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

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Presentation on theme: "Diseases of the Great Vessels Susan A. Raaymakers, MPAS, PA-C, RDCS (AE)(PE) Radiologic and Imaging Sciences - Echocardiography Grand Valley State University,"— Presentation transcript:

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 1. Annulus 2. Sinuses of Valsalva 3. Sinotubular Junction 4. Ascending Tubular Aorta 5. Arch 6. Descending Thoracic Aorta

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

5 Normal Aortic Anatomy  Six Segments Sinuses of Valsalva  Normal aorta dilates at the level of the sinuses by approximately 6 mm/m 2  Three sinuses of Valsalva of equivalent size  Right and left contain ostia of right and left coronaries respectively  Non

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

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

8 Normal Aortic Anatomy  Six Segments Arch  Three major branch vessels  Innominate artery (brachiocephalic), left common carotid and left subclavian Descending Thoracic Aorta

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

10 Echocardiographic Evaluation

11  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) 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  Transesophageal Broader window than transthoracic  Visualization from annulus through ascending and arch to level of gastroesophageal 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

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 Aortic rupturePlaque

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

23 Aortic Aneurysm  Definition: Localized abnormal dilatation of aorta containing all three layers of the aortic wall  Pathophysiology: Weakened media of the aorta Tunica externa Tunica media Tunica intima

24 Aortic Aneurysm Types Saccular Fusiform

25 Locations  Ascending aorta45%  Aortic Arch10%  Descending Thoracic Aorta35%  Abdominal Aorta10%

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)

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

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

30 Aortic Dilatation Secondary  Volume or pressure overload states AI or HTN  Post stenotic aortic dilation Valvular aortic stenosis Feigenbaum

31 Aortic Dilatation  Dilated ascending aorta Effacement (loss of tapering) of the sinotubular junction  Classic effacement  Sinotubular junction: same dimension as Valsalva sinus Non-dynamic

32 Aortic ANEURYSMS  May occur in ascending aorta Typically past sinotubular junction  Better visualized with TEE Feigenbaum

33 Aortic ANEURYSMS 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 (

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 Level of sinuses: 5.8 cm Aortic annulus: 2.8 cm Patient #2 Patient # b Feigenbaum 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 Feigenbaum20.27 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 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

48 Type II Dissection Feigenbaum Feigenbaum Feigenbaum

49 Type II Dissection Feigenbaum Feigenbaum Feigenbaum

50 True and False Lumens  True 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 20.30a Feigenbaum 20.30b 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 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 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 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 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 Feigenbaum

70 Pulmonary Artery Abnormalities

71  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 RPALPA 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 AO SVC MPA RPALPA 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.

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