- Email: [email protected]
Coarctation of the aorta in adolescents and adults: A review of clinical features and CT imaging
Accepted Manuscript Coarctation of the Aorta in Adolescents and Adults: A Review of Clinical Features and CT Imaging John W. Nance, MD, Richard E. Ringel, MD, Elliot K. Fishman, MD, Professor of Radiology, Oncology, and Surgery PII:
S1934-5925(15)30016-2
DOI:
10.1016/j.jcct.2015.11.002
Reference:
JCCT 845
To appear in:
Journal of Cardiovascular Computed Tomograph
Received Date: 12 March 2015 Revised Date:
12 July 2015
Accepted Date: 10 November 2015
Please cite this article as: Nance JW, Ringel RE, Fishman EK, Coarctation of the Aorta in Adolescents and Adults: A Review of Clinical Features and CT Imaging, Journal of Cardiovascular Computed Tomograph (2015), doi: 10.1016/j.jcct.2015.11.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Coarctation of the Aorta in Adolescents and Adults: A Review of Clinical Features and CT Imaging
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Short title: Aortic coarctation in adolescents and adults John W. Nance, MD1; Richard E. Ringel, MD2; Elliot K. Fishman, MD1
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1 – Russell H. Morgan Department of Radiology and Radiological Science Johns Hopkins School of Medicine 601 N. Caroline St Baltimore, MD
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2 – Department of Pediatrics Johns Hopkins School of Medicine 601 N. Caroline St Baltimore, MD
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Corresponding author: Elliot K. Fishman, MD Professor of Radiology, Oncology, and Surgery Department of Radiology and Radiological Sciences Johns Hopkins University School of Medicine 601 N. Caroline St, JHOC 3254 Baltimore, MD 21287 Phone: 410-955-5173 Fax: 410-614-0341 Email: [email protected]
Conflicts of interest related to this article: None. Conflicts of interest not related to this article: Dr. Fishman receives grant support from Siemens and GE. The other authors have no potential conflicts of interest to disclose.
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Coarctation of the Aorta in Adolescents and Adults: A Review of
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Clinical Features and CT Imaging
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Abstract Coarctation of the aorta (CoA), while usually identified and treated in the neonatal/infant period, is increasingly seen in adults, either primarily or (more
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often) following repair. Imaging plays a crucial role in the diagnosis, therapeutic planning, and follow-up of patients with CoA. Clinical management of CoA in
adults optimally involves a multidisciplinary team; accordingly, imagers should be
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familiar with the underlying pathology, associations, and management of CoA in addition to imaging protocoling and interpretation. We will review the relevant
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clinical and imaging features of CoA, with an emphasis on patients beyond
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childhood.
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Key Words: Coarctation of the aorta; congenital heart disease; adult congenital heart disease;
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computed tomography; cardiac computed tomography.
List of abbreviations: CHD – Congenital heart disease
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CoA – Coarctation of the aorta
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1. Introduction First described by Morgagni in 1760, coarctation of the aorta (CoA; from the Latin term coarctare, “to contract”)1 occurrs in 0.4% of live births and is
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recognized in 7% of patients with congenital heart disease (CHD)2. There is a male predominance, with the male to female ratio reported as 1.5:1.3 While
severe cases usually present in the neonatal period, the diagnosis of CoA is
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often not made until adolescence or adulthood. Furthermore, early repair of CoA is subject to recoarctation, necessitating familiarity of the disease and its imaging
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manifestations for both pediatric and adult imagers. The current review will summarize the relevant clinical and CT features of CoA, with an emphasis on
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patients beyond childhood.
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2. Pathology The underlying pathogenesis of CoA is not well understood. Decreased antegrade fetal blood flow through the aorta and abnormal extension of tissue
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from the ductus arteriosus onto the adjacent aortic wall with subsequent
postnatal contraction have been proposed as potential mechanisms (the
hemodynamic and ductal hypotheses, respectively).4, 5 Heredity also plays a role
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in CoA but are not well defined. The sibling of a patient with CoA has a 0.5% risk of having CoA and a 1% risk of any having any form of CHD.6 While most cases
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are sporadic, CoA is associated with other types of CHD (see below).
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3. Associations CoA is associated with a number of other conditions, including PHACES (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac
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defects, eye abnormalities, sternal cleft, and supraumbilical raphe) syndrome,7
Williams-Beuren syndrome,8 Alagille syndrome,9 and Noonan syndrome.10 It has been reported in 15% of patients with Turner syndrome.11 While CoA is a solitary
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lesion in 82% of cases,12 associations with a number of additional vascular and
congenital cardiac anomalies have been shown, particularly left-sided obstructive
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lesions of varying complexity and severity, including hypoplastic left heart syndrome, in which over 80% of patients have associated CoA.13 Bicuspid aortic valve is seen in 20-85% of affected patients (Fig. 1).14 CoA is also associated with cerebral artery aneurysms,15 and Various anomalies of the pulmonary and
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aortic arch vessels have also been reported, highlighting the need for careful
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evaluation of the major thoracic vasculature.
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4. Classification/Morphology CoA is classified as “simple” when isolated and “complex” when associated with additional cardiac anomalies. Classically, the narrowing is
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located at the aortic isthmus, between the left subclavian artery and the
ductus/ligamentum arteriosus (Fig. 2), but atypical CoA can occur anywhere from the transverse aortic arch to the abdominal aortic bifurcation.16 The narrowing
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varies in severity from a mild, discrete coarctation with a localized, posterior shelf-like lesion (Fig. 3) to complete atresia of the aortic lumen. Importantly,
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however, the walls of the aorta remain in continuity, differentiating CoA from interrupted aortic arch, in which there is true discontinuity between the proximal and distal segments. Interrupted aortic arch is almost always associated with other cardiac anomalies and usually necessitates earlier, more invasive
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treatment (i.e. two-stage neonatal surgery with arch reconstruction followed by cardiac anomaly repair);17 therefore, imagers should distinguish interrupted aortic arch and report associated cardiac anomalies. Older classification systems
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differentiated the CoA based on the relationship with the ductus arteriosus (preductal, juxtaductal, or postductal); this system is less commonly used
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currently, as the degree of narrowing, associated anomalies, and relationship with arch vessels is considered more clinically relevant.18 Associated hypoplasia of the transverse arch is present in 40-80% of patients, depending on the criterion applied, and varies in severity.19-22 Older studies used the ratio of arch segments to the ascending aorta to define hypoplasia (diameter of the proximal, distal, or isthmus less than 60%, 50%, or 40% diameter of the ascending aorta,
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respectively)23; however, z-scores are now available, with a score of -2 or lower indicating arch hypoplasia.24 Tubular hypoplasia, representing a combination of small arch diameter and increased length between segments of the arch (>5mm
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in infants), can also be seen.17
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5. Presentation The timing and manifestations of CoA depend on severity of narrowing, relationship with arch vessels, and adequacy of collateral vessel formation.
