DOI:10.2214/AJR.07.3408
AJR 2008; 190:1467-1474
© American Roentgen Ray Society
Interrupted Aortic Arch: Spectrum of MRI Findings
Jonathan R. Dillman1,
Sai G. Yarram2,
Anthony R. D'Amico1 and
Ramiro J. Hernandez2
1 Department of Radiology, University of Michigan Health System, University
Hospital, 1500 E Medical Center Dr., Ann Arbor, MI 48109.
2 Section of Pediatric Radiology, University of Michigan Health System, C. S.
Mott Children's Hospital, Ann Arbor, MI.
Received November 4, 2007;
accepted after revision December 25, 2007.
Address correspondence to J. R. Dillman
(jonadill{at}med.umich.edu).
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Abstract
OBJECTIVE. The objective of this article is to review the types,
pathogenesis, MRI appearance, treatment, and prognosis of interrupted aortic
arch (IAA).
CONCLUSION. IAA is a rare congenital vascular anomaly. Although this
entity has been evaluated traditionally with echocardiography and angiography,
MRI can accurately diagnose and characterize the various forms of IAA and
associated congenital heart defects. MRI can also be used to evaluate for
postoperative complications after repair.
Keywords: aortic arch • cardiac imaging • congenital anomalies • interrupted aortic arch • MRI • vascular imaging
Introduction
Interrupted aortic arch (IAA) is defined as a lack of luminal
continuity between the ascending and descending thoracic aorta. This
discontinuity may be complete or it may be spanned by an atretic fibrous band
[1]. The condition is extremely
rare, representing less than 1.5% of congenital heart disease cases
[2]. Approximately 3-20 in 1
million live births are affected by a form of IAA. This vascular anomaly was
initially described in 1778 [3]
and was first surgically repaired in 1954
[4].
Types of Interrupted Aortic Arch
Traditionally, IAA has been classified into three discrete types on the
basis of the location of the aortic arch discontinuity
[1,
5] (Figs.
1A,
1B, and
1C). Type A is an interruption
just distal to the left subclavian artery and traditionally makes up
approximately one third of IAA cases. The type B defect occurs between the
left common carotid and left subclavian arteries and is responsible for
approximately two thirds of the cases. Type C is the rarest type, occurring in
less than 5% of IAA cases. This form is the most proximal defect, occurring
between the innominate and left common carotid arteries. Schreiber et al.
[6] reviewed 95 cases of IAA
and classified 13% as type A, 84% as type B, and 3% as type C.
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Fig. 1A —Drawings show three types of interrupted aortic arch. Arrow =
patent ductus arteriosus. Type A interruption occurs just distal to left
subclavian artery. Patent ductus arteriosus provides blood flow to descending
thoracic aorta.
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Fig. 1B —Drawings show three types of interrupted aortic arch. Arrow =
patent ductus arteriosus. Type B interruption occurs between left common
carotid and left subclavian arteries. Patent ductus arteriosus provides blood
flow to left subclavian and descending thoracic arteries.
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Fig. 1C —Drawings show three types of interrupted aortic arch. Arrow =
patent ductus arteriosus. Type C interruption occurs between innominate and
left common carotid arteries. Patent ductus arteriosus provides blood flow to
left common carotid, left subclavian, and descending thoracic arteries.
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Pathogenesis and Associated Cardiac Defects
Although the exact cause of IAA is uncertain, several theories have been
proposed. These theories most commonly are based on the supposition that blood
flow during embryogenesis directly affects the enlargement and involution of
blood vessels [1]. Some
investigators have theorized that conditions that cause abnormally decreased
blood flow through the aortic arch contribute to the development of IAA.
Regarding embryology, type A is likely the result of abnormal regression of
the left fourth aortic arch after ascension of the left subclavian artery to
its expected position. Type B occurs when the left fourth aortic arch
regresses before normal ascension of the left subclavian artery to its
expected position. Type C is seen when the ventral portion of the left third
aortic arch and left fourth aortic arch involute, and there is a persistent
ductus caroticus, a structure that normally regresses
[1].
