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GUIDELINES AND STANDARDS Multimodality Imaging of Diseases of the Thoracic Aorta in Adults: From the American Society of Echocardiography and the European Association of Cardiovascular Imaging Endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance Steven A. Goldstein, MD, Co-Chair, Arturo Evangelista, MD, FESC, Co-Chair, Suhny Abbara, MD, Andrew Arai, MD, Federico M. Asch, MD, FASE, Luigi P. Badano, MD, PhD, FESC, Michael A. Bolen, MD, Heidi M. Connolly, MD, Hug Cu ellar-Calabria, MD, Martin Czerny, MD, Richard B. Devereux, MD, Raimund A. Erbel, MD, FASE, FESC, Rossella Fattori, MD, Eric M. Isselbacher, MD, Joseph M. Lindsay, MD, Marti McCulloch, MBA, RDCS, FASE, Hector I. Michelena, MD, FASE, Christoph A. Nienaber, MD, FESC, Jae K. Oh, MD, FASE, Mauro Pepi, MD, FESC, Allen J. Taylor, MD, Jonathan W. Weinsaft, MD, Jose Luis Zamorano, MD, FESC, FASE, Contributing Editors: Harry Dietz, MD, Kim Eagle, MD, John Elefteriades, MD, Guillaume Jondeau, MD, PhD, FESC, Herv e Rousseau, MD, PhD, and Marc Schepens, MD, Washington, District of Columbia; Barcelona and Madrid, Spain; Dallas and Houston, Texas; Bethesda and Baltimore, Maryland; Padua, Pesaro, and Milan, Italy; Cleveland, Ohio; Rochester, Minnesota; Zurich, Switzerland; New York, New York; Essen and Rostock, Germany; Boston, Massachusetts; Ann Arbor, Michigan; New Haven, Connecticut; Paris and Toulouse, France; and Brugge, Belgium (J Am Soc Echocardiogr 2015;28:119-82.) TABLE OF CONTENTS Preamble 121
I. Anatomy and Physiology of the Aorta 121
A. The Normal Aorta and Reference Values 121
1. Normal Aortic Dimensions 122
B. How to Measure the Aorta 124
1. Interface, Definitions, and Timing
of Aortic
Measure- ments
124 From the Medstar Heart Institute at the Washington Hospital Center, Washington, District of Columbia (S.A.G., F.M.A., J.M.L., A.J.T.); Vall d’Hebron University Hospital, Barcelona, Spain (A.E., H.C.-C.); the University of Texas Southwestern Medical Center, Dallas, Texas (S.A.); the National Institutes of Health, Bethesda, Maryland (A.A.); the University of Padua, Padua, Italy (L.P.B.); Cleveland Clinic, Cleveland, Ohio (M.A.B.); Mayo Clinic, Rochester, Minnesota (H.M.C., H.I.M., J.K.O.); the University Hospital Zurich, Zurich, Switzerland (M.C.); Weill Cornell Medical College, New York, New York (R.B.D., J.W.W.); West-German Heart Center, University Duisburg-Essen, Essen, Germany (R.A.E.); San Salvatore Hospital, Pesaro, Italy
(R.F.); Massachusetts General
Hospital, Boston,
Massachusetts (E.M.I.); the Methodist DeBakey Heart & Vascular Center, Houston, Texas; the University of Rostock, Rostock, Germany (C.A.N.); Centro Cardiologico Monzino, IRCCS, Milan, Italy (M.P.); University Hospital Ram on y
Cajal, Madrid, Spain (J.L.Z.); Johns Hopkins University School of Medicine, Baltimore, Maryland (H.D.); the University of Michigan, Ann Arbor, Michigan (K.E.); Yale University School of Medicine, New Haven, Connecticut (J.E.); Hopital Bichat, Paris, France (G.J.); Hopital de Rangueil, Toulouse, France (H.R.); and AZ St Jan Brugge, Brugge, Belgium (M.S.). The following authors reported no actual or potential conflicts of interest in rela- tion to this document: Federico M. Asch, MD, FASE, Michael A. Bolen, MD, Heidi M. Connolly, MD, Hug Cu ellar-Calabria, MD, Martin Czerny, MD, Richard B. De- vereux, MD Harry Dietz, MD, Raimund A. Erbel, MD, FASE, FESC, Arturo Evan- gelista, MD, FESC, Rossella Fattori, MD, Steven A. Goldstein, MD, Guillaume Jondeau, MD, PhD, FESC, Eric M. Isselbacher, MD, Joseph M. Lindsay, MD, Marti McCulloch, MBA, RDCS, FASE, Hector I. Michelena, MD, FASE, Christoph Nienaber, MD, FESC, Mauro