, Elsevier.

Acute General Rx

  • Admit to ICU for monitoring.
  • Target systolic blood pressure 100 to 120 mm Hg; heart rate <60 beats/min to reduce aortic wall stress.1,8 Treatment with beta-blockers has been associated with improved survival in all patients with acute aortic dissections.
  • IV beta-blockers are cornerstones of treatment, but multiple medications may be needed.
    1. Propranolol 1 mg every 3 to 5 min, metoprolol 5 mg IV every 5 min, or labetalol 20 mg IV, then 20 to 80 mg every 10 min, followed by nitroprusside 0.3 to 10 mcg/kg/min.8
    2. Vasodilators should not be used without beta-blockade as they can induce reflex sympathetic stimulation and increase aortic shear stress.8
    3. IV calcium channel blockers with negative inotropy (i.e., verapamil, diltiazem) may be used if beta-blockers are contraindicated.8
  • Pain control, often with morphine.8
Chronic Rx

  • Chronic aortic dissection (>2 wk) managed with aggressive blood pressure control; target <130/80 mm Hg in most patients1,8
  • Statin therapy to reduce low-density lipoprotein <70 mg/dl8
  • Tobacco cessation8
  • Minimize strenuous physical activity such as heavy lifting8
  • Serial imaging of the aorta, with multidetector CT or MRI should be performed at presentation, at 1, 3, 6, and 12 mo given the higher risk of instability early on, followed by yearly clinical and imaging follow-up8
  • As stated above, endovascular repair should be considered in complicated chronic type B dissections, that is, when the aortic diameter exceeds 5.5 cm, when there is uncontrolled pain or blood pressure, or when there is rapid growth of the dissecting aneurysm (>4 mm per yr)8
Disposition

  • 90% mortality rate is within 2 wk for an untreated type A dissection.
  • Proximal dissection is a surgical emergency. Time is critical; mortality rate is 1% to 3%/h, approaching 70% after 48 h.1,8
  • Overall, in-hospital mortality rate is 22% with proximal dissections (27% treated surgically and 56% treated medically) and 13% with distal dissections.3
  • Table 11 summarizes suggested imaging surveillance of asymptomatic thoracic aortic aneurysms.

TABLE 11 Suggested Imaging Surveillance of Asymptomatic Thoracic Aortic Aneurysms∗

Initial Discovery of AneurysmRepeated Imaging at 6 mo to Document Stability
Degenerative Aneurysm†
3.5-4.4 cmAnnual imaging
4.5-5.4 cmAnnual to biannual imaging
MFS, BAV with TAA, and Familial TAA
3.5-4.4 cmAnnual imaging
4.5-5.0 cmBiannual imaging
LDS‡
<4 cmAt least annual imaging
>4 cmBiannual imaging

BAV, Bicuspid aortic valve; LDS, Loeys-Dietz syndrome; MFS, Marfan syndrome; TAA, thoracic aortic aneurysm.

∗For aneurysms growing rapidly, more frequent imaging is recommended. Management of TAA must take into account the family history, age, body size, sex, rate of aneurysm growth, and underlying disease.

† For relatively small degenerative aneurysms found by imaging to be stable from year to year, imaging may be performed every 2-3 yr (Hiratzka et al).

‡ Some recommend surgery for aortic root dimensions larger than 4 cm in adults with LDS, whereas the American College of Cardiology/American Heart Association guidelines for thoracic aortic disease recommend prophylactic surgery at 4.2 cm by TEE and 4.4-4.6 cm by CT or MRI (Hiratzka et al).

From Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine, Circulation 121:e266, 2010; Mann DL et al: Braunwald’s heart disease, ed 10, Philadelphia, 2015, Elsevier.

Referral

For ICU management and surgical intervention

Pearls & Considerations ⬆ ⬇

Other Acute Aortic Syndromes
Acute Intramural Hematoma

  • Acute IMH occurs when the vasa vasorum ruptures within the medial wall. It does not involve an intimal tearing unless a dissection develops.
  • About 10% to 20% of patients with suspected aortic dissection will be found to have acute IMH.8
  • The key imaging findings are the absence of intimal tear and absence of blood flow into a false lumen.
  • Echocardiography shows crescentic or circumferential thickening of aortic wall without an intimal flap.
  • About 8% to 16% of patients with IMH will evolve into aortic dissection.3
  • Management remains controversial, but in the ascending aorta, IMH is generally treated with urgent surgical repair.
  • Similar to aortic dissections, in-hospital mortality is significantly worse among patients with type A IMH compared to type B.3
Penetrating Atherosclerotic Ulcer

  • Penetrating atherosclerotic ulcers occur in the setting of extensive aortic atherosclerosis and hypertension.8
  • These are seen mostly in older adults and occurs in descending aorta in over 90% of cases.8
  • There is an ulceration of atheroma that disrupts the internal elastic lamina and extends into the media and allows hematoma formation.8
  • They should be treated promptly, because there is potential for extensive IMH, pseudoaneurysm or complete rupture of aorta.8

The general principles of the treatment of acute IMHs and penetrating atherosclerotic ulcers are similar to acute aortic dissection.8

Related Content

Aortic Dissection (Patient Information)

Related Content ⬆

    1. Erbel R. : ESC guidelines on the diagnosis and treatment of aortic diseasesEur Heart J. ;35(41):2873-2926, 2014.
    2. Lombardi J. : Society for Vascular Surgery (SVS) and Society of Thoracic Surgeons (STS) reporting standards for type B aortic dissectionsJ Vasc Surg. ;71(3):723-747, 2020.
    3. Evangelista A. : Insights from the International Registry of Acute Aortic Dissection: a 20-year experience of collaborative clinical researchCirculation. ;137(17):1846-1860, 2018.doi:10.1161/CIRCULATIONAHA.117.031264
    4. Takagi H. : Meta-analysis of seasonal incidence of aortic dissectionAm J Cardiol. ;120, 2017.
    5. Pape L.A. : Presentation, diagnosis, and outcomes of acute aortic dissection: 17-year trends from the International Registry of Acute Aortic DissectionJ Am Coll Cardiol. ;66:350-358, 2015.
    6. Nazerian P. : Diagnostic accuracy of the aortic dissection detection risk score plus D-dimer for acute aortic syndromes: The ADvISED Prospective Multicenter StudyCirculation. ;137:250-258, 2018.doi:10.1161/CIRCULATIONAHA.117.029457
    7. Wang Y. : Magnitude of soluble ST2 as a novel biomarker for acute aortic dissectionCirculation. ;137:259-269, 2018.doi:10.1161/CIRCULATIONAHA.117.030469
    8. Hiratzka L.F. : 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular MedicineCirculation. ;121:e266-e369, 2010.
    9. Fukui T. : Management of acute aortic dissection and thoracic aortic ruptureJ Intensive Car. ;6(15), 2018.doi:10.1186/s40560-018-0287-7
    10. Song J.M. : Long-term predictors of descending aorta aneurysmal change in patients with aortic dissectionJ Am Coll Cardiol. ;50:799-804, 2007.
    11. Fattori R. : Interdisciplinary expert consensus document on management of type B aortic dissectionJ Am Coll Cardiol. ;61(16):1661-1678, 2013.