IAA is associated with additional cardiovascular anatomic defects in up to
98% of the cases. The most commonly observed cardiovascular anomaly is a
patent ductus arteriosis, occurring in approximately 97% of patients with IAA
[1]. This vascular structure is
required to supply blood flow beyond the interruption to the descending
thoracic aorta. Ventricular septal defects are also typically present,
occurring in approximately 90% of individuals with IAA
[7,
8]. Conditions that decrease
blood flow to the aortic arch, including subaortic stenosis, bicuspid aortic
valve, truncus arteriosis, and aortopulmonary window, have been associated
with IAA [1,
7,
9]. Rarely, IAA is an isolated
finding without another associated cardiac defect, which suggests the
possibility that an extrinsic compressive or mechanical force is
causative.
Approximately 50% of IAA cases are associated with a chromosome 22q11.2
deletion, particularly in the presence of a right descending thoracic aorta.
This chromosomal abnormality is seen in up to 75% of patients with type B IAA.
Conversely, it is relatively rare in patients with type A, which suggests
either another genetic or a mechanical cause. This chromosomal deletion is
observed in both DiGeorge and velocardiofacial syndromes, and it is associated
with a variety of conotruncal cardiac anomalies. IAA affects up to 42% of
individuals with DiGeorge syndrome
[1,
9].
Imaging of Interrupted Aortic Arch
Proper surgical planning requires an accurate diagnostic imaging evaluation
to correctly characterize aortic and cardiac anatomy and define the exact type
of IAA. Anatomic features that must be identified include the following:
location and length of the aortic vascular defect, caliber of the thoracic
aorta proximal and distal to the interruption, branching pattern and origins
of the great vessels, location and patency of the ductus arteriosus,
appearance of the ventricular outflow tracts, and presence of any other
cardiac anomalies.
Multiple imaging techniques have been described in the evaluation of
patients with suspected IAA. Echocardiography is considered to be the primary
imaging technique for the workup of this entity
[10,
11]. It has the advantages of
being portable and not using ionizing radiation. Although this imaging
technique usually provides excellent anatomic definition of the heart,
evaluation of the aorta and great vessels can occasionally be limited. Thus,
echocardiography may or may not be able to define the exact site of the aortic
arch interruption and its relationship to the origins of the great
vessels.
In the past, catheter angiography also performed an important role in the
evaluation of patients with suspected IAA
[12]. However, this imaging
technique both is invasive and requires ionizing radiation. The use of
noninvasive CT angiography has recently been described in the evaluation of
this entity [13,
14], although concerns about
radiation exposure also afflict this imaging technique. CT angiography may be
useful in certain circumstances, such as when there is no access to
echocardiography or MRI.
The use of MRI, including MR angiography, has been described in the
evaluation of numerous complex congenital heart defects including IAA
[11,
15-18].
MRI can accurately characterize cardiovascular anatomy, including that of the
thoracic aorta and great vessels and coexisting cardiac anomalies. In
addition, MRI can also provide useful information regarding cardiac chamber
and valve function. Like echocardiography, MRI is noninvasive and does not
require ionizing radiation. Sedation or general anesthesia may be required to
limit motion-related artifacts in the pediatric population.
Multiple MRI techniques can be used to evaluate the thoracic aorta and
heart in cases of suspected IAA. MRI sequences can be performed both with and
without IV gadolinium-containing contrast material. Unenhanced evaluation may
include double inversion recovery fast spin-echo "black blood,"
gradient-recalled echo "white blood," and balanced (e.g., 2D and
3D balanced-steadystate free precession [SSFP]) imaging sequences. Balanced
SSFP imaging sequences can be performed in any plane, including a sagittal
oblique plane that displays both ascending and descending thoracic aorta, and
may be acquired as cine loops. Gadolinium-enhanced 3D gradient-recalled echo
MR angiography sequences are also frequently used, and they can be performed
in either the sagittal or coronal plane.