Pepi, MD, FESC, Marc Schepens, MD, Allen J. Taylor, MD, and Jose Luis Zamorano, MD, FESC, FASE. The following authors reported relationships with one or more commercial interests: Suhny Abbara, MD, serves as a consultant for Perceptive Informatics. Andrew Arai, MD, re- ceives research support from Siemens. Luigi P. Badano, MD, PhD, FESC, has received software and equipment from GE Healthcare, Siemens, and TomTec for research and testing purposes and is on the speakers’ bureau of GE Health- care. Kim Eagle, MD, received a research grant from GORE. John Elefteriades, MD, has a book published by CardioText and is a principal investigator on a grant and clinical trial from Medtronic. Jae K. Oh, MD, received a research grant from Toshiba and core laboratory support from Medtronic. Herv e Rousseau, MD, serves as a consultant for GORE, Medtronic, and Bolton. Jonathan W. Weinsaft, MD, received a research grant from Lantheus Medical Imaging. Attention ASE Members: The ASE has gone green! Visit www.aseuniversity.org to earn free continuing medical education credit through an online activity related to this article. Certificates are available for immediate access upon successful completion of the activity. Nonmembers will need to join the ASE to access this great member benefit! Reprint requests: American Society of Echocardiography, 2100 Gateway Centre Boulevard, Suite 310, Morrisville, NC 27560 (E-mail:
). 0894-7317/$36.00 Copyright 2015 by the American Society of Echocardiography. http://dx.doi.org/10.1016/j.echo.2014.11.015 119
2. Geometry of Different Aortic Segments: Impact on Measure- ments
126 a. Aortic Annulus 126
b. Sinuses of Valsalva and STJ 126
c. Ascending Aorta and More Distal Seg- ments 126
C. Aortic Physiology and Function
127 1. Local Indices of Aortic Function 127
2. Regional Indices
of Aortic Stiffness: Pulse- wave Velocity (PWV)
128 II. Imaging Techniques 129 A. Chest X-Ray (CXR) 129 B. TTE
129 C. TEE
130 1. Imaging of the
Aorta 130
D. Three-Dimensional Echo- cardiography 131 E. Intravascular Ultrasound (IVUS)
131 1. Limitations 131 F. CT
131 1. Methodology 132 a. CTA
132 i. Noncontrast CT before Aortog- raphy 133
ii. Electrocardiograph- ically Gated CTA 133
iii. Thoracoabdominal CT after Aortog- raphy 133
iv. Exposure to Ionizing Radia- tion 134
v. Measure- ments
134 G. MRI
135 1. Black-Blood Se- quences
135 2. Cine
MRI Se-
quences 135
3. Flow Mapping 135
4. Contrast-Enhanced MR Angiography (MRA) 135
5. Artifacts 136
H. Invasive Aortog-
raphy 136
I. Comparison of Imaging Techniques 137
III. Acute Aortic Syndromes 138
A. Introduction 138
B. Aortic Dissection 138
1. Classification of Aortic Dissection 138
2. Echocardiography (TTE and TEE) 139
a. Echocardiographic Findings 140
b. Detection of Complications 141
c. Limitations of TEE 141
3. CT 141
4. MRI of Aortic Dissection 143
5. Imaging Algorithm 144
6. Use of TEE to Guide Surgery for Type A Aortic Dissection 144
7. Use of Imaging Procedures to Guide Endovascular Ther- apy
146 8. Serial Follow-Up of Aortic Dissection (Choice of Tests) 147 9. Predictors of Complications by Imaging Techniques 148 a. Maximum Aortic Diameter 148 b. Patent False Lumen 148 c. Partial False Luminal Thrombosis 149 d. Entry Tear Size 149 e. True Luminal Compression 149 10. Follow-Up Strategy 149 C. IMH
149 1. Introduction 149 2. Imaging Hallmarks and Features 149 3. Imaging Algorithm 151 4. Serial Follow-Up of IMH (Choice of Tests) 151 5. Predictors of Complications 151 D. PAU
151 1. Introduction 151 2. Imaging Features 151 3. Imaging Modalities 152 a. CT
152 b. MRI
152 c. TEE