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Lower body hypoperfusion results in activation of the renin-angiotensin-
aldosterone system and consequent upper body hypertension. CoA that remains undetected until adulthood is either less severe or bypassed by generous
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collateral arteries and is often not accompanied by symptoms, other than upper
extremity hypertension. When present, symptoms and complications are usually
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secondary to hypertension, and include headaches, shortness of breath due to left ventricular dysfunction, exercise intolerance, and rarely ruptured cerebral artery aneurysms.15 Premature atherosclerotic disease can lead to myocardial infarctions and cerebrovascular accidents. Dissection (Fig. 4) and/or
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aneurysms/pseudoaneurysms (Fig. 3, Fig. 5) of the aorta and branch vessels can be seen in the initial presentation of CoA, including the spinal and intercostal arteries.25, 26 Lower body hypoperfusion manifests with diminished or delayed
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femoral and peripheral pulses, a pressure gradient between the upper and lower limbs, and (rarely) lower extremity claudication. Initial workup should include
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blood pressure measurements in all 4 extremities.
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6. Management The prognosis for unrepaired CoA is guarded, with reported mortality by age 50 as high as 90%.27 Fortunately, the management of CoA has evolved
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considerably over the past decades, with significantly improved late outcomes. The first surgical repairs of CoA were reported independently by Gross 28 and
Crafoord 29 in the 1940’s. Balloon angioplasty as an alternative to surgery was
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first described in the early 1980’s,30 and stenting has been available since
1989.31 Currently, a number of management strategies are available, and while
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there are general trends in treatment choice for distinct population groups, there is enough heterogeneity that the imager should be familiar with all of the major approaches. Furthermore, surgical repairs performed in neonatal/infant patients are increasingly being seen later in life as these patients survive into adulthood.
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Numerous studies have shown that age at CoA repair is a significant predictor of morbidity, with worse outcomes in patients who were older at the time of repair.32-34 Accordingly, optimal management would involve early repair.
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The main measures of success include abolition of the gradient across the coarctation, control of blood pressure, and absence of complications from
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treatment. The overall data suggests that when possible, resection with end-toend anastomosis (Fig. 1, Fig. 6) is the preferred technique in neonates and infants, with improved late outcomes and a decreased risk of reintervention compared to alternative techniques.35-37 Left subclavian artery flap anastomosis (Fig. 7), a technique that was popular in the 70’s and 80’s, is now less commonly seen due to the success of the resection with end-to-end anastomosis technique,
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but this method is still utilized in very small infants and is preferred in some centers, as it can be performed more rapidly than traditional resection with endto-end anastomosis and requires less dissection.38, 39 Complicated cases, in
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either pediatric or adult populations, may require patch angioplasty and rarely extra-anatomic bypasses (Fig. 8) as an alternative to the more standardized
to high rates of subsequent aneurysm formation.35
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surgical approaches. Patch angioplasty is currently rarely performed secondary
Balloon angioplasty has fallen out of favor for both primary and secondary
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CoA repair outside of infancy, as early and late outcomes are suboptimal relative to both surgery and stenting.35 However, percutaneous interventions with bare metal or covered stents are being increasingly utilized (Figs. 9, 10B), particularly in older children and adults with either primary or recurrent coarctation.40, 41
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Stents have a more limited role in younger children, because of the large introducer size required and the need for serial resizing as the child grows. Despite growing acceptance for percutaneous interventions in adults, there is still
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no official consensus on optimal treatment strategy. According to the 2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart
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Disease, the choice of percutaneous catheter intervention versus surgical repair of native discrete CoA should be determined by a multidisciplinary team, including cardiologists, interventionalists, and surgeons, at an adult CHD center (Level of Evidence: C). The guidelines for when to treat adults are better defined: treatment for primary CoA should be initiated when the peak-to-peak CoA gradient is ≥20mmHg (Level of Evidence: C) or when the gradient is less than
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20mmHg but there is anatomic imaging evidence of significant CoA with radiological evidence of significant collateral blood flow (Level of Evidence: C). In cases of recurrent CoA, the guidelines recommend percutaneous catheter
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intervention when there is discrete CoA and a peak-to-peak gradient of at least 20mmHg (Level of Evidence: B) and surgical treatment for previously repaired CoA and either a long recoarctation segment or concomitant aortic arch
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hypoplasia (Level of Evidence: B); however, there is also a class IIB
recommendation that stent placement for long-segment CoA may be considered
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with the caveat that the usefulness, long-term efficacy, and safety of this technique are unknown (Level of Evidence: C).42
Complications and late outcomes following CoA repair are partially dependent on treatment strategy. Recurrent laryngeal nerve paralysis, phrenic
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nerve injury, hemorrhage/re-exploration for bleeding, chylothorax, paraplegia, and paradoxical hypertension are possible early complications following open surgical repair.43-45 Extensive collateral vessels (Fig. 11, Fig. 4C), while
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decreasing the risk of ischemia, increase the risk of complications from hemorrhage and should therefore be described on preoperative imaging reports.
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Early complications following endovascular treatment are less frequent,35 and include technical complications such as stent migration or brachiocephalic vessel occlusion46; aortic wall complications such as dissection, aneurysm and pseudoaneurysm formation (Fig. 12), and rupture47, 48; peripheral vascular complications such as CVA, peripheral emboli, and access vessel injury49; and paradoxical hypertension. Of note, aortic injuries have been reported to be more
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frequent in older populations undergoing repair 35 and possibly in patients with concomitant bicuspid aortic valve.40 Late stent-related complications include fracture (Fig. 13) and in-stent restenosis. Late complications in all treatment
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strategies include stenosis/recoarctation (Fig. 14), aneurysm and
pseudoaneurysm formation (Fig. 15), and late hypertension.35 Late hypertension (and subsequent complications) is the major cause of late morbidity, and may be
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more common in patients who were repaired during childhood or adulthood (>25% experience late hypertension) than those who were repaired during
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infancy (<5%).34 Interestingly, while late hypertension is expected in patients with important residual aortic arch obstruction, it is also common in patients without an anatomic stenosis or significant gradient,33 necessitating lifetime hypertension
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screening.
