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A. Ehsan and F. W. Sellke

Overview andHistory ofAortic Dissection andOther Acute Aortic Syndromes
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Aortic Anatomy andthePathophysiology
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ofAcute Aortic Syndromes
LaurenV.Huckaby andThomasG.Gleason
Introduction
Fascination with the pathogenesis of acute aortic dissection dates back to the death
of King George II in 1760 who, after collapsing suddenly, was found at autopsy to
have a “transverse ssure on the inner side of the ascending aorta 3.75cm long,
through which blood had recently passed in its external coat to form a raised ecchymosis” [1]. This was one of the rst reports of aortic dissection and underscores key
themes of acute aortic disease that remain true today. Though much progress has
been made in the diagnosis, management, and prevention of aortic catastrophe,
knowledge gaps remain in the understanding of the pathophysiology of thoracic
aortic disease and in the identication of those patients most at risk for acute aortic events.
The foundations for optimal management of patients with thoracic aortic disease
lie in the appreciation of the anatomy and pathophysiology of the thoracic aorta.
Furthermore, recognition of the distinct etiologies and presentations of acute aortic
syndromes is also a key component of management decisions. Consider, for
instance, the case of an acute aortic dissection in a 20-year-old with Marfan syndrome compared to that of a 70-year-old with a degenerative thoracic aortic aneurysm. The underlying etiology will inform intra-operative decision-making and
post-operative care and may be responsible for widely varying outcomes in these
two patients. Technology has also driven a renewed focus on the anatomy and
pathophysiology of aortic disease. Advances in radiologic imaging have increasingly been able to delineate subtle ndings, such as intramural hematoma or
L. V. Huckaby
Department of Surgery, University of Pittsburgh, Pittsburgh, PA, USA
T. G. Gleason (
Division of Cardiac Surgery, Department of Surgery, University of Maryland School of
Medicine, Baltimore, MD, USA
e-mail: tgleason@som.umaryland.edu
J. S. Coselli et al. (eds.), Aortic Dissection and Acute Aortic Syndromes,
https://doi.org/10.1007/978-3-030-66668-2_2
*)
17© Springer Nature Switzerland AG 2021

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penetrating atherosclerotic ulcer, which demand further investigation to accurately
incorporate these ndings into what is already known about the prognosis and natural history of thoracic aortic disease. Adoption of new operative techniques, including endovascular approaches, should also be accompanied by thorough consideration
of the characteristics of the native aorta. This chapter reviews the embryology, anatomy, histology and pathophysiology of the normal and diseased thoracic aorta
(Fig.1).
L. V. Huckaby and T. G. Gleason
Embryology
The third week of gestation marks the onset of development of the aorta and great
vessels. By the eighth week, coordinated regression and persistence of the embryologic branches has resulted in the formation of an aortic arch more closely resembling that seen in the adult (Fig.2). The thoracic aorta and its branches begin as two
aortae, the dorsal and ventral, which are connected by six paired branchial arch
arteries. These arches form in a craniocaudal manner. By the end of the fourth week,
only the third, fourth and six arches are still present. The rst and second arches
have regressed, and the fth arch never fully forms. By the fth week, the internal
carotid arteries form from the third arch. The dorsal aorta between the third and
fourth aortic arches begins to atrophy, and this is complete by the end of the sixth
week. The seventh cervical intersegmental arteries, which arise from the dorsal
aorta, enlarge to contribute to the bilateral subclavian arteries. Additionally, by the
end of the sixth week, the pulmonary arteries are formed from the ventral portion of
the sixth aortic arch. While the right-sided dorsal portion of the sixth arch ultimately
atrophies, the left dorsal sixth arch gives rise to the ductus arteriosus.
At this point, asymmetric development is responsible for the most common aortic conguration, with the aorta taking a leftward course. The aortic arch and proximal descending aorta develop from the left fourth aortic arch and left dorsal aorta,
the right dorsal aorta having atrophied. The right subclavian artery develops from
the right fourth aortic arch, a portion of the remaining right dorsal aorta and the
intersegmental artery. The innominate artery on the right and the left common
carotid artery form from the ventral aorta. The left subclavian artery arises from a
left intersegmental artery. At the end of the eighth week, the primitive aorta resembles that seen in the adult.
Neural crest cells migrate to the primitive aortic arches to contribute to branching
patterns and ultimately give rise to medial smooth muscle cells. Neuroectoderm has
a signicant contribution in aortopulmonary septation, driving differentiation of the
aortic root and pulmonary trunk. The most proximal aspect of the aorta appears to
have a greater inuence from neural crest cells compared to the arch and descending
aorta, although the exact role of neural crest in aortic development has yet to be
fully elucidated. The origin of the coronary arteries is not rmly established but they
likely arise from the epicardium of the heart and later connect with the aorta. The
aortic valve itself is primarily derived from mesodermal cells.

