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Cardiac: Thoracic Aorta Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Cardiac ❯ Thoracic Aorta

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  • To achieve optimal aortic visualization, an opacification exceeding 250 Hounsfield units (HU)— ideally surpassing 300 HU—is advised. Typically, an iodinated contrast medium is administered at a flow rate of 3 to 6 mL/s, using a total volume between 100 and 125 mL. This is subsequently followed by a 40 mL normal saline flush at a rate of 4 mL/s. The post-contrast image acquisition is either automated through bolus tracking software or manually set using a test bolus, which typically involves 15 to 20 mL of contrast. Given the ease of use, the automated contrast bolus tracking software is preferred over the test bolus method. In addition, it involves reduced contrast doses and an enhanced signal-to-background ratio, ensuring no inadvertent opacification of parenchymal organs from test dose contrast.
    Computed Tomography Angiography for Aortic Diseases
    Ishan Garg et al.
    Radiol Clin N Am - (2024) -–- https://doi.org/10.1016/j.rcl.2024.01.001
  • On the other hand, VR images are created from 3D data sets by utilizing predetermined density thresholds. This is particularly useful in distinguishing between intraluminal contrast and stent materials, given that stent materials usually exhibit a higher density compared to intraluminal contrast. MIP images preferentially display only the highest density pixels from the data into a single plantar image. This allows excellent visualization of a highdensity material such as contrast, calcification, endograft, and collateral circulation (in scenarios with vascular occlusion or pronounced stenosis).
    Computed Tomography Angiography for Aortic Diseases
    Ishan Garg et al.
    Radiol Clin N Am - (2024) -–- https://doi.org/10.1016/j.rcl.2024.01.001
  • An acute PAU occurs due to the ulceration of an atherosclerotic plaque deep into the arterial wall. It accounts for 2% to 7% of all AAS and affects elderly patients with severe atherosclerotic disease.  It is most commonly located in the descending thoracic aorta. Clinically acute PAU presents similar to aortic dissection and other AAS. Acute PAU can be associated with IMH from hemorrhage in the media and can progress to frank aortic dissection (intimomedial tear with true and false lumen), aneurysm, or rupture (penetrating through all 3 layers of the aortic wall resulting in saccular, fusiform, or pseudoaneurysm, which may ultimately rupture) based on level of penetration. Overall, rupture is uncommon.
    Computed Tomography Angiography for Aortic Diseases
    Ishan Garg et al.
    Radiol Clin N Am - (2024) -–- https://doi.org/10.1016/j.rcl.2024.01.001
  • The most widely used classification system used for grading the severity of BTTAI was developed by Azizzadeh and colleagues in 2009, and since then, it has been endorsed by the Society for Vascular Surgery clinical practice guidelines.  
    Grade 1: Intimal tear, intimal flap, or both.  
    Grade 2: Intramural hematoma  
    Grade 3: Aortic wall disruption with pseudoaneurysm  
    Grade 4: Aortic wall disruption with free rupture  
    Grade 1 and 2 BTTAIs can be managed with either a nonoperative or operative approach. In contrast, the severity of Grade 3 and 4 injuries typically necessitates prompt, definitive management using endovascular repair. Frequently, the term, “minimal aortic injury” is used for sub-centimeter intimo-medial abnormality with no external contour deformity.
    Computed Tomography Angiography for Aortic Diseases
    Ishan Garg et al.
    Radiol Clin N Am - (2024) -–- https://doi.org/10.1016/j.rcl.2024.01.001 
  • “Type A aortic dissection is a surgical emergency occurring when an intimal tear in the aorta creates a false lumen in the ascending aorta. Prompt diagnosis and surgical treatment are imperative to optimize outcomes. Surgical repair requires replacement of the ascending aorta with or without aortic root or aortic arch replacement. Surgical outcomes for this highly lethal diagnosis have improved, with contemporary survival to discharge at Centers of Excellence of 85% to 90%. Survival is related to prompt treatment, preexisting medical comorbidities, presence or absence of end organ malperfusion, extent of aortic repair required, and the development of postoperative complications.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “The aorta is a conduit that extends from the left ventricle that delivers pulsatile blood distally to organs and tissue beds. Acute dissection of the ascending aorta is a lethal disease that requires prompt diagnosis and surgical intervention. Once almost exclusively a postmortem diagnosis, improvements in the accuracy of diagnostic modalities, anesthetic techniques, extracorporeal perfusion, methods of end organ protection during aortic replacement, types of prosthetic grafts, surgical techniques, and critical care have markedly improved outcomes over the last 40 years.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “It is useful to understand the anatomy of the aorta in order to appreciate the complexity of acute aortic syndromes . The annulus, aortic valvular leaflets, and coronary arteries along with the sinus of Valsalva comprise the aortic root. It is followed anatomically by the ascending aorta, which extends from the sinotubular junction to the innominate artery. The aortic arch, where supra-aortic branches arise, extends from the innominate artery to the left subclavian artery. The descending thoracic aorta, where numerous intercostal arteries arise, extends from the left subclavian artery to the level of the diaphragm. Finally, the abdominal aorta is defined by the length of the aorta below the diaphragm down to the iliac bifurcation.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • - Type A aortic dissection occurs when an intimal tear in the aorta creates a false lumen in the ascending aorta.