Several MRI findings suggest the diagnosis of IAA. The most specific
imaging finding is nonvisualization of a portion of the aortic arch (Figs.
2A,
2B,
2C,
2D,
2E,
2F,
3A,
3B,
3C,
3D,
4A,
4B,
4C,
4D,
5A,
5B,
5C,
6A,
6B,
6C, and
6D). Such a defect should be
confirmed on more than one imaging sequence and in multiple planes if
possible. Multiplanar reformatted imaging can be helpful when using
gadolinium-enhanced 3D MR angiography and 3D balanced SSFP imaging.
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Fig. 2A —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus. Coronal
gadolinium-enhanced MR angiography image shows small-caliber ascending aorta
(AA) arising from left ventricle. Right common carotid artery (RCCA) and main
pulmonary artery (MPA) can also be seen. Incidental note is also made of
venous contamination (V).
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Fig. 2B —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus. Left common
carotid artery (LCCA) arises from aortic arch, and origin of right pulmonary
artery (RPA) is visualized at same level.
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Fig. 2C —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus. Aortic arch
terminates as left subclavian artery (LSCLA). There is apparent interruption
(INT) of aortic arch between left subclavian artery and descending thoracic
aorta. Both right and left vertebral arteries (RVA and LVA, respectively) are
also seen.
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Fig. 2D —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus. Large patent
ductus arteriosus (PDA) arises from left pulmonary artery (LPA).
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Fig. 2E —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus. Patent ductus
arteriosus (PDA) provides blood flow to right descending thoracic aorta
(DA).
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Fig. 2F —1-week-old female neonate with type A interrupted aortic
arch, ventricular septal defect, and patent ductus arteriosus.
Maximum-intensity-projection image also shows site of aortic arch interruption
(INT).
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Fig. 3A —1-week-old male neonate with type B interrupted aortic arch
(IAA) and ventricular septal defect. Coronal double inversion recovery fast
spin-echo black blood MR image reveals normal ascending aorta (AA) arising
from left ventricle. Main pulmonary artery (MPA) and ventricular septal defect
(VSD) are also visualized.
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Fig. 3B —1-week-old male neonate with type B interrupted aortic arch
(IAA) and ventricular septal defect. MR image shows right and left common
carotid arteries (RCCA and LCCA, respectively) arise from proximal aortic
arch.
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Fig. 3C —1-week-old male neonate with type B interrupted aortic arch
(IAA) and ventricular septal defect. Slightly more posterior within thorax,
patent ductus arteriosus (PDA) arises from left pulmonary artery (LPA). Right
pulmonary artery (RPA) and left atrium (LA) are also seen at this level.
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Fig. 3D —1-week-old male neonate with type B interrupted aortic arch
(IAA) and ventricular septal defect. Sagittal black blood MR image shows
vascular arch that is almost completely visualized in single sagittal plane.
This structure is formed from main pulmonary artery (MPA) and patent ductus
arteriosus (PDA) and appears flattened compared with normal aortic arch,
confirming presence of IAA. Patent ductus arteriosus provides blood flow to
descending thoracic aorta (DA). Left pulmonary artery (LPA) is also seen.
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Fig. 4A —3-day-old male neonate with type B interrupted aortic arch,
large aortopulmonary window, and pulmonary sling. Coronal gradient-recalled
echo "white blood" MR image shows abnormal communication between
ascending aorta (AA) and main pulmonary artery (MPA), so-called aortopulmonary
window (APW).
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Fig. 4B —3-day-old male neonate with type B interrupted aortic arch,
large aortopulmonary window, and pulmonary sling. MR image shows innominate
artery (IA) and left common carotid artery (LCCA) arising from proximal aortic
arch in V configuration.
