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Review

Thoracic aortic aneurysm: How to counsel, when to refer

Frank Cikach, MD, Milind Y. Desai, MD, FACC, FAHA, FESC, Eric E. Roselli, MD, FACS and Vidyasagar Kalahasti, MD
Cleveland Clinic Journal of Medicine June 2018, 85 (6) 481-492; DOI: https://doi.org/10.3949/ccjm.85a.17039
Frank Cikach
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH
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Milind Y. Desai
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Eric E. Roselli
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Vidyasagar Kalahasti
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    Figure 2

    Echocardiographic image of an aneurysmal aortic root (white arrow) that tapers to normal dimensions at the sinotubular junction (yellow arrow) and ascending aorta. See videos (this image and a bicuspid aortic valve).

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    Figure 3

    An aortic root aneurysm in a patient with Mar-fan syndrome using magnetic resonance angiography. See videos (this image, follow-up after surgical repair, and a computed tomographic study).

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    Figure 4

    Top, 3D reconstruction of an ascending aortic aneurysm in a patient with a bicuspid aortic valve. See video. Bottom, 3D reconstruction of a young patient with an aortic root aneurysm. Note the tapering from the aneurysmal aortic root to the normalsized ascending aorta. LMCA = left main coronary artery; RCA = right coronary artery.

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    Figure 5

    Initial screening and follow-up of thoracic aortic aneurysm.

Tables

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    TABLE 1

    Aortic diameters: Upper limits of normala

    Age (years)BSA (m2)Ascending aorta (mm) Descending aorta (mm)
    Women (n = 1,147)Men (n = 1,805)Women (n = 736)Men (n = 1,195)
    < 45< 1.7033.833.023.0NA
    1.70–1.8934.436.324.626.6
    1.90–2.0935.036.322.726.7
    > 2.1NA38.3NA28.3
    45–54< 1.7035.238.624.324.2
    1.70–1.8937.238.125.427.5
    1.90–2.0938.939.727.229.2
    > 2.140.640.628.329.6
    55–64< 1.7036.936.325.926.1
    1.70–1.8937.039.727.128.6
    1.90–2.0939.041.227.829.9
    > 2.142.043.131.731.6
    ≥ 65< 1.7037.538.527.0NA
    1.70–1.8939.241.027.432.4
    1.90–2.0942.742.229.031.0
    > 2.1NA42.429.832.5
    • ↵a Upper limits of normal are 2 standard deviations above the mean. Not calculated if there were fewer than 6 patients in a group. BSA = body surface area; NA = not available

    • Information from Wolak A, Gransar H, Thomson LE, et al. Aortic size assessment by noncontrast cardiac computed tomography: normal limits by age, gender, and body surface area. J Am Coll Cardiol Cardiovasc Imaging 2008; 1(2):200–209. doi:10.1016/j.jcmg.2007.11.005

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    TABLE 2

    Common causes of thoracic aortic aneurysm

    Idiopathic
    Bicuspid aortic valve
    Genetic syndromes
    Marfan syndrome
    Loeys-Dietz syndrome
    Ehlers-Danlos syndrome
    Familial thoracic aortic aneurysm and dissection
    Autosomal dominant polycystic kidney disease
    Turner syndrome
    Inflammatory vasculitis
    Takayasu arteritis
    Giant cell arteritis
    Behçet arteritis
    Ankylosing spondylitis
    Other
    Atherosclerosis
    Syphilis
    Previous dissection
    Infectious aortitis
    Trauma
    Risk factors
    Hypertension
    Smoking
    Advanced age
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    TABLE 3