Aortic arch
Ascending
ta
Aortic Anatomy andthePathophysiology ofAcute Aortic Syndromes
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19
aorta
Right subclavian
Innominate artery
Tu bular
ascending
aorta
Aortic root
artery
Right
coronary
artery
Right common
carotid artery
Zone 0
Sinuses
of valsalva
Zone 1
Left
coronary
artery
Diaphragm
Left common
carotid artery
Zone 2
Zone 3
Zone 5
Zone 4
Left subclavian
artery
Descending
thoracic aor
Fig. 1 Thoracic aorta. The thoracic aorta, from the aortic valve to the diaphragmatic hiatus, is
divided into the ascending aorta (including the aortic root and tubular ascending aorta), aortic arch
and descending aorta. The aortic root includes the aortic valve, aortic annulus, sinuses of Valsalva
and the origins of the right and left coronary arteries
Regions oftheThoracic Aorta
The thoracic aorta can be subdivided into three regions: the ascending aorta, the
aortic arch, and the descending thoracic aorta, and these have more recently been
subdivided into six zones (Fig.1). Aberrations in course and branching patterns may
occur as a result of altered embryological development. The most common

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6 Weeks 7 Weeks
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L. V. Huckaby and T. G. Gleason
Embryonic 5 Weeks
3rd Pharyngeal arches
4th Pharyngeal arches
6th Pharyngeal arches
Ventral aorta
Dorsal aorta
Fig. 2 Embryology of the thoracic aorta. The thoracic aorta is derived from the dorsal and ventral
aortae and the paired pharyngeal vascular arches. Coordinated regression and persistence of the
arches determines the course of the aortic arch and the conguration of the branches

Aortic Anatomy andthePathophysiology ofAcute Aortic Syndromes
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congurations will be described rst, and further attention will be directed to these
anomalies in the next section.
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Aortic Root andAscending Aorta
The ascending aorta is composed of the aortic root and the tubular ascending aorta.
The aortic root is the most proximal component and begins at the aortic annulus, a
brous ring that supports the aortic valve and marks the transition from the left
ventricle. From there, the aorta balloons slightly outward to form the three sinuses
of Valsalva. The origins of the right and left coronary arteries are present at these
sinuses and the aortic cusps and sinuses are named accordingly: right, left and noncoronary. The sinotubular junction delineates the transition between the aortic root
and the beginning of the tubular ascending aorta. At this point, the aorta assumes a
mostly uniform caliber and begins a superior path towards the arch. The aortic
diameter is positively correlated with age. Variations in imaging modalities and in
the level of aortic measurements in relation to other structures can inuence reported
values of normal aortic diameter. With these caveats, a study of 1442 subjects undergoing computed tomography angiography (CTA) showed that the ascending aorta at
the level of the mid-pulmonary artery measures approximately 3.1cm in females
and is larger at approximately 3.4cm in males [2]. In the retrosternal position, the
remnant of the thymus overlies the aorta. The right atrial appendage lies anteromedial to the proximal ascending aorta with the pulmonary trunk coursing posterolaterally. Immediately to the right of the ascending aorta lies the superior vena cava
(SVC). The transition from the ascending aorta to the arch also marks the superior
extent of the pericardium.
Aortic Arch
The aortic arch commences at the origin of the innominate (brachiocephalic)
artery which arises from the superior aspect of the aorta (Fig.3a). This trunk is
situated slightly anterior to the other arch vessels and takes a right and posterior
course where it branches into the right subclavian and right common carotid arteries. The aortic arch curves posteriorly and to the left with its superior convexity
reaching the level of the mid-manubrium. The subsequent great vessel branches
from the arch are the left common carotid and left subclavian arteries, each arising
successively posterior to the takeoff of the innominate artery. The left brachiocephalic vein courses anteriorly to all three head vessels at their most proximal
aspects. The arch is intimately related to multiple mediastinal structures. It passes
immediately anterior to the trachea (Fig.3b). The left phrenic nerve runs anteriorly
at the distal portion of the arch, and the right phrenic nerve lies lateral to the
SVC.The right vagus nerve passes anterior to the right subclavian artery, giving