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7. Imaging Imaging plays a prominent role in the diagnosis, pre-surgical planning, and follow-up of patients with CoA. According to the 2008 ACC/AHA Guidelines,
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every adult patient with CoA (repaired or not) should undergo at least one
cardiovascular MRI or CT in order to evaluate the thoracic aorta and intracranial vessels (Level of Evidence: B). Following repair, late postoperative imaging of
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the thoracic aorta should be performed to assess for aortic dilatation or aneurysm formation (Level of Evidence: B). And evaluation of the CoA repair site by MRI or
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CT should be performed at intervals of 5 years or less, depending on the specific anatomic findings before and after repair (Level of Evidence: C).42 The specific choice of imaging varies depending on patient- and situationspecific factors. In general, echocardiography is more useful in younger patients
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(particularly neonates and infants), as they have more favorable transthoracic acoustic windows.40 Echocardiography is less useful in adult patients and postoperative patients, with sensitivity for aneurysm detection as low as 24%
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compared to more advanced cross sectional imaging.50 MRI poses several advantages relative to CT, such as a lack of ionizing radiation and the ability to
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quantify collateral vessel flow and flow across the narrowing using phasecontrast techniques;51 however, CT may be more appropriate in some situations. CT has shown very high diagnostic accuracy (>95%) in the detection of CoA, associated anomalies, and post-operative complications in both children and adults.50, 52-56 CT has the highest spatial resolution amongst noninvasive modalities57; provides a large field of view allowing simultaneous visualization of
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the ascending aorta, aortic arch, descending aorta, and aortic valve (some MR sequences also have the ability to image the heart and thoracic aorta simultaneously); can be acquired more rapidly than other noninvasive modalities,
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without the need for extended breath holding or sedation; and allows
simultaneous evaluation of the coronary arteries. CT and MR 3D reconstructions and multiplanar reformations have been shown to add diagnostic value 58 and, in
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our experience, are highly valued by referring clinicians for instructional (both for providers and patients) and procedural planning purposes (Fig. 10). Imagers
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must consider CT in several situations in which MRI is impossible or limited: in patients with standard contraindications, such as pacemakers or other non-MRIsafe implanted devices; in patients with severe claustrophobia or inability to hold their breath; and in patients following stent implantation for CoA repair.59 While
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newer platinum stents suffer from susceptibility artifacts less than stainless steel stents, platinum stents are rarely utilized in the United States, and MRI can miss small aneurysms and stent fractures.60 Of note, there are limited reports of
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surgical clips following surgical repair of CoA causing diagnostic difficulties with MRI, namely overestimation of the degree of stenosis due to susceptibility
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artifact.61 Finally, advances in CT technology and protocols have resulted in a dramatic decrease in the associated burden of ionizing radiation (up to 80-90% dose reductions without compromising image quality or diagnostic accuracy62), with one low dose, prospectively triggered, high pitch dual-source CT protocol in children providing overall diagnostic accuracy of 96.25% in evaluating complex CoA while delivering a mean effective radiation dose of only 0.2±0.1 mSv.55
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While the diagnosis of CoA is usually straightforward, there are several discrete entities that can simulate the process, including pseudocoarctation and interrupted aortic arch. Pseudocoarctation is a rare congenital anomaly in which
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the aorta is angulated and potentially mildly narrowed at the level of the
ligamentum arteriosus (Fig. 16); however, there is no significant gradient across the lesion.63 Interrupted aortic arch, in which there is true discontinuity rather than
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narrowing of the thoracic aorta, is rarely seen as a primary finding in adults but has been reported.64
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Initial and pre-interventional CT reports in patients with known or suspected CoA should describe the morphology of the narrowing: whether it is discrete or elongated, and the diameter of the coarcted segment. Diameter of the ascending aorta, the proximal and distal transverse arch, the aortic isthmus just
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above the coarctation, and diameter of the thoracic aorta at the level of the diagram should all be reported.40 The relationship with and any anomalies of the aortic arch vessels should be described, and, when present, a “Gothic” or
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triangular shaped high cervical arch should be reported (Fig. 12, Fig. 16), as this finding has been associated with increased vascular thickening and increased
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late-onset hypertension compared to the “Romanesque” or rounded arch.65 The imager should evaluate for any associated cardiac or vascular anomalies, particularly left-sided obstructive lesions, including bicuspid aortic valve. Finally, collateral vessels should be described, as they can affect the surgical approach and will be monitored for change on follow-up imaging. The most commonly
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involved vessels include the internal mammary arteries, intercostal arteries, thyrocervical and thoracoacromial trunks.66 Follow-up imaging should focus on the potential complications described
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above, which will be in part dependent upon the type of repair.
Hemorrhage/aortic rupture will be most common in the immediate postoperative period. Recoarctation and aneurysm formation can occur at any time. Small
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pseudoaneurysms can be very difficult to see, especially when located adjacent to a stent. Note that in cases repaired via the left subclavian artery flap
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technique, the left subclavian artery will not be visible and perfusion to the left upper extremity will occur primarily via retrograde flow through the vertebral artery. Stents should be evaluated for fracture, migration, or in-stent stenosis/thrombosis. The size and extent of collateral vessels (Fig. 11) should be
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serially followed, as significant residual or worsening collateralization could indicate a significant residual gradient across the coarcted site. At least one study demonstrated a significant decrease in left and right ventricular mass and
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increased left ventricular function following stenting67; this information may be helpful for referring clinicians. One must keep in mind, however, that functional
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imaging will require at minimum dose modulated retrospective gating, which will increase radiation burden, and the benefits versus risks of higher dose protocols should be analyzed on a per-patient basis.
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8. Conclusion Both primary and repaired CoA are increasingly encountered in older children and adults, necessitating an understanding of potential associations and
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complications. There are several options for repair, which are subject to different complications and will manifest with varying imaging findings. The imager should be aware of the advantages and disadvantages of available imaging modalities
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complications of CoA before and after repair.
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and capable of reporting the relevant morphology, associations, and
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References:
1.
Backer CL, Mavroudis C. Congenital Heart Surgery Nomenclature and
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Database Project: patent ductus arteriosus, coarctation of the aorta,
interrupted aortic arch. The Annals of thoracic surgery. 2000;69:S298-307. 2.
Report of the New England Regional Infant Cardiac Program. Pediatrics.
3.
SC
1980;65:375-461.
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease:
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prevalence at livebirth. The Baltimore-Washington Infant Study. American journal of epidemiology. 1985;121:31-36. 4.
Hutchins GM. Coarctation of the aorta explained as a branch-point of the ductus arteriosus. Am J Pathol. 1971;63:203-214.
Krediet P. AN HYPOTHESIS OF THE DEVELOPMENT OF
TE D
5.
COARCTATION IN MAN. Acta morphologica Neerlando-Scandinavica. 1965;6:207-212.
Boon AR, Roberts DF. A family study of coarctation of the aorta. Journal
EP
6.
of medical genetics. 1976;13:420-433. Arora SS, Plato BM, Sattenberg RJ, Downs RK, Remmel KS, Heidenreich
AC C
7.
