1. Diseases of the Great Vessels
Susan A. Raaymakers, MPAS, PA-C, RDCS (AE)(PE)
Radiologic and Imaging Sciences - Echocardiography
Grand Valley State University, Grand Rapids, Michigan

2. Tyler Kahle Story (20 minutes duration)

3. Normal Aortic Anatomy Six Segments Annulus Sinuses of Valsalva
Sinotubular Junction
Ascending Tubular Aorta
Arch
Descending Thoracic Aorta
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

4. Normal Aortic Anatomy Six Segments Annulus
Represents the junction of the prox. Ao and the LVOT.
Part of fibrous skeleton of the heart and is contiguous with the anterior mitral valve leaflet and perimembranous septum
Fibrinous structure so relatively stable and resistant to dilation:useful for indexing to remaining aortic sizing
NL 13 +/-1 mm/m2
NL size cm
Proximal portion: annulus to proximal ascending aorta commonly referred to as the root
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

5. Normal Aortic Anatomy Six Segments Sinuses of Valsalva
Normal aorta dilates at the level of the sinuses by approximately 6 mm/m2
Three sinuses of Valsalva of equivalent size
Right and left contain ostia of right and left coronaries respectively
Non
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

6. Normal Aortic Anatomy Six Segments Sinotubular Junction
Aorta tapers to within 2 to 3 mm of annular size
Crucial to nature of aortic valve coaptation
Insertion of aortic valve cusps: continuous from the level of the annuls up through the sinuses to the level of the sinotubular junction
Dilatation of sinotubular junction may result in splaying of coaptation line of the aortic cusps leading to secondary aortic insufficiency
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

7. Normal Aortic Anatomy Six Segments Ascending Tubular Aorta
Dimension similar to sinotubular junction
Ascending aorta terminates at the left innominate artery (brachiocephalic) where aortic arch begins and continuous to the left subclavian and ligamentum arteriosum
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

8. Normal Aortic Anatomy Six Segments Arch Descending Thoracic Aorta
Three major branch vessels
Innominate artery (brachiocephalic), left common carotid and left subclavian
Descending Thoracic Aorta
Anulus: Represents the junction of the prox. Ao and the LVOT
NL size cm
Sinus of Val.: Three sinus of equal size. Left, Right, Noncoronary .
left –left Main Coronary artery, Right- RCA
NL size cm
ST Junction: narrowing just after the sinus of valsalva
NL size cm
Three Head vessels: Inominate, left common carotid, left subclavian

9. Walls of the Aorta Intima Media Adventitia Thin and smooth
Elastic and muscular
Adventitia
Outer layer

10. Echocardiographic Evaluation

11. Echocardiographic Evaluation
Evaluation of the intrathoracic portion of the aorta and of aortic disease
TTE: limited to proximal ascending aorta and a small portion of the descending aorta behind LA
Major use of TEE: high-resolution view of entire length of aorta form aortic valve to approximately the diaphragm
Accuracy equivalent to computed tomography (CT) and magnetic resonance imaging (MRI)

12. Echocardiographic Evaluation
Superior angulation in parasternal long-axis view
Emphasizes visualization of normal ascending aorta (typically 4 -5 cm may be seen)
20.03 Feigenbaum

13. Echocardiographic Evaluation
Suprasternal notch view
Images more feasible in children and adolescents
Occasional discomfort of ultrasound probe in this area
20.4 Feigenbaum

14. Echocardiographic Evaluation
Descending thoracic aorta seen in
Parasternal long axis behind LA
Level of the gastroesophageal junction, posterior apical four chamber view
Non-dynamic

15. Echocardiographic Evaluation

16. Echocardiographic Evaluation
Transesophageal
Broader window than transthoracic
Visualization from annulus through ascending and arch to level of gastroesophageal
Left: FIGURE Transesophageal longitudinal view of the heart including visualization of the ascending aorta. Transesophageal echocardiogram of the ascending aorta recorded in a normal disease-free individual. A: Longitudinal (127-degree) view that provides imaging analogous to that of the transthoracic long-axis view seen in Figure Again note the symmetric dilation at the level of the sinuses and the narrowing at the level of the sinotubular junction.
Right: FIGURE Transesophageal echocardiogram recorded at 53-degree probe rotation at the base of the heart. These images were acquired at the same transducer position as those in Figure With this probe orientation, a short-axis view of the aorta is obtained at the level of the sinuses, revealing the left (L), right (R), and non (N) coronary sinuses. The left atrium (LA), right atrium (RA), and proximal pulmonary artery (PA) are well visualized. A: Image recorded in diastole, and three symmetric sinuses are noted as well as three coaptation lines of the cusps.
Non-dynamic