    - Type A aortic dissection is a surgical emergency requiring prompt diagnosis and treatment.
    - Computed tomographic angiogram is the diagnostic modality of choice.
    - Repair involves replacement of the ascending aorta with or without aortic root and/or arch replacement.
    - Contemporary in-hospital mortality is 10% to 15% but is highly related to comorbidities, extent of repair needed, and postoperative complications.
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “If the dissection involves the ascending aorta, it is a Stanford type A. If the ascending aorta proximal to the innominate artery is not involved in the process, the dissection is called a Stanford type B. The less commonly used DeBakey classification system was initially proposed in 1955 and then modified in 1965 and 1982 to correspond more closely with the Stanford classification system based on whether the ascending aorta was involved regardless of the site of the intimal tear or distal extent of the dissection.DeBakey type I and II dissections both involve the ascending aorta; however, a type I extends beyond the innominate artery, whereas a type II is confined to the ascending aorta. DeBakey type I and II dissections both correspond to a Stanford type A dissection. A DeBakey type III dissection corresponds to a Stanford type B dissection, where the ascending aorta proximal to the innominate artery is not involved.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “Aortic dissection is the most common and disastrous event to affect the aorta. It occurs nearly 3 times as frequently as rupture of abdominal aortic aneurysms in the United States.Studies of acute aortic syndromes recorded at tertiary care centers suggest that type A aortic dissection remains the most frequently transferred emergency through regional rapid transport systems.It occurs with a greater frequency than type B aortic dissections, while both types occur more frequently in men in the sixth decade of life. Women with aortic dissection are more likely to present at an older age than men and to have atypical symptoms, which often delays the diagnosis and subsequent treatment, leading to higher mortality in some studies.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “Aortic dissection is the most common and disastrous event to affect the aorta. It occurs nearly 3 times as frequently as rupture of abdominal aortic aneurysms in the United States.Studies of acute aortic syndromes recorded at tertiary care centers suggest that type A aortic dissection remains the most frequently transferred emergency through regional rapid transport systems.It occurs with a greater frequency than type B aortic dissections, while both types occur more frequently in men in the sixth decade of life.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • Risk factors for aortic dissection
    Lifestyle and cardiovascular risk factors
    - Long-term hypertension
    - Old age
    - Dyslipidemia
    - Pregnancy-induced hypervolemia
    - Weight-lifting
    - Smoking
    - Cocaine abuse
  • Risk factors for aortic dissection
    Congenital and connective tissue disorders
    - Bicuspid aortic valve
    - Marfan syndrome
    - Loeys-Dietz syndrome
    - Ehlers-Danlos syndrome
    - Turner syndrome
  • Risk factors for aortic dissection
    Trauma-Aortic transection
    - Motor vehicle deceleration injury
    - Falling from height
  • Risk factors for aortic dissection
    Iatrogenic
    - Cardiac catheterization
    - Arterial cannulation for cardiopulmonary bypass
    - Aortic cross-clamping during valvular or aortic surgery
    - Intra-aortic balloon pumps
  • Risk factors for aortic dissection
    Vascular inflammation
    Autoimmune disease
    - Giant cell arteritis
    - Takayasu arteritis
    - Bechet disease
  • Risk factors for aortic dissection
    Infectious disease
    - Syphilis
    - Tuberculosis
  • “Type A aortic dissection is a surgical emergency occurring when an intimal tear in the aorta creates a false lumen in the ascending aorta. Prompt diagnosis and surgical treatment are imperative to optimize outcomes. Surgical repair requires replacement of the ascending aorta with or without aortic root or aortic arch replacement. Surgical outcomes for this highly lethal diagnosis have improved, with contemporary survival to discharge at Centers of Excellence of 85% to 90%. Survival is related to prompt treatment, preexisting medical comorbidities, presence or absence of end organ malperfusion, extent of aortic repair required, and the development of postoperative complications.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “The aorta is a conduit that extends from the left ventricle that delivers pulsatile blood distally to organs and tissue beds. Acute dissection of the ascending aorta is a lethal disease that requires prompt diagnosis and surgical intervention. Once almost exclusively a postmortem diagnosis, improvements in the accuracy of diagnostic modalities, anesthetic techniques, extracorporeal perfusion, methods of end organ protection during aortic replacement, types of prosthetic grafts, surgical techniques, and critical care have markedly improved outcomes over the last 40 years.