JO. Adult presentation of PHACES syndrome. Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences. 2011;17:137-146.
20
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8.
Kammache I, Acar P, Kreitmann B, Fraisse A. Williams-Beuren syndrome: an unusual cause of neonatal and infantile coarctation. The Journal of thoracic and cardiovascular surgery. 2010;140:e80-81. Agarwal V, Ramnarine I, Corno AF, Pozzi M. Recurrent coarctation in a
RI PT
9.
patient with Alagille syndrome. Interactive cardiovascular and thoracic surgery. 2005;4:514-516.
Marino B, Digilio MC, Toscano A, Giannotti A, Dallapiccola B. Congenital
SC
10.
heart diseases in children with Noonan syndrome: An expanded cardiac
M AN U
spectrum with high prevalence of atrioventricular canal. The Journal of pediatrics. 1999;135:703-706. 11.
Ho VB, Bakalov VK, Cooley M, et al. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features.
12.
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Circulation. 2004;110:1694-1700.
Aboulhosn J, Child JS. Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and
13.
EP
coarctation of the aorta. Circulation. 2006;114:2412-2422. Elzenga NJ, Gittenberger-de Groot AC. Coarctation and related aortic
AC C
arch anomalies in hypoplastic left heart syndrome. International journal of cardiology. 1985;8:379-393.
14.
Jenkins NP, Ward C. Coarctation of the aorta: natural history and outcome
after surgical treatment. QJM : monthly journal of the Association of Physicians. 1999;92:365-371.
21
ACCEPTED MANUSCRIPT
15.
Benyounes N, Blanc R, Boissonnet H, Piotin M. Subarachnoid hemorrhage revealing aortic coarctation in a young man. Neuroradiology. 2011;53:931-932. Sebastia C, Quiroga S, Boye R, Perez-Lafuente M, Castella E, Alvarez-
RI PT
16.
Castells A. Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 2003;23 Spec No:S79-91.
Matsui H, Adachi I, Uemura H, Gardiner H, Ho SY. Anatomy of
SC
17.
coarctation, hypoplastic and interrupted aortic arch: relevance to
2007;5:871-880. 18.
M AN U
interventional/surgical treatment. Expert review of cardiovascular therapy.
Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown). 2007;8:123-128.
Conte S, Lacour-Gayet F, Serraf A, et al. Surgical management of
TE D
19.
neonatal coarctation. The Journal of thoracic and cardiovascular surgery. 1995;109:663-674; discussion 674-665. Hager A, Schreiber C, Nutzl S, Hess J. Mortality and restenosis rate of
EP
20.
surgical coarctation repair in infancy: a study of 191 patients. Cardiology.
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2009;112:36-41.
21.
Lacour-Gayet F, Bruniaux J, Serraf A, et al. Hypoplastic transverse arch and coarctation in neonates. Surgical reconstruction of the aortic arch: a study of sixty-six patients. The Journal of thoracic and cardiovascular surgery. 1990;100:808-816.
22
ACCEPTED MANUSCRIPT
22.
Thomson JD, Mulpur A, Guerrero R, Nagy Z, Gibbs JL, Watterson KG. Outcome after extended arch repair for aortic coarctation. Heart. 2006;92:90-94. Kimura-Hayama ET, Melendez G, Mendizabal AL, Meave-Gonzalez A,
RI PT
23.
Zambrana GF, Corona-Villalobos CP. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics.
24.
SC
2010;30:79-98.
Elgamal MA, McKenzie ED, Fraser CD, Jr. Aortic arch advancement: the
M AN U
optimal one-stage approach for surgical management of neonatal coarctation with arch hypoplasia. The Annals of thoracic surgery. 2002;73:1267-1272; discussion 1272-1263. 25.
Aoun SG, El Ahmadieh TY, Soltanolkotabi M, et al. Ruptured spinal artery
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aneurysm associated with coarctation of the aorta. World neurosurgery. 2014;81:441 e417-422. 26.
Luo ZQ, Lai YQ, Zhu JM, Li JH, Liu KY, Zhou QW. Intercostal artery
EP
aneurysm associated with coarctation of aorta in an adult. Journal of cardiac surgery. 2010;25:719-720. Campbell M. Natural history of coarctation of the aorta. British heart
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27.
journal. 1970;32:633-640.
28.
Gross RE. Surgical correction for coarctation of the aorta. Surgery. 1945;18:673-678.
29.
Crafoord C, Ejrup B, Gladnikoff H. Coarctation of the aorta. Thorax. 1947;2:121-152.
23
ACCEPTED MANUSCRIPT
30.
Singer MI, Rowen M, Dorsey TJ. Transluminal aortic balloon angioplasty for coarctation of the aorta in the newborn. Am Heart J. 1982;103:131132. O'Laughlin MP, Perry SB, Lock JE, Mullins CE. Use of endovascular
RI PT
31.
stents in congenital heart disease. Circulation. 1991;83:1923-1939. 32.
Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the
correction. Circulation. 1989;80:840-845.
Hager A, Kanz S, Kaemmerer H, Schreiber C, Hess J. Coarctation Long-
M AN U
33.
SC
aorta. Long-term follow-up and prediction of outcome after surgical
term Assessment (COALA): significance of arterial hypertension in a cohort of 404 patients up to 27 years after surgical repair of isolated coarctation of the aorta, even in the absence of restenosis and prosthetic
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material. The Journal of thoracic and cardiovascular surgery. 2007;134:738-745. 34.
Seirafi PA, Warner KG, Geggel RL, Payne DD, Cleveland RJ. Repair of
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coarctation of the aorta during infancy minimizes the risk of late hypertension. The Annals of thoracic surgery. 1998;66:1378-1382. Forbes TJ, Kim DW, Du W, et al. Comparison of surgical, stent, and
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35.
balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). Journal of the American College of Cardiology. 2011;58:2664-2674.
24
ACCEPTED MANUSCRIPT
36.
Fruh S, Knirsch W, Dodge-Khatami A, Dave H, Pretre R, Kretschmar O. Comparison of surgical and interventional therapy of native and recurrent aortic coarctation regarding different age groups during childhood.
RI PT
European journal of cardio-thoracic surgery : official journal of the
European Association for Cardio-thoracic Surgery. 2011;39:898-904. 37.
McGuinness JG, Elhassan Y, Lee SY, et al. Do high-risk infants have a
SC
poorer outcome from primary repair of coarctation? Analysis of 192 infants over 20 years. The Annals of thoracic surgery. 2010;90:2023-2027. Zannini L, Gargiulo G, Albanese SB, et al. Aortic coarctation with
M AN U
38.
hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options. The Annals of thoracic surgery. 1993;56:288294.
