16
Diseases of the Great Arteries
Basic Principles
Aortic dilation associated with hypertension or atherosclerosis is characterized by normal contours of the sinuses of Valsalva and narrowing at the sinotubular junction, with enlargement primarily in the ascending aorta. A bicuspid aortic valve often is accompanied by dilation of the aortic sinuses or ascending aorta, but some narrowing at the sinotubular junction usually is preserved. Inherited connective tissue disorders that cause aneurysms of the ascending aorta, such as Marfan syndrome and Loeys-Dietz syndrome, are characterized by effacement of the sinotubular junction and enlargement of the sinuses of Valsalva, in addition to dilation of the ascending aorta, resulting in a “water balloon” appearance of the proximal aorta (Fig. 16-1). Systemic inflammatory diseases, such as ankylosing spondylitis, are associated with aortic aneurysms and often also affect the valve tissue. Aneurysms also may be seen in tertiary syphilis (with a characteristic pattern of calcification), aortic arteritis (such as Takayasu arteritis and giant cell arteritis), and as a result of blunt or penetrating chest trauma. Large aortic aneurysms are prone to rupture, so prophylactic repair often is recommended.
Figure 16–1 Typical echo findings in Marfan syndrome.
The proximal aorta is markedly dilated with effacement of the sinotubular junction. Aortic annular dilation results in inadequate aortic leaflet apposition with a central jet of aortic regurgitation (AR) and consequent LV enlargement (LVE). Often, the anterior mitral leaflet (AMVL) is long and redundant.
An aortic dissection is a life-threatening situation in which an intimal tear in the aortic wall allows passage of blood into a “false” channel between the intima and the media (Fig. 16-2). This false channel may be localized or may propagate downstream, often in a spiral fashion, because of the pressure of blood flow in the channel. Complications related to the false lumen include:
Figure 16–2 Schematic diagram of an aortic dissection.
This example shows an entry site above the sinotubular junction into a false lumen. In real time, the intimal flap shows rapid undulating motion, independent of the cardiac cycle. As indicated by the arrows across the intimal flap in the descending aorta, multiple flow communications between the true and false lumens may be seen with color flow imaging.
Expansion with compression of the true aortic lumen (which supplies major branch vessels)
Propagation down major branch vessels
Sinus of Valsalva aneurysms (Fig. 16-3) may be congenital or may be due to infection, Marfan syndrome, or previous surgical procedures. A sinus of Valsalva aneurysm protrudes into adjacent chambers and may be associated with a fistula. Specifically, an aneurysm of the right coronary sinus protrudes into the right ventricular (RV) outflow tract, the left coronary sinus into the left atrium (LA), and the noncoronary sinus into the right atrium (RA).
Figure 16–3 Schematic diagram of a congenital sinus of Valsalva aneurysm.
A long, “wind sock”-like membranous outpouching of the right coronary cusp (RCC) protrudes into the RV outflow tract. If there are fenestrations in the aneurysm, an aortic-to-RV fistula is seen. Note that an aneurysm of the left coronary cusp (LCC) would protrude into the LA, whereas an aneurysm of the right coronary cusp would protrude into the RA.
Echocardiographic Approach
Echocardiographic Imaging
On TTE imaging, the proximal ascending aorta is well seen in parasternal long- and short-axis views (Fig. 16-4). Depending on ultrasound penetration, images of additional segments of the ascending aorta may be obtained by moving the transducer cephalad one or more interspaces. Image quality is enhanced by positioning the patient in a steep left lateral decubitus position, bringing the aorta in contact with the anterior chest wall (Figs. 16-5 and 16-6). In all adults, aortic diameter measurements should be made:
Figure 16–4 Evaluation of the aorta from a transcutaneous approach.
From a parasternal long-axis (PLAX) window, the sinuses and a segment of the ascending aorta are seen; from the suprasternal notch (SSN) window, the arch and proximal descending thoracic aorta are seen; from a posteriorly angulated apical two-chamber (A2C) approach, the mid-segment of the descending thoracic aorta is seen; and from a subcostal (SC) approach, the distal thoracic aorta and proximal abdominal aorta are seen. In some individuals, the segment of ascending aorta between the standard PLAX and SSN views can be imaged from a high parasternal position. However, in other patients, this segment of the aorta is “missed” on TTE imaging.
Figure 16–5 
Marfan syndrome.
The parasternal long-axis view (A) shows dilated sinuses with effacement of the sinotubular junction. In a short-axis view (B), the trileaflet valve in systole is stretched so the orifice is triangular, instead of the normal circular opening. DA, descending aorta; RVOT, RV outflow tract.
Figure 16–6 
Imaging the ascending aorta.
A, The standard parasternal long-axis view in a patient with Marfan syndrome shows only the aortic sinuses. B, With the transducer moved up one interspace, the loss of the normal contour of the sinotubular junction (arrow) and more of the ascending aorta are seen.