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “It is useful to understand the anatomy of the aorta in order to appreciate the complexity of acute aortic syndromes . The annulus, aortic valvular leaflets, and coronary arteries along with the sinus of Valsalva comprise the aortic root. It is followed anatomically by the ascending aorta, which extends from the sinotubular junction to the innominate artery. The aortic arch, where supra-aortic branches arise, extends from the innominate artery to the left subclavian artery. The descending thoracic aorta, where numerous intercostal arteries arise, extends from the left subclavian artery to the level of the diaphragm. Finally, the abdominal aorta is defined by the length of the aorta below the diaphragm down to the iliac bifurcation.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • - Type A aortic dissection occurs when an intimal tear in the aorta creates a false lumen in the ascending aorta.
    - Type A aortic dissection is a surgical emergency requiring prompt diagnosis and treatment.
    - Computed tomographic angiogram is the diagnostic modality of choice.
    - Repair involves replacement of the ascending aorta with or without aortic root and/or arch replacement.
    - Contemporary in-hospital mortality is 10% to 15% but is highly related to comorbidities, extent of repair needed, and postoperative complications.
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “If the dissection involves the ascending aorta, it is a Stanford type A. If the ascending aorta proximal to the innominate artery is not involved in the process, the dissection is called a Stanford type B. The less commonly used DeBakey classification system was initially proposed in 1955 and then modified in 1965 and 1982 to correspond more closely with the Stanford classification system based on whether the ascending aorta was involved regardless of the site of the intimal tear or distal extent of the dissection.DeBakey type I and II dissections both involve the ascending aorta; however, a type I extends beyond the innominate artery, whereas a type II is confined to the ascending aorta. DeBakey type I and II dissections both correspond to a Stanford type A dissection. A DeBakey type III dissection corresponds to a Stanford type B dissection, where the ascending aorta proximal to the innominate artery is not involved.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “Aortic dissection is the most common and disastrous event to affect the aorta. It occurs nearly 3 times as frequently as rupture of abdominal aortic aneurysms in the United States.Studies of acute aortic syndromes recorded at tertiary care centers suggest that type A aortic dissection remains the most frequently transferred emergency through regional rapid transport systems.It occurs with a greater frequency than type B aortic dissections, while both types occur more frequently in men in the sixth decade of life. Women with aortic dissection are more likely to present at an older age than men and to have atypical symptoms, which often delays the diagnosis and subsequent treatment, leading to higher mortality in some studies.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • “Aortic dissection is the most common and disastrous event to affect the aorta. It occurs nearly 3 times as frequently as rupture of abdominal aortic aneurysms in the United States.Studies of acute aortic syndromes recorded at tertiary care centers suggest that type A aortic dissection remains the most frequently transferred emergency through regional rapid transport systems.It occurs with a greater frequency than type B aortic dissections, while both types occur more frequently in men in the sixth decade of life.”
    Acute Type A Aortic Dissection.
    Elsayed RS1, Cohen RG1, Fleischman F1, Bowdish ME2.
    Cardiol Clin. 2017 Aug;35(3):331-345
  • Risk factors for aortic dissection
    Lifestyle and cardiovascular risk factors
    - Long-term hypertension
    - Old age
    - Dyslipidemia
    - Pregnancy-induced hypervolemia
    - Weight-lifting
    - Smoking
    - Cocaine abuse
  • Risk factors for aortic dissection
    Congenital and connective tissue disorders
    - Bicuspid aortic valve
    - Marfan syndrome
    - Loeys-Dietz syndrome
    - Ehlers-Danlos syndrome
    - Turner syndrome
  • Risk factors for aortic dissection
    Trauma-Aortic transection
    - Motor vehicle deceleration injury
    - Falling from height
  • Risk factors for aortic dissection
    Iatrogenic
    - Cardiac catheterization
    - Arterial cannulation for cardiopulmonary bypass
    - Aortic cross-clamping during valvular or aortic surgery
    - Intra-aortic balloon pumps
  • Risk factors for aortic dissection
    Vascular inflammation
    Autoimmune disease
    - Giant cell arteritis
    - Takayasu arteritis
    - Bechet disease
  • Risk factors for aortic dissection
    Infectious disease
    - Syphilis
    - Tuberculosis
  • “Abnormal dilatation of the thoracic aorta has been defined as a diameter greater than 50% above the high end of the normal range .The normal range of aortic diameters depends on age, sex, and body size, as well as on the anatomic segment.The average aortic diameter at the sinuses of Valsalva is 3.0 cm ± 0.5.Thoracic aortic measurements greater than 4 cm are generally considered to be consistent with an aneurysm.” 