Barreiro CJ, Ellison TA, Williams JA, Durr ML, Cameron DE, Vricella LA.
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39.
Subclavian flap aortoplasty: still a safe, reproducible, and effective treatment for infant coarctation. European journal of cardio-thoracic
EP
surgery : official journal of the European Association for Cardio-thoracic Surgery. 2007;31:649-653. Godart F. Intravascular stenting for the treatment of coarctation of the
AC C
40.
aorta in adolescent and adult patients. Archives of cardiovascular diseases. 2011;104:627-635.
41.
Harris KC, Du W, Cowley CG, Forbes TJ, Kim DW. A prospective
observational multicenter study of balloon angioplasty for the treatment of
25
ACCEPTED MANUSCRIPT
native and recurrent coarctation of the aorta. Catheter Cardiovasc Interv. 2014;83:1116-1123. 42.
Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines
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for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the
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Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart
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Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Journal of the American College of Cardiology. 2008;52:e143-263.
Ala-Kulju K, Heikkinen L. Aneurysms after patch graft aortoplasty for
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43.
coarctation of the aorta: long-term results of surgical management. The Annals of thoracic surgery. 1989;47:853-856. Knyshov GV, Sitar LL, Glagola MD, Atamanyuk MY. Aortic aneurysms at
EP
44.
the site of the repair of coarctation of the aorta: a review of 48 patients.
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The Annals of thoracic surgery. 1996;61:935-939.
45.
Sealy WC. Paradoxical hypertension after repair of coarctation of the aorta: a review of its causes. The Annals of thoracic surgery. 1990;50:323-
329.
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46.
van Gameren M, Witsenburg M, Takkenberg JJ, et al. Early complications of stenting in patients with congenital heart disease: a multicentre study. European heart journal. 2006;27:2709-2715. Balaji S, Oommen R, Rees PG. Fatal aortic rupture during balloon
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47.
dilatation of recoarctation. British heart journal. 1991;65:100-101. 48.
Fletcher SE, Cheatham JP, Froeming S. Aortic aneurysm following
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primary balloon angioplasty and secondary endovascular stent placement in the treatment of native coarctation of the aorta. Catheterization and
49.
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cardiovascular diagnosis. 1998;44:40-44.
Boshoff DE, Eyskens B, Gewillig M. Late redilation of a stent in the aorta crossing the subclavian artery complicated with a cerebellar infarction. Acta cardiologica. 2007;62:295-297.
Hoffman JL, Gray RG, LuAnn Minich L, et al. Screening for aortic
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50.
aneurysm after treatment of coarctation. Pediatr Cardiol. 2014;35:47-52. 51.
Padang R, Dennis M, Semsarian C, et al. Detection of serious
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complications by MR imaging in asymptomatic young adults with repaired coarctation of the aorta. Heart, lung & circulation. 2014;23:332-338. Chakrabarti S, Kenny D, Morgan G, et al. Balloon expandable stent
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52.
implantation for native and recurrent coarctation of the aorta--prospective computed tomography assessment of stent integrity, aneurysm formation and stenosis relief. Heart. 2010;96:1212-1216.
53.
Chen X, Qu YJ, Peng ZY, Lu JG, Ma XJ. Diagnosis of congenital aortic arch anomalies in chinese children by multi-detector computed
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tomography angiography. Journal of Huazhong University of Science and Technology. Medical sciences = Hua zhong ke ji da xue xue bao. Yi xue Ying De wen ban = Huazhong keji daxue xuebao. Yixue Yingdewen ban.
54.
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2013;33:447-451.
Nie P, Wang X, Cheng Z, et al. The value of low-dose prospective ECGgated dual-source CT angiography in the diagnosis of coarctation of the
55.
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aorta in infants and children. Clinical radiology. 2012;67:738-745.
Xu J, Zhao H, Wang X, et al. Accuracy, Image Quality, and Radiation
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Dose of Prospectively ECG-Triggered High-Pitch Dual-Source CT Angiography in Infants and Children with Complex Coarctation of the Aorta. Academic radiology. 2014. 56.
Turkvatan A, Akdur PO, Olcer T, Cumhur T. Coarctation of the aorta in
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adults: preoperative evaluation with multidetector CT angiography. Diagnostic and interventional radiology (Ankara, Turkey). 2009;15:269274.
Gopal A, Loewinger L, Budoff MJ. Aortic coarctation by cardiovascular CT
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57.
angiography. Catheter Cardiovasc Interv. 2010;76:551-552. Hu XH, Huang GY, Pa M, et al. Multidetector CT angiography and 3D
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58.
reconstruction in young children with coarctation of the aorta. Pediatr
Cardiol. 2008;29:726-731.
59.
Therrien J, Thorne SA, Wright A, Kilner PJ, Somerville J. Repaired coarctation: a "cost-effective" approach to identify complications in adults. Journal of the American College of Cardiology. 2000;35:997-1002.
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60.
Kenny DP, Hamilton M, Martin R. CT or MRI for post-procedural aortic stenting? Heart. 2011;97:164; author reply 165.
61.
Salam S, Myerson S, Wilson N. Artefactual limitations of magnetic
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resonance imaging in the diagnosis of recoarctation of the aorta. Heart. 2008;94:753. 62.
Gopal A, Mao SS, Karlsberg D, et al. Radiation reduction with prospective
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ECG-triggering acquisition using 64-multidetector Computed Tomographic
2009;25:405-416. 63.
Atalay MK, Kochilas LK. Familial pseudocoarctation of the aorta. Pediatr Cardiol. 2011;32:692-695.
64.
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angiography. The international journal of cardiovascular imaging.
Gordon EA, Person T, Kavarana M, Ikonomidis JS. Interrupted aortic arch
65.
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in the adult. Journal of cardiac surgery. 2011;26:405-409. Ou P, Celermajer DS, Mousseaux E, et al. Vascular remodeling after "successful" repair of coarctation: impact of aortic arch geometry. Journal
66.
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of the American College of Cardiology. 2007;49:883-890. Ho SY, Anderson RH. Coarctation, tubular hypoplasia, and the ductus
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arteriosus. Histological study of 35 specimens. British heart journal. 1979;41:268-274.
67.
Babu-Narayan SV, Mohiaddin RH, Cannell TM, Muhll IV, Dimopoulos K, Mullen MJ. Cardiovascular changes after transcatheter endovascular stenting of adult aortic coarctation. International journal of cardiology.
2011;149:157-163.
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Figure Legends
Figure 1. 26-year-old male with a history of CoA status post repair with resection
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and end-to-end anastomosis (arrowhead in A). The patient also has a bicuspid
aortic valve (arrow in B) and associated dilatation of the ascending aorta (arrow
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in A).