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Fig. 4C —3-day-old male neonate with type B interrupted aortic arch,
large aortopulmonary window, and pulmonary sling. MR image shows that left
pulmonary artery (LPA) arises from right pulmonary artery (RPA) at level of
left atrium (LA), confirming presence of pulmonary sling. Patent ductus
arteriosus (PDA) provides blood flow to descending thoracic aorta (DA).
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Fig. 4D —3-day-old male neonate with type B interrupted aortic arch,
large aortopulmonary window, and pulmonary sling. Sagittal gradient
recalled-echo white blood MR image shows complete vascular arch in single
sagittal plane is formed from pulmonary artery and patent ductus arteriosus
(PDA). DA = descending thoracic aorta.
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Fig. 5A —4-day-old female neonate with DiGeorge syndrome, type 2
truncus arteriosus, surgically confirmed interrupted aortic arch, postductal
origins of left carotid and left subclavian arteries, and aberrant
retroesophageal right innominate arteries. Sagittal oblique subvolume
maximum-intensity-projection (MIP) gadolinium-enhanced 3D MR angiography image
reveals that ascending aorta and main pulmonary artery arise from single
outflow tract, consistent with truncus arteriosus (TA). Patent ductus
arteriosus (PDA) directly communicates with descending thoracic aorta (DA).
Both right and left common carotid arteries (RCCA and LCCA, respectively)
arise from postductal aorta. This interrupted aortic arch branching pattern
does not fit criteria for any of the three previously described types. Normal
aortic arch is not seen.
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Fig. 5B —4-day-old female neonate with DiGeorge syndrome, type 2
truncus arteriosus, surgically confirmed interrupted aortic arch, postductal
origins of left carotid and left subclavian arteries, and aberrant
retroesophageal right innominate arteries. Subvolume MIP image in sagittal
obliquity slightly different from A confirms that right and left
pulmonary arteries (RPA and LPA, respectively) arise separately from common
trunk, consistent with type 2 truncus arteriosus (TA). Origin of patent ductus
arteriosus (PDA) is also seen. DA = descending thoracic aorta.
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Fig. 5C —4-day-old female neonate with DiGeorge syndrome, type 2
truncus arteriosus, surgically confirmed interrupted aortic arch, postductal
origins of left carotid and left subclavian arteries, and aberrant
retroesophageal right innominate arteries. Axial subvolume MIP image confirms
postductal aberrant retroesophageal innominate artery (IA) gives rise to right
common carotid and right subclavian arteries. PDA = patent ductus arteriosus,
TA = truncus arteriosus.
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Fig. 6A —3-day-old female neonate with interrupted aortic arch,
aberrant left subclavian artery from left patent ductus arteriosus, aberrant
right subclavian artery from descending thoracic aorta, and right descending
thoracic aorta. Gadolinium-enhanced 3D MR angiography images show
small-caliber ascending aorta (AA in A) arising from left ventricle.
Right and left common carotid arteries (RCCA and LCCA, respectively) form V
configuration.
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Fig. 6B —3-day-old female neonate with interrupted aortic arch,
aberrant left subclavian artery from left patent ductus arteriosus, aberrant
right subclavian artery from descending thoracic aorta, and right descending
thoracic aorta. Gadolinium-enhanced 3D MR angiography images show
small-caliber ascending aorta (AA in A) arising from left ventricle.
Right and left common carotid arteries (RCCA and LCCA, respectively) form V
configuration.
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Fig. 6C —3-day-old female neonate with interrupted aortic arch,
aberrant left subclavian artery from left patent ductus arteriosus, aberrant
right subclavian artery from descending thoracic aorta, and right descending
thoracic aorta. Coronal oblique subvolume maximum-intensity-projection (MIP)
and volume-rendered images confirm presence of interrupted aortic arch. Right
patent ductus arteriosus (RPDA) supplies blood flow to descending thoracic
aorta (DA). Left subclavian artery (LSCLA) arises from small left patent
ductus arteriosus (LPDA), and right subclavian artery (RSCLA) arises from
postductal descending thoracic aorta. This interrupted aortic arch branching
pattern does not fit criteria for any of the three previously described types.