17. Echocardiographic Evaluation
Transesophageal
Typically imaging begins with imaging of the ascending aorta with probe behind the left atrium
Proximal 5 to 10 cm of the ascending aorta can be visualized
Scanning at a 120-degree imaging plane
Non-dynamic

18. Echocardiographic Evaluation
Transesophageal
Rotate probe 30-60°
Series of short-axis views of proximal ascending aorta including short axis of aortic valve
Non-dynamic

19. Echocardiographic Evaluation
Transesophageal
Descending aorta
Insertion of TEE probe deeper toward gastroesophageal junction
Non-dynamic
Note: probe is not inserted very deeply for imaging of aorta and therefore results in more curvature and may be less tolerated
Non-dynamic

20. Intravascular Ultrasound
Performed with high-frequency: MHz
Used in diagnosis and management of aortic dissection and as a primary imaging tool
Allows highly detailed, high-resolution
Plaque
Aortic rupture

21. Diseases of the Aorta Aortic Dilatation Aortic Dissection
Thoracic Aortic Aneurysms
Traumatic Injury
Aortic Atherosclerosis
Sinus of Valsalva Aneurysms

22. Aortic Dilatation Dilatation can occur at any point along aorta
Primary
Secondary
Idiopathic dilatation
Also referred to as Anuloaortic ectasia
Unclear whether distinct disease entity due to aging, hypertension or unrecognized disease of aorta
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN

23. Aortic Aneurysm
Definition: Localized abnormal dilatation of aorta containing all three layers of the aortic wall
Pathophysiology: Weakened media of the aorta
Tunica intima
Saccular: a weakening in the vessel wall at one point which results in a pouch with a small neck
Fusiform: uniform dilitation of the entire circumference of the vessel
Tunica media
Tunica externa

24. Aortic Aneurysm Types Saccular Fusiform
Saccular: a weakening in the vessel wall at one point which results in a pouch with a small neck
Fusiform: uniform dilitation of the entire circumference of the vessel

25. Locations Ascending aorta 45% Aortic Arch 10%
Descending Thoracic Aorta 35%
Abdominal Aorta 10%

26. Causes for Aortic Aneurysms
Atherosclerosis
Medial Degeneration
Idiopathic (annuloaortic ectasia)
Marfan's Syndrome
Other heritable disorders
Associated with Bicuspid Aortic Valve

27. More Causes
Aortic Dissection with Dilitation of persisting false lumen
Trauma with incomplete aortic rupture
Syphilis
Mycotic (Bacterial, Fungal, Tuberculous aortitis)
Noninfectious aortitis (Giant-cell, Takayasu’s Syndrome)
Takayasu’s Def. Inflamation of the aorta and it’s proximal branches
Typically in pts less than 40yrs
Intima becomes thickened and inflamed
can look like plaque

28. Aortic Dilatation Primary Occurs with cystic medial necrosis
Typified by Marfan’s
May be seen in other connective ts disorders
Results in weakening of medial layers
Subsequent dilation and aneurysm formation
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN

29. Aortic Dilatation Primary Marfan’s
Characteristically involves ascending aorta and sinuses
Imaging recommendations:
Radiography for skeletal abnormalities
Serial chest radiography for demonstration of progressive aortic dilation
2D echocardiography for early dx and monitoring of aortic dilation
CT or MRI for evaluation of aortic disease
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN

30. Aortic Dilatation Secondary Volume or pressure overload states
AI or HTN
Post stenotic aortic dilation
Valvular aortic stenosis
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN
20.11 Feigenbaum

31. Aortic Dilatation Dilated ascending aorta
Effacement (loss of tapering) of the sinotubular junction
Classic effacement
Sinotubular junction: same dimension as Valsalva sinus
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN
Non-dynamic