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Figure 2. Typical location of CoA at the aortic isthmus (arrow).
Figure 3. Discrete CoA in a 68-year-old male. Curved multiplanar reconstructions demonstrate a localized, shelflike lesion at the aortic isthmus (arrow in A). There is smooth ectasia of the descending aorta distal to the coarctation (arrow in B).
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Anatomic relationships are nicely demonstrated using 3D volume rendering (C), where we also see a tiny residual ductal diverticulum (arrow in C).
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Figure 4. 20-year-old male with CoA (arrow in A) initially presented emergently with acute aortic dissection. He underwent emergent surgery with aortic valve
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and ascending aortic replacements seven months prior to this study. A residual dissection is present in the transverse arch (arrow in B). Also note significant dilatation of the internal mammary arteries secondary to collateralization (C). He subsequently underwent CoA dilatation and covered stent placement.
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Figure 5. 48-year-old male with known CoA (arrowhead in A) was asymptomatic but hypertensive (systolic blood pressure >200mmHg) despite multiple medications. Patient had initially refused surgery. CT imaging shows a
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pseudoaneurysm at the coarctation site (arrows in B and C).
without (B) an interposition graft (arrow in A).
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Figure 6. Resection with end-to-end anastomosis of a discrete CoA with (A) and
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Figure 7. Left subclavian artery flap anastomosis. The left subclavian artery is ligated just proximal to the origin of the vertebral artery. An aortotomy is made below the coarctation and extends across the isthmus and left subclavian artery. The subclavian is divided just proximal to the ligature and the flap is moved
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caudad (curved arrow) for anastomosis over the aortotomy.
Figure 8. CoA treated with an extra-anatomic conduit from the ascending aorta to
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the descending thoracic aorta (arrow).
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Figure 9. Angioplasty and stent placement for CoA. A wire is passed across the stenosis (arrow), balloon angioplasty is performed (arrowhead), and bare metal or covered stent is placed across the coarctation (double arrows).
Figure 10. 55-year-old male with previously unknown CoA (arrow in A) with an associated left subclavian artery aneurysm (arrowhead in A and B). Patient
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underwent dilatation and stenting (arrow in B), which is nicely demonstrated on 3D volume-rendered image.
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Figure 11. Examples of collateral vessels involving the thyrocervical trunks and intercostal arteries.
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Figure 12. Small pseudoaneurysm (arrow) extending posteriorly from a stent in an 18 year-old male with prior patch angioplasty that was followed by stent
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placement for recoarctation. Also note the high (“Gothic”) arch, which has been associated with increased late-onset hypertension.
Figure 13. 16-year-old female with CoA initially underwent resection with end-to-
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end anastomosis as an infant; recoarctation necessitated angioplasty and stenting. Follow-up examination demonstrates a fracture through the stent (arrow in A), which was subsequently treated with an additional covered stent (arrows in
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B and C).
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Figure 14. 12-year-old male who presented with previously unknown CoA of the transverse arch, just distal to the origin of the left common carotid artery (arrows in A and B), associated with significant collateralization of the right internal mammary artery (C). The patient was treated with resection and interposition graft placement with reimplantation of the left subclavian artery; post-operative imagine revealed no early complications (D). Follow-up imaging one year after
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surgery demonstrated occlusion of the left subclavian artery (arrows in E and F) with retrograde filling via the vertebral artery.
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Figure 15. 46-year-old female with history of CoA repaired with resection and
interposition graft placement demonstrates several irregular pseudoaneurysms
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(arrows in A-E).
Figure 16. 36-year-old male initially presented to an outside emergency
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department with chest pain and left arm tingling; chest CT raised the possibility of CoA. Dedicated aortic imaging revealed a high (“Gothic”) aortic arch (arrow in A) with kinking (arrowhead in A) of the aortic isthmus but without significant narrowing (arrow in B). The patient had no hypertension or diminished lower
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extremity pulses on serial follow-up visits, and imaging findings were attributed to
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aortic pseudocoarctation.
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Coarctation of the aorta is increasingly seen in older patients.
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Imaging plays a crucial role in diagnosis, surgical planning, and follow-up of these patients. Imagers should be familiar with the various available surgical techniques.
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CT angiography is a fast, reliable method in the diagnosis and follow-up of
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these patients.
S1934-5925(15)30016-2
DOI:
10.1016/j.jcct.2015.11.002
Reference:
JCCT 845
To appear in:
Journal of Cardiovascular Computed Tomograph
Received Date: 12 March 2015 Revised Date:
12 July 2015
Accepted Date: 10 November 2015
Please cite this article as: Nance JW, Ringel RE, Fishman EK, Coarctation of the Aorta in Adolescents and Adults: A Review of Clinical Features and CT Imaging, Journal of Cardiovascular Computed Tomograph (2015), doi: 10.1016/j.jcct.2015.11.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Coarctation of the Aorta in Adolescents and Adults: A Review of Clinical Features and CT Imaging
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Short title: Aortic coarctation in adolescents and adults John W. Nance, MD1; Richard E. Ringel, MD2; Elliot K. Fishman, MD1
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1 – Russell H. Morgan Department of Radiology and Radiological Science Johns Hopkins School of Medicine 601 N. Caroline St Baltimore, MD
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2 – Department of Pediatrics Johns Hopkins School of Medicine 601 N. Caroline St Baltimore, MD
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Corresponding author: Elliot K. Fishman, MD Professor of Radiology, Oncology, and Surgery Department of Radiology and Radiological Sciences Johns Hopkins University School of Medicine 601 N. Caroline St, JHOC 3254 Baltimore, MD 21287 Phone: 410-955-5173 Fax: 410-614-0341 Email: [email protected]
Conflicts of interest related to this article: None. Conflicts of interest not related to this article: Dr. Fishman receives grant support from Siemens and GE. The other authors have no potential conflicts of interest to disclose.
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Coarctation of the Aorta in Adolescents and Adults: A Review of
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Clinical Features and CT Imaging
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Abstract Coarctation of the aorta (CoA), while usually identified and treated in the neonatal/infant period, is increasingly seen in adults, either primarily or (more
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often) following repair. Imaging plays a crucial role in the diagnosis, therapeutic planning, and follow-up of patients with CoA. Clinical management of CoA in
adults optimally involves a multidisciplinary team; accordingly, imagers should be
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familiar with the underlying pathology, associations, and management of CoA in addition to imaging protocoling and interpretation. We will review the relevant
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clinical and imaging features of CoA, with an emphasis on patients beyond
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childhood.