    Pre- and Postoperative Imaging of the Aortic Root 
Hanneman K et al. 
RadioGraphics 2016; 36:19-37
  • Severe Aortic Calcification or “Porcelain Aorta”
    - “ Severe aortic calcification (“porcelain aorta”) is a clinically significant preoperative finding indicating that the aortic valve replacement surgery may be technically difficult or impossible. Aortotomy, cross clamping, cannulation, and suturing are more likely to cause embolic phenomena, aortic dissection, mural laceration and hemorrhage in the presence of severe calcification.”
  • “ Severe aortic calcification (“porcelain aorta”) is a clinically significant preoperative finding indicating that the aortic valve replacement surgery may be technically difficult or impossible. Aortotomy, cross clamping, cannulation, and suturing are more likely to cause embolic phenomena, aortic dissection, mural laceration and hemorrhage in the presence of severe calcification.”
    CT and MR Imaging of the Aortic Valve: Radiologic –Pathologic Correlation
     Bennett CJ et al.
    RadioGraphics 2012;32:1399-1420
  • Aortic Transection: Facts
    - 10-20% of patients survive the initial event
    - Occurs most commonly at aortic isthmus (space between brachiocephalic trunk and that of the ligamentum arteriosus)
    - Mediastinal hematoma (anterior of posterior mediastinum) is common but not diagnostic of aortic injury
    - Hematoma of interest most commonly around the aorta
  • Aortic Transection: Facts
    - The direct signs of aortic transection include;
    - Vessel caliber change
    - Pseudoaneurysm
    - Intramural flap
  • Cardiac Trauma: Differential Diagnosis
    - Aortic transection
    - Valvular rupture
    - Hemopericardium
    - Cardiac tamponade
  • Hypovolemic Shock: CT Findings
    - Bright adrenals
    - Diffuse fluid filled dilated small bowel
    - Hyperenhancement of the small bowel
    - Hyperenhancement of the gall bladder mucosa
    - Reduced splenic perfusion
    - Intense enhancement of the kidneys
    - Peripancreatic edema
  • CARDIAC TAMPONADE
    - Accumulation of pericardial fluid, blood, tumor, or air that increases intrapericardial pressure, restricting cardiac filling, and decreasing cardiac output.
    - Cardiac emergency that can be fatal.
  • CARDIAC TAMPONADE:
    ACUTE
    - Rapid onset seen in cardiac/great vessel trauma or s/p invasive procedure
    - Beck triad: hypotension, jugular venous distention, and distant heart sounds
    - Effusion may be small, given the relative inelasticity of the pericardium
  • CARDIAC TAMPONADE:
    SUBACUTE
    - More gradual process of fluid accumulation
    - Allows for stretching of pericardium and much larger effusions than seen acutely
    - The most common type of tamponade seen in malignancy, TB, uremia
    - S+S more subtle, some or all of Beck’s triad may be absent
  • CARDIAC TAMPONADE:
    CT FINDINGS
    - Enlarged SVC - adjacent thoracic aorta
    - Enlarged IVC > 2X adjacent aorta
    - Contrast reflux into IVC, azygos vein
    - Enlarged hepatic and renal veins
  • CARDIAC TAMPONADE:
    CT FINDINGS
    - <0 HU: chylopericardium- CA, infection
    - 0-20 HU: simple serous effusion- CHF, renal failure, or non-hemorrhagic CA
    - > 20 HU hemopericardium, CA, purulent exudates, or myxedematous effusion
  • "A right aortic arch can occur in isolation or in association with congenital heart disease, especially in tetralogy of Fallot and truncus arteriosus. Edwards embryologic double aortic arch model can help explain the embryogenesis of many variations of right aortic arch."