MPA = main pulmonary artery; in C, RPA = right pulmonary artery; in
D, AA = ascending aorta, RCCA = right common carotid artery, LCCA =
left common carotid artery.
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Fig. 6D —3-day-old female neonate with interrupted aortic arch,
aberrant left subclavian artery from left patent ductus arteriosus, aberrant
right subclavian artery from descending thoracic aorta, and right descending
thoracic aorta. Coronal oblique subvolume maximum-intensity-projection (MIP)
and volume-rendered images confirm presence of interrupted aortic arch. Right
patent ductus arteriosus (RPDA) supplies blood flow to descending thoracic
aorta (DA). Left subclavian artery (LSCLA) arises from small left patent
ductus arteriosus (LPDA), and right subclavian artery (RSCLA) arises from
postductal descending thoracic aorta. This interrupted aortic arch branching
pattern does not fit criteria for any of the three previously described types.
MPA = main pulmonary artery; in C, RPA = right pulmonary artery; in
D, AA = ascending aorta, RCCA = right common carotid artery, LCCA =
left common carotid artery.
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Another relatively specific imaging finding for IAA is visualization of a
single complete thoracic vascular arch on a single sagittal image (resembling
a "normal" aortic arch) (Figs.
3A,
3B,
3C,
3D,
4A,
4B,
4C and
4D). As a rule, the aortic
arch cannot be seen in its entirety on a single sagittal image because the
vessel typically courses within the thorax from anterior and right to
posterior and left. In patients with IAA, the patent ductus arteriosus may
mimic a "normal" aortic arch. This structure is oriented in an
anteroposterior direction, and it is best seen on sagittal imaging
communicating between the pulmonary artery and aorta. Consequently, sagittal
MRI can be misleading if not reviewed carefully and may erroneously suggest
the presence of an intact aortic arch in the setting of interruption. A
visualized patent ductus arteriosus typically lacks the classic morphologic
appearance of a normal aortic arch; instead it appears somewhat flat (Figs.
3A,
3B,
3C,
3D,
4A,
4B,
4C,
4D,
5A,
5B and
5C).
Additional MRI findings can also be observed in the setting of IAA. The
great vessels may show a V configuration on coronal imaging (Figs.
3A,
3B,
3C,
3D,
4A,
4B,
4C,
4D, and
6A,
6B,
6C,
6D). The ascending thoracic
aorta may be smaller in caliber than expected due to decreased blood flow
(Figs. 2A,
2B,
2C,
2D,
2E,
2F,
6A,
6B,
6C and
6D). Also, note that a right
aortic arch with a left descending thoracic aorta and hypo plastic
retroesophageal segment may mimic the appearance of IAA
[19] (Figs.
7A and
7B). MRI should be used to
distinguish this entity from IAA because surgical correction may differ.
Rarely, a form of IAA may be encountered that does not fit the exact criteria
for any of the three described types (Figs.
5A,
5B,
5C,
6A,
6B,
6C and
6D).
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Fig. 7A —2-day-old female neonate with Down syndrome, right aortic
arch with left descending thoracic aorta and hypoplastic retroesophageal
segment, ventricular septal defect, and bilateral superior venae cavae.
Coronal maximum-intensity-projection gadolinium-enhanced 3D MR angiography
image shows apparent interruption (INT) of aortic arch between left common
carotid artery (LCCA) and left subclavian artery (LSCLA). Right pulmonary
artery (RPA) is seen. PDA = patent ductus arteriosus, DA = descending thoracic
aorta.