In a two-dimensional (2D) long-axis plane through the center of the aortic sinuses and ascending aorta
At end-diastole (onset of the QRS signal)
From the white-black to black-white interface defining the aortic lumen
At the aortic sinuses (maximum diameter) and mid-ascending aorta
When aortic dimensions are above normal or when the shape of the aortic sinuses and sinotubular junction is abnormal, additional measurements should be made as described below (Tables 16-1 and 16-2). Aortic sinus diameter also can be measured with an M-mode tracing at the level of the valve leaflets when a perpendicular orientation between the ultrasound beam and the aortic sinuses can be obtained. With M-mode tracings a leading edge-to-leading edge measurement convention is used. M-mode tracings may allow for more accurate identification of the aortic wall because of the high temporal sampling rate.
TABLE 16-1
Upper Limits of Normal Aortic Dimensions in Adults
Data sources: Roman MJ et al: Am J Cardiol 64:507-512, 1989 for annulus, sinuses, junction, and proximal ascending aorta based on TTE data; Hannuksela M et al: Scand Cardiovasc J 40:175-178, 2006 for arch and descending aorta based on CT data. (Upper limits are estimated from mean diameter plus 2 standard deviations.)
TABLE 16-2
Equations for Calculation of Expected Aortic Sinus Dimension Based on Body Size
| Patient Age | Expected Sinus Dimension (cm) |
| <20 years | 1.02 + 0.98 (BSA) |
| 20-39 years | 0.97 + 1.12 (BSA) |
| ≥40 years | 1.92 + 0.74 (BSA) |
Data from Roman et al: Am J Cardiol 64: 507-512, 1989.
The aortic arch is imaged from a suprasternal notch or supraclavicular approach with the patient in a supine position with the neck extended. Both longitudinal (Fig. 16-7) and transverse views of the arch are obtainable in nearly all individuals. Usually only a short segment of the ascending aorta is visible from the suprasternal notch window, but this is variable among patients. Also note that the descending aorta appears to taper because of an oblique image plane with respect to its curvature (i.e., the descending aorta is only partially in the image plane).
Figure 16–7 
Suprasternal notch view of the aorta.
The long-axis view (A) shows the ascending aorta (Ao), arch, and descending aorta with the right pulmonary artery (PA) in short-axis and the LA imaged inferiorly. The short-axis suprasternal notch view (B) shows the aortic arch, pulmonary artery, and LA.
The descending thoracic aorta is seen in cross section posterior to the LA in the parasternal long-axis view. A longitudinal section of this segment of the descending aorta may be obtained by clockwise rotation and lateral angulation of the transducer. From the suprasternal notch approach, a small portion of the descending thoracic aorta is seen. From the apical two-chamber view, a longitudinal section of a segment of the descending aorta is seen by lateral angulation and clockwise rotation of the transducer (Fig. 16-8).
Figure 16–8 
Descending thoracic aorta.
The posteriorly angulated apical two-chamber view shows the descending thoracic aorta (Ao) along its long axis in a patient with Marfan syndrome and prior aortic root replacement for type A dissection.
From the subcostal approach, the distal thoracic and proximal abdominal aorta is seen as it traverses the diaphragm. In patients with a left pleural effusion, images of the aorta also may be obtained by imaging through the fluid from the left posterior chest (paraspinal) with the patient in a right lateral decubitus position.
Doppler Flows
The use of low wall filter settings is needed to appreciate this normal flow pattern (Fig. 16-9). Flow abnormalities may be related to aortic disease (e.g., coarctation), shunts (e.g., patent ductus arteriosus), or aortic valve disease (e.g., regurgitation). Flow patterns in the proximal abdominal aorta are similar to those in the descending thoracic aorta and are easily recorded from the subcostal approach (Fig. 16-10).
Figure 16–9 Normal flow pattern in the descending thoracic aorta.
Pulsed Doppler recording of flow in the descending aorta from a suprasternal notch long-axis view shows antegrade flow in systole with a maximum velocity of 1.1 m/s and a normal systolic ejection curve. In diastole, there is brief early diastolic flow reversal, followed by low-velocity antegrade flow in mid-diastole and absence of flow (or low-velocity reversal) in end-diastole.
Figure 16–10 Normal flow pattern in the proximal abdominal aorta.
Subcostal view of the proximal abdominal aorta with color Doppler (top). The pulsed Doppler signal (bottom) shows normal antegrade flow toward the transducer in systole, followed by brief early diastolic flow reversal and slight antegrade flow in mid-diastole.
Limitations of Transthoracic Imaging of the Aorta
The major limitations of the TTE approach to ultrasound evaluation of the aorta are acoustic access and image quality. In many individuals, acoustic access is suboptimal or minimal from one or more of the windows needed for full evaluation of the aorta, leaving “gaps” in the echocardiographic examination. Even when acoustic access is adequate, image quality often is poor because of beam width at the depth of the aorta—particularly the descending thoracic aorta from apical and parasternal windows. Beam-width artifact, noise, and poor lateral resolution make differentiation of intraluminal defects from artifacts difficult. Because of these limitations, evaluation by TEE or other imaging modalities is appropriate in many patients with acute or chronic aortic disease.