    Right Aortic Arch and Its Variants
    Kanne JP, Godwin D
    J Cardiovascular Comput Tomogr (2010) 4,293-300

  • Double Aortic Arch: Facts
    - Most common symptomatic vascular aortic arch
    - The right arch is larger , posterior, ad more cephalad than the left arch in two thirds of patients and the descending aorta is usually contralateral to the dominant arch
    - Trachea compression is common
    - Descending aortic diverticulum (Kommerell) is not uncommon
  • Aberrant left subclavian artery: facts
    - Left subclavian arise as last arch vessel and crosses right to left (patient has right sided arch) and posterior to the esophagus
    - Bulblike dilatation of abberrant subclavian can occur and is called diverticulum of Kommerell
    - When large the diverticulum of Kommerell can cause symptoms of dysphagia
  • Aortic Arch Anomalies
    - Aberrant left subclavian artery
    - Mirror image branching
    - Right aortic arch with left descending aorta
    - Right aortic arch with aberrant branchiocephalic artery
    - Right aortic arch with isolated left subclavian artery
    - Double aortic arch
    - Cervical aortic arch
  • Anomalies of the Aortic Arch: facts
    - Frequency ranges from 0.5% to 3%
    - Most patients asymptomatic though others may have dysphagia, dyspnea, hypertension or congestive heart failure
    - Right aortic arch occurs in 0.1% of adults
    - There are 6 paired arteries arising from the aortic sac during embryogenesis with the fourth left arch forming the aortic arch
  • "A right aortic arch can occur in isolation or in association with congenital heart disease, especially in tetralogy of Fallot and truncus arteriosus. Edwards embryologic double aortic arch model can help explain the embryogenesis of many variations of right aortic arch."

    Right Aortic Arch and Its Variants
    Kanne JP, Godwin D J
    Cardiovascular Comput Tomogr (2010) 4,293-300

     

  • "Aneurysmal dilatation most often involves the right coronary Valsalva sinus, followed by the noncoronary and left coronary sinus. The clinical manifestations of ruptured and nonruptured Valsalva sinus aneurysms very widely, ranging from an asymptomatic heart murmor and insidiously progressive dyspnea to acute chest pain and cardiac arrest."

    Valsalva Sinus Aneurysms: Findings at CT and MR Imaging
    Bricker AO et al.
    RadioGraphics 2010; 30:99-110

  • Valsalva Sinus Aneurysms: Complications
    - Aortic regurgitation occurs in 30-50% of patients (both in ruptured and non-ruptured sinus aneurysms)
    - Compression can comprimise coronary artery and lead to MI
    - When they rupture they go most commonly into the right ventricle (55.6%) and right atrium (30.3%)
  • Valsalva Sinus Aneurysms: Facts
    - Rare
    - Can be either congenital or acquired
    - Slightly more common in men
    - Acquired causes; bacterial endocarditis, syphilis, TB, atherosclerosis, deceleration injury (rare)
    - Congenital causes; Marfans, Ehlers-Danlos syndrome
  • "Our study shows the feasibility of preoperative CT in aortic infective endocarditis for providing relevant data about the presence and relationships of aortic valvular pseudoaneurysms."

    Preoperative Evaluation in Aortic Endocarditis: Findings on Cardiac CT Gahide G et al.
    AJR 2010; 194:574-578

  • Why do we gate evaluation of the thoracic aorta?
    - Quality evaluation of the entire thoracic aorta including the aortic sinus and aortic valve
    - Definition of the coronary arteries especially in the proximal portions of the coronaary vessels
  • "Optimal image quality for either technique is obtained with a relatively slow heart rate, which may require beta-blocker medication."

    Prospective and Retrospective ECG Gating for Thoracic CT Angiography: A Comparitive Study
    Wu W et al.
    AJR 2009;193:955-963

  • "Compared with retrospective ECG-gated thoracic CT angiography, prospective ECG-gated thoracic CT angiography was associated with a lower radiation dose, slightly increased contrast load, increased aortic attenuation values, and equivalent image quality."