32. Aortic ANEURYSMS May occur in ascending aorta
Typically past sinotubular junction
Better visualized with TEE
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN
20.11 Feigenbaum

33. Aortic ANEURYSMS 20.13 Feigenbaum
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN
20.13 Feigenbaum

34. Aortic ANEURYSMS and DILATION
Rupture or dissection
Directly related to degree of dilation
Indication for prophylactic aortic surgery
55 mm
Many centers use 50 mm
Rapid change in dilation (<than 5 mm per year)
Primary: caused by cystic medial necrosis such as Marfans syndrome
Secondary: caused by volume or pressure overload such as AI or HTN
Non-dynamic images

35. Marfan’s Syndrome Inherited connective tissue disorder
Echocardiography: initial screening tool for patients or first-degree relatives
TEE for more specific information
Marked dilation of ascending aorta
Disproportionate involvement of sinuses of Valsalva
Early cases
Mild dilation of sinus
Sinotubular effacement
Malcoaptation of aortic cusps
Resultant in AI

36. Marfan’s Syndrome Patient #1 Patient #2 20.20b Feigenbaum
High prevalence for mitral valve prolapse. Range may be mild to massive.
Aortic insufficiency due to dilation of the sinotubular junction, which results in loss of coaptation
Level of sinuses: 5.8 cm
Aortic annulus: 2.8 cm
20.21a Feigenbaum

37. Valsalva Sinus Aneurysm
May form from any of the three Valsalva sinuses
Most often arise form the right sinus
Size: highly variable
Aneurysms arising from the right Valsalva sinus typically protrude down into the right atrium
Appear as “windsock” structure in the right atrium

38. Valsalva Sinus Aneurysm
Right sinus of Valsalva aneurysm
Protruding into right ventricular outflow tract
20.24a Feigenbaum
20.24b Feigenbaum

39. Valsalva Sinus Aneurysm
Right sinus of Valsalva aneurysm
Protruding into right ventricular outflow tract
20.24a-c Feigenbaum

40. Valsalva Sinus Aneurysm
Colorflow
Major complication of Valsalva Sinus Aneurysm: rupture
Most common location for rupture: right atrium
Results in instantaneous elevation of right heart pressures
Jugular distension
Loud continuous murmur
20.26 Feigenbaum
20.27 Feigenbaum

41. Valsalva Sinus Aneurysm
Colorflow
Major complication of Valsalva Sinus Aneurysm: rupture
Most common location for rupture: right atrium
Results in:
Instantaneous elevation of right heart pressures

42. Aortic Dissection Acute
Symptoms
Sudden onset of severe chest pain and/or back pain
Wide range of secondary cardiovascular and physiologic abnormalities
Typically occurs with pre-existing
Aortic dilation
Cystic medial fibrosis due to Marfan’s syndrome
Long standing hypertension
Any aspect of the aorta may dissect

43. Aortic Dissection
Two Basic Variants
Classic
Spontaneous hematoma

44. Aortic Dissection Classic
Tear from lumen through the intima into the medial layer with subsequent propagation of a column of blood
Further dissect the intima away form the media
Propagation may be both proximal and distal to the initial intimal tear

45. Aortic Dissection Classic Typically begins either
At the area of the ligamentum arteriosum
Propagates through the arch and into the ascending aorta
Or starts in ascending aorta and propagate distally

46. Aortic Dissection Spontaneous Intramural Hematoma
Clinical presentation with respect to nature of symptoms: virtually identical to classic dissection
Hemorrhage into the medial layer then dissects proximally or distally to a variable degree WITHOUT rupture into the adventitia

47. Aortic Dissection Two schemes for identification Types Stanford (A-B)
DeBakey (1, 2, 3)
Types
Type A(1): (70% occurrence) Throughout ascending and descending aorta
Type A(2): (5% occurrence) Confined to ascending aorta
Type B(3): (25% occurrence) Confined to descending aorta
Isolated: Aortic arch
Crucial factor: does it involve the ascending aorta: greater likelihood of rupture, pericardial effusion, aortic insufficiency or coronary involvement, which may be lethal.
Ascending aortic dissection is considered surgical emergency. Descending is best managed medically