Transesophageal Approach
From a high TEE probe position with the image plane rotated to approximately 45°, the aortic valve and sinuses of Valsalva are seen in short-axis. Slight withdrawal of the probe in the esophagus may allow short-axis imaging of the proximal ascending aorta, but more distal segments are obscured by interposition of the air-filled trachea between the esophagus (and transducer) and the ascending aorta. The long-axis view of the aortic valve, sinuses of Valsalva, and ascending aorta is obtained by rotating the image plane to approximately 120° (Fig. 16-11). In the long-axis plane, more cephalad segments of the ascending aorta may be seen by slowly moving the transducer to a higher esophageal position.
Figure 16–11 
TEE imaging of the normal ascending aorta.
The aortic long-axis view (A) is obtained at about 120° rotation and the short-axis view (B) at about 30° to 40° rotation. Both image planes are aligned to the cardiac landmarks, analogous to TTE views, with the exact degree of rotation varying between patients.
The descending thoracic aorta and the proximal abdominal aorta are well seen by the TEE approach. The descending thoracic aorta lies immediately lateral and slightly posterior to the esophagus, so posterior rotation of the probe provides excellent images in either a cross-sectional (transverse plane at 0°) or long-axis (at 90° to 120°) view (Figs. 16-12 and 16-13). Slight turning of the TEE probe is needed at different levels as the aorta curves relative to the esophagus. From a transgastric position, the proximal abdominal aorta is seen posterior to the stomach. Many examiners prefer to examine the length of the aorta in sequential cross-sectional views as the probe is slowly withdrawn from the stomach and esophagus, with imaging of the aortic arch just before probe removal. Any areas of abnormality can then be further examined in long-axis views.
Figure 16–12 
Dissection flap with fenestration.
A, On TEE imaging, a 2D short-axis view of the descending thoracic aorta shows a dissection flap separating the smaller true lumen (TL), with systolic flow in red, from the false lumens (FL), with spontaneous contrast due to low-velocity flow. B, In a long-axis view, a fenestration is seen with flow from the true lumen to the false lumen.
Figure 16–13 Descending aortic dissection.
TEE imaging of the descending aorta shows a dissection flap with spontaneous contrast in the false lumen (F) due to low flow in both short-axis (A) and long-axis (B) views. T, true lumen.
Doppler Flows
TEE color flow imaging of the aorta shows the normal antegrade flow pattern in the ascending aorta, arch, and descending aorta and is helpful in the evaluation of abnormal blood flow patterns when aortic dissection is present. However, quantitative evaluation of aortic flow velocities is challenging from the TEE approach. The ascending aorta sometimes can be evaluated from a transgastric long-axis view, although the intercept angle may not be parallel to flow, which results in velocity underestimation, particularly when high velocities are present. For the descending aorta, the direction of blood flow is nearly perpendicular to the direction of the ultrasound beam, limiting spectral Doppler velocity measurement, although flow signals may be obtained in a long-axis plane with the Doppler beam aligned at the distal end of the aorta. This approach likely underestimates velocity because of the nonparallel intercept angle, but it is useful to evaluate flow patterns, such as holodiastolic flow reversal with aortic regurgitation.
Bicuspid aortic valve disease with eccentric regurgitation
Annular dilation with “stretching” of the valve leaflets, resulting in central noncoaptation
Dilation of the sinuses or sinotubular junction, leading to inadequate leaflet overlap
Commisural involvement by aortic dissection, resulting in inadequate support of the leaflets
A flail aortic leaflet due to extension of a dissection flap into the valve tissue
Diffuse aortic wall and valve leaflet thickening due to an inflammatory process
Evaluation of aortic regurgitation includes the measurement of vena contracta width, identification of the origin and etiology of regurgitation, and evaluation of left ventricle (LV) size and systolic function (see Chapter 12).
Aortic Dilation and Aneurysm
Aortic dilation often is first recognized on the chest radiograph or on an echocardiographic examination requested for other reasons. In specific clinical settings, such as Marfan syndrome, aortic dilation is an expected consequence of a systemic disease (Table 16-3). In these cases, echocardiography is requested to assess the presence and degree of aortic abnormality. In patients with a bicuspid aortic valve, aortic dimension should be routinely measured (Fig. 16-14).
TABLE 16-3
Aortic Disease: Clinical Echocardiographic Correlates
CMR, cardiac magnetic resonance imaging; CT, computed tomography; STJ, sinotubular junction.
Figure 16–14 
Bicuspid aortic valve associated aortopathy.
Left, TTE imaging in a patient with a bicuspid aortic valve shows dilation of the ascending aorta with a maximum dimension of 5.26 cm at end-diastole. Center, TEE images confirm dilation of the ascending aorta, although dimension is slightly underestimated because of a slightly oblique image plane. Right, 3D reconstruction of the contrast computed tomographic images shows the location and severity of aortic dilation, but the timing of measurements during the cardiac cycle may not exactly match the echocardiographic data.
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