152 d. Aortography 152 4. Imaging Algorithm 153 5. Serial Follow-Up of PAU (Choice of Tests) 153 IV. Thoracic Aortic Aneurysm 153 A. Definitions and Terminology 153 B. Classification of Aneurysms 154 C. Morphology 154 D. Serial Follow-Up of Aortic Aneurysms (Choice of Tests) 154 1. Algorithm for Follow-Up 155 E. Use of TEE to Guide Surgery for TAAs 155 F. Specific Conditions 156 1. Marfan Syndrome 156 a. Aortic Imaging in Unoperated Patients with Marfan Syn- drome 156
b. Postoperative Aortic Imaging in Marfan Syndrome 157
c. Postdissection Aortic Imaging in Marfan Syndrome 157
d. Family Screening 157
2. Other Genetic Diseases of the Aorta in Adults 157
a. Turner Syndrome 157
b. Loeys-Dietz Syndrome 157
c. Familial TAAs 157
d. Ehlers-Danlos Syndrome 157
3. BAV-Related Aortopathy 157
a. Bicuspid Valve–Related Aortopathy 157
b. Imaging of the Aorta in Patients with Unoperated BAVs 158
c. Follow-Up Imaging of the Aorta in Patients with Unoperated BAVs
158 d. Postoperative Aortic Imaging in Patients with BAV-Related Aortopathy 158
e. Family Screening 159
V. Traumatic Injury to the Thoracic Aorta 159
A. Pathology 159
B. Imaging Modalities 160
1. CXR 160
2. Aortography 160
3. CT 160
Abbreviations AAS
= Acute aortic syndrome AR = Aortic regurgitation ASE = American Society of Echocardiography BAI
= Blunt aortic injury BSA
= Body surface area CT = Computed tomography CTA = Computed tomographic aortography CXR
= Chest x-ray EACVI
= European Association of Cardiovascular Imaging EAU
= Epiaortic ultrasound GCA
= Giant-cell (temporal) arteritis ICM = Iodinated contrast media IMH
= Intramural hematoma IRAD
= International Registry of Acute Aortic Dissection MDCT = Multidetector computed tomography MIP
= Maximum-intensity projection MR = Magnetic resonance MRI = Magnetic resonance imaging PWV
= Pulsewave velocity STJ
= Sinotubular junction TA = Takayasu arteritis TEE = Transesophageal echocardiography TEVAR
= Transthoracic endovascular aortic repair 3D = Three-dimensional TTE = Transthoracic echocardiography 2D = Two-dimensional ULP = Ulcerlike projection 120 Goldstein et al Journal of the American Society of Echocardiography February 2015
4. TEE 161
5. IVUS 161
6. MRI 162
C. Imaging Algorithm 162
D. Imaging in Endovascular Repair 162
VI. Aortic Coarctation 162
A. Aortic Imaging in Patients with Unoperated Aortic Coarcta- tion
163 B. Postoperative Aortic Imaging in Coarctation 164 VII. Atherosclerosis 164 A. Plaque Morphology and Classification 164 B. Imaging Modalities 165 1. Echocardiography 165 2. Epiaortic Ultrasound (EAU) 165 3. CT
166 4. MRI
166 C. Imaging Algorithm 166 D. Serial Follow-Up of Atherosclerosis (Choice of Tests) 167 VIII.Aortitis 167 A. Mycotic Aneurysms of the Aorta 167 B. Noninfectious Aortitis 168 IX. Postsurgical Imaging of the Aortic Root and Aorta 169 A. What the Imager Needs to Know 169 B. Common Aortic Surgical Techniques 169 1. Interposition Technique 169 2. Inclusion Technique 169 3. Composite Grafts 169 4. Aortic Arch Grafts 169 5. Elephant Trunk Procedure 169 6. Cabrol Shunt Procedure 170 7. Technical Adjuncts 170 C. Normal Postoperative Features 170 D. Complications after Aortic Repair 170 1. Pseudoaneurysm 170 2. False Luminal Dilatation 170 3. Involvement of Aortic Branches 171 4. Infection 171 E. Recommendations for Serial Imaging Techniques and Schedules 171
X. Summary 171
Notice and Disclaimer 171
References 171