48. Type II Dissection
20.34 Feigenbaum
20.31 Feigenbaum
20.35 Feigenbaum

49. Type II Dissection
20.34 Feigenbaum
20.31 Feigenbaum
20.35 Feigenbaum

50. True and False Lumens True lumen False lumen Pulsatile aortic flow
Expand w/systole
Circular or ovale typically
In descending aorta: usually smaller of the two lumens
False lumen
Continuous flow in venous flow pattern
Often filled with twirling homogenous echoes (stasis of blood or frank thrombus)
Tags of tissue (small muscle remnants where the intima has been sheared from the media)
Non-dynamic

51. True and False Lumens True lumen False lumen 20.36a Feigenbaum

52. Type III Aortic Dissection
Descending aortic dissection
20.39b Feigenbaum

53. Imaging Goals for Dissection
Ascending dissection
Most are detected by TEE rather than TTE
20.30b Feigenbaum
20.30a Feigenbaum

54. Intramural Hematoma May occur at any point along the aorta
More common in the descending aorta and arch
Appears as a smooth homogenous concentric thickening of the wall
Typically > 7 mm in thickness
No active flow with the “lumen” and no tear in the intima
20.44 Feigenbaum

55. Goals of Imaging Confirmation of diagnosis Location
Extent of dissection
Mechanism of AI
Presence of pericardial or pleural effusions
Coronary artery involvement
Head vessel involvement
Detection of rupture
Location of:
Intimal tear (entry)
Re-entry site

56. Transesophogeal Echo Superior over transthoracic echo
CT, MRI and Aortography are also common methods of diagnosis
Pitt falls of TEE
Reverberations, catheters
Mirror image artifacts
Thoracic aneurysm with mural thrombus

57. Echo Findings 2D MM Color/Doppler
Presence of an intimal flap, which may appear as a thin linear structure
A true and a false lumen
Pericardial effusion MM
Dilated aorta >4.2cm
Increased aortic wall thickness
Color/Doppler AI
Flow between the true and false lumen

58. Role of TEE Best for seeing ascending aortic aneurysms
Helps rule out aortic dissection versus aortic aneurysm
Instant results
Non-dynamic

59. Mechanisms of AI Dilatation of aortic root
Asymmetric dissection causes faulty coaptation of the aortic valve
Prolapsing intimal flap back through the aorta

60. Aortic Atheroma Atherosclerosis of the aorta
Frequently encountered in TEE
May be identified in SSN view
Common in advanced age, HTN, elevated cholesterol
May be a component of atherosclerotic aneurysm and intramural hematoma of the aorta
Most common in descending aorta
Less frequently in ascending aorta

61. Aortic Atheroma
Characterized as symmetric and crescentic (curved shaped)
Smooth, homogenous crescent filling a portion of the aortic lumen, protruding or complex
Complex: defined as atherosclerotic disease with epedunculated or mobile components
Grading System
I = 1 – 3.9mm thickness
II = > 4mm thickness
III = Debris (mobile regardless of size)

62. Aortic Atheroma Two Different Patients 20.50a Feigenbaum
20.50b Feigenbaum

63. Miscellaneous Conditions Aortic Pseudoaneurysm
Contained rupture of the aorta
Characterized by an extraluminal aneurysmal sack communicating with the true lumen by a relatively narrow neck
Occur in several situations
Spontaneous rupture of an aortic aneurysm with subsequent sealing off of the hemorrhage
Sequelae of aortic dissection with further rupture through the adventitial layers
Rare occasions: iatrogenic injury
20.55 Feigenbaum

64. Miscellaneous Conditions Aortic Trauma
Wide spread of extent of injury/pathology
Simple contusion
Intimal tear
Intramural hematoma
False Aneurysm
Frank rupture (transection)
Major dissection NOT a feature of aortic trauma(usually no underlying medial disease)

65. Miscellaneous Conditions Aortic Trauma
Blunt chest injury
High-speed impact injury (i.e. unrestrained MVA)
Partial or complete transection of the descending aorta, classically at area of ligamentum arteriosum
Complete = nearly instantaneous fatal event
Partial = hemorrhage and shock
CT or MRI typically primary diagnostic tool

66. Traumatic Injury Cont. Intimal Lacerations (transection)
The mechanism of injury historically was thought to be rapid deceleration with "whipping" of the aorta at points of attachment (i.e., aortic root, ligamentum arteriosus, diaphragmatic hiatus).
More recent evidence suggests that injury at the most common site, the aortic isthmus, is due to
Compression of the anterior chest wall and pinching of the aorta between structures of the anterior bony thorax and the thoracic spine.