22
Innominate
vian
Right vagus
Right phrenic
Left vagus
Left phrenic
Ligamentum
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L. V. Huckaby and T. G. Gleason
Fig. 3 Thoracic aorta and
surrounding structures. (a)
shows the aortic root and
tubular ascending aorta.
(b) shows the arch and
proximal descending aorta.
Note the proximity of the
thoracic aorta to the
pulmonary arteries,
superior vena cava, vagus
and phrenic nerves, trachea
and esophagus. The
vertebral column (not
shown) lies directly
posterior and to the right of
the descending thoracic
aorta
a
Superior
vena cava
Pulmonary
b
Trachea
nerve
Aorta
trunk
artery
nerve
nerve
nerve
arteriosum
Esophagus
Left common
carotid artery
Ascending
aorta
Esophagus
Left subcla
artery

Aortic Anatomy andthePathophysiology ofAcute Aortic Syndromes
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off its recurrent branch and then descending posterior to the right pulmonary
hilum. The left vagus nerve passes anterior to the arch just at or medial to the takeoff of the left subclavian artery and gives off the left recurrent laryngeal branch
which continues inferiorly along the concave border of the arch just lateral to the
ligamentum arteriosum, the brous remnant of the fetal ductus arteriosus and then
ascends in the tracheoesophageal groove. The left pulmonary artery lies in close
contact to the lesser curve of the arch. The aortic arch ends at approximately the
T4-T5 vertebral level.
23
Descending Thoracic Aorta
The descending thoracic aorta begins after the origin of the left subclavian artery at
vertebral level T4. This section runs immediately to the left of the vertebral bodies
early in its course and gradually assumes a more medial position so that it lies
almost at midline as it passes through the diaphragm. The descending aorta gives
rise to pericardial, bronchial, esophageal, mediastinal, intercostal, and superior
phrenic artery branches. The esophageal artery branches provide blood supply to
the mid-esophagus. The posterior intercostal artery branches consist of nine paired
vessels running along the undersurface of the lower nine intercostal spaces. The
artery of Adamkiewicz originates from the 9th through 12th intercostal arteries and
feeds the anterior spinal artery. In cases of descending thoracic aorta and thoracoabdominal aorta repair, reimplantation of intercostal vessels may be necessary to
ensure adequate spinal perfusion via this artery. The descending aorta enters the
diaphragm at the aortic hiatus at T12. The mid-descending thoracic aorta diameter
is slightly smaller than the normal ascending aorta and measures approximately
2.70cm in men and 2.46cm in women [3].
Congenital Anomalies oftheThoracic Aorta
Left Aortic Arch withAberrant Branching
In the majority of the population, the aortic arch takes a leftward course and descends
to the left of the thoracic spine (Fig.4a). With this conguration, up to 30% of the
population display a bovine arch in which the left common carotid arises from the
innominate artery (Fig.4b). The aortic arch may also have four branches, as is the
case with an aberrant right subclavian artery. With this, the right subclavian originates on the arch distal to the left subclavian, often from a dilated portion termed a
diverticulum of Kommerell (Fig.4c). It then runs posterior to the esophagus. The
etiology of this conguration is an early regression of the right fourth pharyngeal
arch in combination with persistence of the eighth segment of the dorsal aorta. The
majority of individuals are asymptomatic, however dilatation of the aberrant

24
Right
Right
Left
Left
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L. V. Huckaby and T. G. Gleason
SCA
Right
SCA
Right
SCA
Right
CCA
Right
SCA
Right
CCA
Left
CCA
Left
CCA
Left
SCA
Left
SCA
Right
CCA
CCA
Left
CCA
CCA
Left
SCA
SCA
Left
pulmonar
artery
Fig. 4 Congenital variants of the aortic arch and branches. The most common aortic conguration
(a) consists of a left-sided aorta which may display aberrant branching such as a bovine arch (b) or
an aberrant right subclavian artery (c). A right-sided aortic arch (d) results from persistence of the
right fourth pharyngeal arch whereas a double aortic arch (e) occurs as a result of persistence of both
the right and left fourth pharyngeal arches. Common carotid artery (CCA), subclavian artery (SCA)
e
Trachea
Right arch
Right
CCA
Right
SCA
Main pulmonary
artery
Esophagus
Left SCA
Left CCA
Left
arch
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