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Fig. 7B —2-day-old female neonate with Down syndrome, right aortic
arch with left descending thoracic aorta and hypoplastic retroesophageal
segment, ventricular septal defect, and bilateral superior venae cavae.
Volume-rendered image confirms presence of hypoplastic retroesophageal aortic
segment (REA) on closer inspection. Patent ductus arteriosus (PDA) also
supplies blood flow to descending thoracic aorta (DA). MPA = main pulmonary
artery.
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Prognosis and Treatment
IAA is associated with a mortality rate of more than 90% at 1 year of age
if untreated [4]. Mean patient
survival is approximately 4-10 days
[20]. Death is typically due
to a combination of increasing left-to-right shunt, ventricular failure, and
ductus arteriosus closure. Closure of the ductus arteriosus results in
hypoperfusion-related complications, including renal failure and metabolic
acidosis [1]. Rarely, patients
with IAA can present in adulthood due to the presence of unusual collateral
vessels [1,
21,
22].
The initial treatment of IAA is IV prostaglandin therapy to preserve ductus
arteriosus patency and blood flow beyond the interruption. Surgical correction
is then performed after appropriate imaging evaluation and operative planning.
Multiple surgical reparative techniques are available, including both
one-stage and multistage procedures. A one-stage repair includes direct aortic
arch primary anastomosis (either end-to-end or end-to-side) with or without
placement of synthetic graft material across the defect and possible
ventricular septal defect repair. A multistage procedure is often considered
in the setting of IAA and associated complex congenital heart disease. The
initial stage typically involves aortic arch reconstruction and pulmonary
artery banding in the presence of a ventricular septal defect. At least one
more surgery is then required to remove the pulmonary artery band and repair
additional cardiac defects
[23-25].
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Fig. 8 —21-year-old man with history of type A interrupted aortic
arch (IAA) being evaluated following surgical repair. Sagittal
maximum-intensity-projection (MIP) gadolinium-enhanced 3D MR angiography image
shows vertically oriented ascending aorta (A) giving rise to origins of all
great vessels (arrow). Synthetic patch is seen bridging area of
aortic arch interruption and supplies blood flow to descending aorta (DA).
Main pulmonary artery (PA) is also seen.
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Fig. 9A —14-year-old boy with history of type A interrupted aortic
arch (IAA) being evaluated for status after repair of IAA. Synthetic graft
material was used to bridge interrupted portion of aortic arch. Axial double
inversion recovery fast spin-echo black blood MR image reveals area of
proximal anastomotic narrowing (arrow).
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Fig. 9B —14-year-old boy with history of type A interrupted aortic
arch (IAA) being evaluated for status after repair of IAA. Synthetic graft
material was used to bridge interrupted portion of aortic arch. Sagittal
oblique 2D balanced steady-state free precession image shows that ascending
aorta (A) is not dilated proximally. Finding seen in A (arrow)
is confirmed.
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After surgical correction, estimates of early mortality rates have ranged
from less than 8% to 37% [6,
23,
24]. Long-term survival has
increased over the past 3 decades and now approaches 92% 1 year after surgical
repair [6,
24]. Because patients with IAA
are living longer, MRI is particularly useful in the postoperative setting to
detect complications such as anastomotic narrowing and graft aneurysms (Figs.
8,
9A and
9B). MRI is invaluable in the
evaluation of older children and adults with repaired IAA because suitable
echocardiography windows may not be available to evaluate the aortic arch and
great vessels.
Conclusions
IAA is a rare form of congenital vascular anomaly. Although
echocardiography is more commonly used to evaluate this condition, MRI can
play a complementary role because of its ability to accurately define aortic
and cardiac anatomy. MRI can be used to identify the site and length of aortic
arch interruption, the origins of the great vessels, and associated congenital
cardiac defects. MRI may be particularly beneficial in the workup of suspected
IAA when the defect cannot be adequately evaluated by echocardiography and in
the postoperative setting.
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Abstract
Introduction