67. Aortic Infections – mentioned earlier under causes of aortic aneurysm
Bacterial or fungal infections of the aorta is rare
Manifest as a pedunculated mobile mass
Syphilic aortic disease: rare encountered in contemporary practice
Results in inflammatory thickening of the proximal aorta

68. Aortic Thrombus
Rare
Bland mobile thrombus form within the thoracic aorta
More common in the proximal descending thoracic aorta and is often associated with evidence of peripheral embolization
Thrombi are highly mobile echo-dense masses within the lumen, appear attached to the aortic wall by a fairly thin stalk
20.59 Feigenbaum

69. Takayasu Arteritis
Inflammatory disease of the aorta and its proximal branches
Occurs in patients <40 years old
Results in marked, irregular intimal thickening and accumulation of inflammatory tissue in the proximal aorta and ostia of major branches including the coronary arteries
Echo: appears similar to atheroclerotic disease
20.61 Feigenbaum

70. Pulmonary Artery Abnormalities

71. Pulmonary Artery Abnormalities
Most abnormalities of PA: congenital
Postenotic dilation
Branch pulmonary artery stenosis
Abnormal position of the pulmonary artery
Transposition of the great vessels
May also be involved by systemic diseases such as Takayasu arteritis
Pulmonary artery dissection: rare
Reported in patients with chronic pulmonary hypertension

72. Pulmonary Artery Abnormalities
Dilated pulmonary arteries
Right-sided volume overload (e.g., atrial septal defect)
Pulmonary hypertension
Idiopathic dilation of the pulmonary artery (Rare)
Finding of dilated PA mandates careful evaluation for:
Right-sided pressure or volume overload

73. Pulmonary Artery Abnormalities
Standard views
PSAX
RVOT
Subcostal four with severe anterior tilt
SSN (right pulmonary artery)
High parasternal short axis
RPA
LPA
Non-dynamic

74. Pulmonary Artery Abnormalities
Transesphageal
0° esophageal level superior the LA
Long axis of pulmonary artery
May not be obtained in all patients d/t interposition of the air-filled bronchus
RPA
LPA
SVC AO
MPA
Feigenbaum

75. Pulmonary Artery Abnormalities
Transesphageal
90° plane (analogous to TTE RVOT view)
Image quality may be suboptimal due to distance of PA from transducer in this view
Alternative studies CT
MRI
Contrast angiography
Radionuclide ventilation/perfusion scan for evaluation for pulmonary embolism

76. Pulmonary Artery Abnormalities
Limitations and alternative approaches
Acoustic access and quality
Body habitus and skill of sonographer
Interpretation must consider the likelihood of
False-positive findings from beam-width artifacts, reverberations and oblique image planes
False-negative findings due to limited acoustic access and poor resolution
Chest CT
Advantages: wide field of view, high accuracy and wide availability
Disadvantages: ionizing radiation and non-portable nature of the study
MRI
Advantages: high resolution, high diagnostic accuracy, wide field of view and ability to orient the images along the long axis of the aorta

77. Sources
Feigenbaum H, Armstrong W. (2004). Echocardiography. (6th Edition). Indianapolis. Lippincott Williams & Wilkins.
Goldstein S., Harry M., Carney D., Dempsey A., Ehler D., Geiser E., Gillam L., Kraft C., Rigling R., McCallister B., Sisk E., Waggoner A., Witt S., Gresser C.. (2005). Outline of Sonographer Core Curriculum in Echocardiography.
Otto C. (2004). Textbook of Clinical Echocardiography. (3rd Edition). Elsevier & Saunders.
Reynolds T. (2000). The Echocardiographer's Pocket Reference. (2nd Edition). Arizona. Arizona Heart Institute.