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Key Words: Coarctation of the aorta; congenital heart disease; adult congenital heart disease;
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computed tomography; cardiac computed tomography.
List of abbreviations: CHD – Congenital heart disease
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CoA – Coarctation of the aorta
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1. Introduction First described by Morgagni in 1760, coarctation of the aorta (CoA; from the Latin term coarctare, “to contract”)1 occurrs in 0.4% of live births and is
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recognized in 7% of patients with congenital heart disease (CHD)2. There is a male predominance, with the male to female ratio reported as 1.5:1.3 While
severe cases usually present in the neonatal period, the diagnosis of CoA is
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often not made until adolescence or adulthood. Furthermore, early repair of CoA is subject to recoarctation, necessitating familiarity of the disease and its imaging
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manifestations for both pediatric and adult imagers. The current review will summarize the relevant clinical and CT features of CoA, with an emphasis on
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patients beyond childhood.
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2. Pathology The underlying pathogenesis of CoA is not well understood. Decreased antegrade fetal blood flow through the aorta and abnormal extension of tissue
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from the ductus arteriosus onto the adjacent aortic wall with subsequent
postnatal contraction have been proposed as potential mechanisms (the
hemodynamic and ductal hypotheses, respectively).4, 5 Heredity also plays a role
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in CoA but are not well defined. The sibling of a patient with CoA has a 0.5% risk of having CoA and a 1% risk of any having any form of CHD.6 While most cases
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are sporadic, CoA is associated with other types of CHD (see below).
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3. Associations CoA is associated with a number of other conditions, including PHACES (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac
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defects, eye abnormalities, sternal cleft, and supraumbilical raphe) syndrome,7
Williams-Beuren syndrome,8 Alagille syndrome,9 and Noonan syndrome.10 It has been reported in 15% of patients with Turner syndrome.11 While CoA is a solitary
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lesion in 82% of cases,12 associations with a number of additional vascular and
congenital cardiac anomalies have been shown, particularly left-sided obstructive
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lesions of varying complexity and severity, including hypoplastic left heart syndrome, in which over 80% of patients have associated CoA.13 Bicuspid aortic valve is seen in 20-85% of affected patients (Fig. 1).14 CoA is also associated with cerebral artery aneurysms,15 and Various anomalies of the pulmonary and
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aortic arch vessels have also been reported, highlighting the need for careful
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evaluation of the major thoracic vasculature.
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4. Classification/Morphology CoA is classified as “simple” when isolated and “complex” when associated with additional cardiac anomalies. Classically, the narrowing is
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located at the aortic isthmus, between the left subclavian artery and the
ductus/ligamentum arteriosus (Fig. 2), but atypical CoA can occur anywhere from the transverse aortic arch to the abdominal aortic bifurcation.16 The narrowing
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varies in severity from a mild, discrete coarctation with a localized, posterior shelf-like lesion (Fig. 3) to complete atresia of the aortic lumen. Importantly,
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however, the walls of the aorta remain in continuity, differentiating CoA from interrupted aortic arch, in which there is true discontinuity between the proximal and distal segments. Interrupted aortic arch is almost always associated with other cardiac anomalies and usually necessitates earlier, more invasive
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treatment (i.e. two-stage neonatal surgery with arch reconstruction followed by cardiac anomaly repair);17 therefore, imagers should distinguish interrupted aortic arch and report associated cardiac anomalies. Older classification systems
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differentiated the CoA based on the relationship with the ductus arteriosus (preductal, juxtaductal, or postductal); this system is less commonly used
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currently, as the degree of narrowing, associated anomalies, and relationship with arch vessels is considered more clinically relevant.18 Associated hypoplasia of the transverse arch is present in 40-80% of patients, depending on the criterion applied, and varies in severity.19-22 Older studies used the ratio of arch segments to the ascending aorta to define hypoplasia (diameter of the proximal, distal, or isthmus less than 60%, 50%, or 40% diameter of the ascending aorta,
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respectively)23; however, z-scores are now available, with a score of -2 or lower indicating arch hypoplasia.24 Tubular hypoplasia, representing a combination of small arch diameter and increased length between segments of the arch (>5mm
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in infants), can also be seen.17
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5. Presentation The timing and manifestations of CoA depend on severity of narrowing, relationship with arch vessels, and adequacy of collateral vessel formation.
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Lower body hypoperfusion results in activation of the renin-angiotensin-
aldosterone system and consequent upper body hypertension. CoA that remains undetected until adulthood is either less severe or bypassed by generous
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collateral arteries and is often not accompanied by symptoms, other than upper
extremity hypertension. When present, symptoms and complications are usually
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secondary to hypertension, and include headaches, shortness of breath due to left ventricular dysfunction, exercise intolerance, and rarely ruptured cerebral artery aneurysms.15 Premature atherosclerotic disease can lead to myocardial infarctions and cerebrovascular accidents. Dissection (Fig. 4) and/or
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aneurysms/pseudoaneurysms (Fig. 3, Fig. 5) of the aorta and branch vessels can be seen in the initial presentation of CoA, including the spinal and intercostal arteries.25, 26 Lower body hypoperfusion manifests with diminished or delayed
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femoral and peripheral pulses, a pressure gradient between the upper and lower limbs, and (rarely) lower extremity claudication. Initial workup should include
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blood pressure measurements in all 4 extremities.
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6. Management The prognosis for unrepaired CoA is guarded, with reported mortality by age 50 as high as 90%.27 Fortunately, the management of CoA has evolved
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considerably over the past decades, with significantly improved late outcomes. The first surgical repairs of CoA were reported independently by Gross 28 and
Crafoord 29 in the 1940’s. Balloon angioplasty as an alternative to surgery was
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first described in the early 1980’s,30 and stenting has been available since
1989.31 Currently, a number of management strategies are available, and while
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there are general trends in treatment choice for distinct population groups, there is enough heterogeneity that the imager should be familiar with all of the major approaches. Furthermore, surgical repairs performed in neonatal/infant patients are increasingly being seen later in life as these patients survive into adulthood.
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Numerous studies have shown that age at CoA repair is a significant predictor of morbidity, with worse outcomes in patients who were older at the time of repair.32-34 Accordingly, optimal management would involve early repair.