PREAMBLE Aortic pathologies are numerous, presenting manifestations are varied, and aortic diseases present to many clinical services, including primary physicians, emergency department physicians, cardiologists, cardiac sur- geons, vascular surgeons, echocardiographers, radiologists, computed tomography (CT) and magnetic resonance (MR) imaging (MRI) im- agers, and intensivists. Many aortic diseases manifest emergently and are potentially catastrophic unless suspected and detected promptly and accurately. Optimal management of these conditions depends on the reported findings from a handful of imaging modalities, including echocardiography, CT, MRI, and to a lesser extent invasive aortography. In the past decade, there have been remarkable advances in nonin- vasive imaging of aortic diseases. This document is intended to provide a comprehensive review of the applications of these noninvasive imaging modalities to aortic disease. Emphasis is on the advantages and disad- vantages of each modality when applied to each of the various aortic diseases. Presently, there is a lack of consensus on the relative role (comparative effectiveness) of these imaging modalities. An attempt has been made to determine first-line and second-line choices for some of these specific conditions. Importantly, we have emphasized the need for uniform terminology and measurement techniques. Whenever possible, these recommendations are evidence based, following a critical review of the literature. In some instances, the recom- mendations reflect a consensus of the expert writing group and include ‘‘vetting’’ by additional experts from the supporting imaging societies. Because of the importance of prompt recognition to their successful treatment, this review emphasizes acute aortic syndromes (AAS), such as aortic dissection and its variants (e.g., intramural hematoma [IMH]), rupture of ascending aortic aneurysm, aortic trauma, and penetrating ulcer. Other entities, such as Takayasu aortitis (TA), giant-cell (temporal) arteritis (GCA), and mycotic aneurysm, are discussed briefly. Less com- mon aortic diseases such as aortic tumors (because of their rarity) and congenital anomalies of the coronary arteries, aortic arch, and sinus of Valsalva aneurysms are not addressed. Several other topics are also beyond the scope of this review, including the important and emerging role of genetics in the evaluation and management of aortic diseases. Moreover, this document is not intended to replace or extend the rec- ommendations of prior excellent guidelines in decision making and management for these conditions. 1 To summarize, the focus of this document is the fundamental role of the major noninvasive imaging techniques. In addition to clinical acumen and suspicion, knowledge of these imaging modalities is crucial for the assessment and management of the often life- threatening diseases of the aorta. I. ANATOMY AND PHYSIOLOGY OF THE AORTA A. The Normal Aorta and Reference Values The aorta is the largest and strongest artery in the body; its wall consists of three layers: the thin inner layer or intima, a thick middle layer or me- dia, and a rather thin outer layer, or adventitia. The endothelium-lined aortic intima is a thin, delicate layer and is easily traumatized. The media is composed of smooth muscle cells and multiple layers of elastic laminae that provide not only tensile strength but also distensibility and elasticity, properties vital to the aorta’s circulatory role. The adven- titia contains mainly collagen as well as the vasa vasorum, which nourish the outer half of the aortic wall and a major part of the media. The elastic properties of the aorta are important to its normal func- tion. The elasticity of the wall allows the aorta to accept the pulsatile output of the left ventricle in systole and to modulate continued forward flow during diastole. With aging the medial elastic fibers undergo thinning and fragmentation. The ordinary concentric arrangement of the laminae is disturbed. These degenerative changes are accompanied by increases in collagen and ground substance. The loss of elasticity and compliance of the aortic wall contributes to the increase in pulse pressure commonly seen in the elderly and may be accompanied by progressive dilatation of the aorta. A geometrically complex organ, the aorta begins at the bulb-shaped root (level 1 in Figure 1