    Prospective and Retrospective ECG Gating for Thoracic CT Angiography: A Comparitive Study
    Wu W et al.
    AJR 2009;193:955-963

  • Takayasu Arteritis: Numano Classification
    - Type IV: involvement of only the abdominal aorta with or without the renal arteries
    - Type V: involvement of the entire aorta with branches
    - Involvement of the coronary arteries or the pulmonary arteries is indicated with a C(+) or P(+) respectively
  • Takayasu Arteritis: Numano Classification
    - Type I: involvement of only the branches of the aortic arch
    - Type IIA: involvement of the ascending aorta or the aortic arch with or without branches
    - Type IIB: involvement of the descending thoracic aorta with or without the ascending aorta or the aortic arch with its branches
    - Type III: involvement of the entire descending aorta with or without the renal arteries
  • Takayasu Arteritis: Facts
    - Large vessel arteritis that affects aorta and its branches
    - Affects woman and young girls most commonly (80-90% are femal in second and third decade of life)
    - Circumferential wall thickening is earliest finding
    - Treatment includes steroids or immunosuppressive therapy as well as surgical revascularization of sites of involvement
  • Marfans Syndrome: Surgery
    - Ascending aorta 45 mm or growing greater than 0.5 cm/yr is indication for surgery
    - Repair is of root , aortic valve and ascending aorta
  • Marfans Syndrome: Cardiovascular Findings
    - Minor Criteria
    • Dilatation or dissection of the descending or abdominal aorta before the age of 50 years
    • Dilatation of the main pulmonary artery before age 40
    • Mitral valve prolapse
    • Calcification of the mitral valve before age 40
  • Marfans Syndrome: Cardiovascular Findings
    - Major Criteria
    • Dilatation of the ascending aorta involving at least the sinuses of Valsalva with or without aortic regurgitation
    • Dissection of the ascending aorta
  • Ghent Criteria: Major and Minor Findings
    2 major and 1 minor criteria

    or

    1 major and 4 minor criteria 
  • Marfan Syndrome: Facts
    - Autosomal dominant
    - Caused by mutations in the fibrillin-1 gene (FBN1) on chromosome 15
    - Diagnosis based on Ghent criteria which include cardiovascular, ocular, and pulmonary abnormalities
    - Average age of death in untreated patients is 35 years while in treated patients up to age 75 years
  • Supravalvular Aortic Stenosis: facts
    - Focal or diffuse narrowing of the aorta starting at the sinotubular junction and often involves the entire ascending aorta
    - SVAS may be associated with Williams-Beuren syndrome which is an autosomal dominant multisystematic disorder that may manifest with SVAS (71% of cases), mitral valve prolapse and pulmonary artery stenosis
  • Coarctation of the Aorta: Facts
    - Male predominance (1.5:1)
    - Aortic narrowing in region of ligamentum arteriosum just distal to the left subclavian artery
    - Solitary lesion in 82% of cases but can be associated with Turner syndrome, bicuspid aortic valve, intracranial aneurysms, VSD and ASD defects, and Shone complex (left ventricular outflow tract obstruction and parachute mitral valve)
  • Patent Ductus Arteriosus: Facts
    - PDA occurs in 1 of 2,000 children born full term
    - More common in woman (2-1)
    - Treatment includes surgical closure or percutaneous placement of an occluder device
  • Patent Ductus Arteriosus: Facts
    - Connects the proximal descending aorta, immediately distal to the origin of the left subclavian artery, with the proximal left pulmonary artery at the junction of the main pulmonary artery
    - Closes functionally 18-24 hours after birth and anatomically at one month
    - If it is patent after 3 months it is a PDA
  • "With its high spatial resolution and isotropic and volumetric information multidetector CT performed with or without ECG-gated technique allows accurate and fast noninvasive characterization of aortic pathologic conditions."

    Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
    Kimura-Hayama ET, et al
    RadioGraphics 2010; 30;79-98

     

  • "Multidetector CT is an alternative tool helpful in establishing the primary diagnosis, defining anatomic landmarks and their relationships, and identifying associated cardiovascular anomalies. It is also an adjunct in the evaluation of complications during follow-up."

    Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
    Kimura-Hayama ET, et al
    RadioGraphics 2010; 30;79-98

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