    Inherited connective tissue diseases and thoracic aortic aneurysm

    Inherited conditionImplicated geneClinical features
    Marfan syndromeFBN1Aneurysm of aortic root, dilation of pulmonary artery, and aortic dissection
    Ehlers-Danlos syndromeCOL5A1, COL5A2, COL3A1Arterial mid-sized rupture, especially involving thoracic vasculature
    Loeys-Dietz syndromeTGFBR1, TGFBR2, SMAD3, TGFB2, TGFB3Premature and aggressive aneurysm and dissection; aneurysm may involve aortic segments other than the root
    Familial thoracic aortic aneurysm and dissectionACTA2, MYH11, PRKG1, MYLK, TGFBR2Thoracic aortic aneurysm and dissection; associated vascular disease (eg, patent ductus arteriosus)
    Bicuspid aortic valveUnknown (may be associated with ACTA2, MYH11, syndromic connective tissue diseases)Aortic dilation typically involving the aortic root and ascending aorta
    Autosomal dominant polycystic kidney diseasePKD1, PKD2Dilation of the aorta and thoracic aortic dissection
    Turner syndrome45,XThoracic aortic aneurysms and dissections, bicuspid aortic valve, aortic coarctation
    • Reprinted from Cury M, Zeidan F, Lobato AC. Aortic disease in the young: genetic aneurysm syndromes, connective tissue disorders, and familial aortic aneurysms and dissections. Int J Vasc Med 2013(2013); 2013:267215. doi:10.1155/2013/267215

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    TABLE 4

    Imaging studies for aortic aneurysm

    Imaging studyAdvantagesDisadvantages
    Cardiac-gated multidetector computed tomographic angiographyHighly specific and sensitive Can diagnose vascular anomalies, tortuosityLarge doses of ionizing radiation and contrast
    Magnetic resonance angiographyHighly specific and sensitive Can be accurate without using contrast Ability to assess valve diseaseProlonged scanning time Difficult for those with claustrophobia
    Transthoracic echocardiographyNoninvasive, readily available Can assess valve diseaseLimited visualization of ascending aorta Accuracy reduced with “oblique” measurements
    Transesophageal echocardiographyHighly specific and sensitive Ability to assess valve diseaseRequires skilled personnel Invasive, requires procedural sedation
    AngiographyHighly sensitive and specificInvasive Requires contrast
    • Based on Smith AD, Schoenhagen P. CT imaging for acute aortic syndrome. Cleve Clin J Med 2008; 75(1):7–17. pmid:18236724

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Cleveland Clinic Journal of Medicine: 85 (6)
Cleveland Clinic Journal of Medicine
Vol. 85, Issue 6
1 Jun 2018
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Thoracic aortic aneurysm: How to counsel, when to refer
Frank Cikach, Milind Y. Desai, Eric E. Roselli, Vidyasagar Kalahasti
Cleveland Clinic Journal of Medicine Jun 2018, 85 (6) 481-492; DOI: 10.3949/ccjm.85a.17039

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Thoracic aortic aneurysm: How to counsel, when to refer
Frank Cikach, Milind Y. Desai, Eric E. Roselli, Vidyasagar Kalahasti
Cleveland Clinic Journal of Medicine Jun 2018, 85 (6) 481-492; DOI: 10.3949/ccjm.85a.17039
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  • Article
    • ABSTRACT
    • LIKELY UNDERDETECTED
    • RISK FACTORS FOR THORACIC AORTIC ANEURYSM
    • DIAMETER INCREASES WITH AGE, BODY SURFACE AREA
    • HOW IS TAA DIAGNOSED?
    • HOW IS TAA CLASSIFIED?
    • HOW IS ANEURYSM MANAGED ON AN OUTPATIENT BASIS?
    • WHEN SHOULD A PATIENT BE REFERRED?
    • WHEN SHOULD TAA BE REPAIRED?
    • HOW SHOULD PATIENTS BE SCREENED? WHAT FOLLOW-UP IS NECESSARY?
    • BACK TO OUR PATIENT WITH A BICUSPID AORTIC VALVE
    • BACK TO OUR PATIENT WITH MARFAN SYNDROME
    • Footnotes
    • REFERENCES
  • Figures & Data
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