) and then courses through the chest and abdomen in a candy cane–shaped configuration, with a var- iable orientation to the long axis of the body, until it terminates in the iliac bifurcation. The aorta consists of five main anatomic segments: the aortic root, the tubular portion of the ascending aorta, the aortic arch, the descending thoracic aorta, and the abdominal aorta. The most proximal part of the ascending aorta, the aortic root (segment I in
Figure 1 ), includes the aortic valve annulus, aortic valve cusps, Journal of the American Society of Echocardiography Volume 28 Number 2 Goldstein et al 121
coronary ostia, and sinuses of Valsalva. Distally the root joins the tubular portion of the ascending aorta (segment II) at an easily recog- nized landmark termed the sinotubular junction (STJ). The tubular portion of the ascending aorta extends from the STJ to the origin of the brachiocephalic artery. This relatively long segment is subdivided into segment IIa, which extends from the STJ to the pulmonary artery level, and segment IIb, from the pulmonary artery level to the brachio- cephalic artery. The aortic arch (segment III) extends from the bra- chiocephalic artery to the left subclavian artery. The descending thoracic aorta (segment IV) may be subdivided into the proximal part (segment IVa), which extends from the left subclavian artery to the level of the pulmonary artery, and the distal part (segment IVb), which extends from the level of the pulmonary artery to the dia- phragm. The abdominal aorta (segment V) may be subdivided into the proximal part (segment Va), which extends from the diaphragm to the ostia of the renal arteries, and the distal part (segment Vb), from the renal arteries to the iliac bifurcation. 1. Normal Aortic Dimensions. Because of the ease with which it can be visualized and its clinical relevance, 2,3 the aortic root is the segment for which the greatest amount of data are available. Several large studies have reported normal aortic root diameters in the parasternal long-axis view by two-dimensional (2D) transthoracic echocardiography (TTE). 4-10 Measurement of the aortic root diameter should be made perpendicular to the axis of the proximal aorta, recorded from several slightly differently oriented long-axis views. The standard measurement is taken as the largest diameter from the right coronary sinus of Valsalva to the posterior (usually non- coronary) sinus. Most studies report aortic root diameter measure- ments at end-diastole using the leading edge–to–leading edge technique ( Figure 2
). In adults, aortic dimensions are strongly positively correlated with age 5-8,10,11 and body size. 4-6,8,10,11 They are larger in men than in women of the same age and body size. 6,12,13 Although in several reports, aortic diameters have been normalized to body surface area (BSA), 10,13,14 this approach has not been entirely satisfactory because it is systematically lower in smaller than in larger normal adults. Fortunately, among children, the regression line of aortic diameter and height (rather than BSA) has a near-zero intercept, so that normalization to height has proved to be a simple and accurate alternative in growing children. 15 Benchmark values from which the guidelines have been taken 1,16
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