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The main measures of success include abolition of the gradient across the coarctation, control of blood pressure, and absence of complications from
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treatment. The overall data suggests that when possible, resection with end-toend anastomosis (Fig. 1, Fig. 6) is the preferred technique in neonates and infants, with improved late outcomes and a decreased risk of reintervention compared to alternative techniques.35-37 Left subclavian artery flap anastomosis (Fig. 7), a technique that was popular in the 70’s and 80’s, is now less commonly seen due to the success of the resection with end-to-end anastomosis technique,
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but this method is still utilized in very small infants and is preferred in some centers, as it can be performed more rapidly than traditional resection with endto-end anastomosis and requires less dissection.38, 39 Complicated cases, in
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either pediatric or adult populations, may require patch angioplasty and rarely extra-anatomic bypasses (Fig. 8) as an alternative to the more standardized
to high rates of subsequent aneurysm formation.35
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surgical approaches. Patch angioplasty is currently rarely performed secondary
Balloon angioplasty has fallen out of favor for both primary and secondary
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CoA repair outside of infancy, as early and late outcomes are suboptimal relative to both surgery and stenting.35 However, percutaneous interventions with bare metal or covered stents are being increasingly utilized (Figs. 9, 10B), particularly in older children and adults with either primary or recurrent coarctation.40, 41
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Stents have a more limited role in younger children, because of the large introducer size required and the need for serial resizing as the child grows. Despite growing acceptance for percutaneous interventions in adults, there is still
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no official consensus on optimal treatment strategy. According to the 2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart
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Disease, the choice of percutaneous catheter intervention versus surgical repair of native discrete CoA should be determined by a multidisciplinary team, including cardiologists, interventionalists, and surgeons, at an adult CHD center (Level of Evidence: C). The guidelines for when to treat adults are better defined: treatment for primary CoA should be initiated when the peak-to-peak CoA gradient is ≥20mmHg (Level of Evidence: C) or when the gradient is less than
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20mmHg but there is anatomic imaging evidence of significant CoA with radiological evidence of significant collateral blood flow (Level of Evidence: C). In cases of recurrent CoA, the guidelines recommend percutaneous catheter
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intervention when there is discrete CoA and a peak-to-peak gradient of at least 20mmHg (Level of Evidence: B) and surgical treatment for previously repaired CoA and either a long recoarctation segment or concomitant aortic arch
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hypoplasia (Level of Evidence: B); however, there is also a class IIB
recommendation that stent placement for long-segment CoA may be considered
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with the caveat that the usefulness, long-term efficacy, and safety of this technique are unknown (Level of Evidence: C).42
Complications and late outcomes following CoA repair are partially dependent on treatment strategy. Recurrent laryngeal nerve paralysis, phrenic
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nerve injury, hemorrhage/re-exploration for bleeding, chylothorax, paraplegia, and paradoxical hypertension are possible early complications following open surgical repair.43-45 Extensive collateral vessels (Fig. 11, Fig. 4C), while
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decreasing the risk of ischemia, increase the risk of complications from hemorrhage and should therefore be described on preoperative imaging reports.
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Early complications following endovascular treatment are less frequent,35 and include technical complications such as stent migration or brachiocephalic vessel occlusion46; aortic wall complications such as dissection, aneurysm and pseudoaneurysm formation (Fig. 12), and rupture47, 48; peripheral vascular complications such as CVA, peripheral emboli, and access vessel injury49; and paradoxical hypertension. Of note, aortic injuries have been reported to be more
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frequent in older populations undergoing repair 35 and possibly in patients with concomitant bicuspid aortic valve.40 Late stent-related complications include fracture (Fig. 13) and in-stent restenosis. Late complications in all treatment
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strategies include stenosis/recoarctation (Fig. 14), aneurysm and
pseudoaneurysm formation (Fig. 15), and late hypertension.35 Late hypertension (and subsequent complications) is the major cause of late morbidity, and may be
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more common in patients who were repaired during childhood or adulthood (>25% experience late hypertension) than those who were repaired during
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infancy (<5%).34 Interestingly, while late hypertension is expected in patients with important residual aortic arch obstruction, it is also common in patients without an anatomic stenosis or significant gradient,33 necessitating lifetime hypertension
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screening.
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7. Imaging Imaging plays a prominent role in the diagnosis, pre-surgical planning, and follow-up of patients with CoA. According to the 2008 ACC/AHA Guidelines,
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every adult patient with CoA (repaired or not) should undergo at least one
cardiovascular MRI or CT in order to evaluate the thoracic aorta and intracranial vessels (Level of Evidence: B). Following repair, late postoperative imaging of
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the thoracic aorta should be performed to assess for aortic dilatation or aneurysm formation (Level of Evidence: B). And evaluation of the CoA repair site by MRI or
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CT should be performed at intervals of 5 years or less, depending on the specific anatomic findings before and after repair (Level of Evidence: C).42 The specific choice of imaging varies depending on patient- and situationspecific factors. In general, echocardiography is more useful in younger patients
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(particularly neonates and infants), as they have more favorable transthoracic acoustic windows.40 Echocardiography is less useful in adult patients and postoperative patients, with sensitivity for aneurysm detection as low as 24%
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compared to more advanced cross sectional imaging.50 MRI poses several advantages relative to CT, such as a lack of ionizing radiation and the ability to
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quantify collateral vessel flow and flow across the narrowing using phasecontrast techniques;51 however, CT may be more appropriate in some situations. CT has shown very high diagnostic accuracy (>95%) in the detection of CoA, associated anomalies, and post-operative complications in both children and adults.50, 52-56 CT has the highest spatial resolution amongst noninvasive modalities57; provides a large field of view allowing simultaneous visualization of
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the ascending aorta, aortic arch, descending aorta, and aortic valve (some MR sequences also have the ability to image the heart and thoracic aorta simultaneously); can be acquired more rapidly than other noninvasive modalities,
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without the need for extended breath holding or sedation; and allows
simultaneous evaluation of the coronary arteries. CT and MR 3D reconstructions and multiplanar reformations have been shown to add diagnostic value 58 and, in
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our experience, are highly valued by referring clinicians for instructional (both for providers and patients) and procedural planning purposes (Fig. 10). Imagers
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must consider CT in several situations in which MRI is impossible or limited: in patients with standard contraindications, such as pacemakers or other non-MRIsafe implanted devices; in patients with severe claustrophobia or inability to hold their breath; and in patients following stent implantation for CoA repair.59 While
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newer platinum stents suffer from susceptibility artifacts less than stainless steel stents, platinum stents are rarely utilized in the United States, and MRI can miss small aneurysms and stent fractures.60 Of note, there are limited reports of
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surgical clips following surgical repair of CoA causing diagnostic difficulties with MRI, namely overestimation of the degree of stenosis due to susceptibility
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artifact.61 Finally, advances in CT technology and protocols have resulted in a dramatic decrease in the associated burden of ionizing radiation (up to 80-90% dose reductions without compromising image quality or diagnostic accuracy62), with one low dose, prospectively triggered, high pitch dual-source CT protocol in children providing overall diagnostic accuracy of 96.25% in evaluating complex CoA while delivering a mean effective radiation dose of only 0.2±0.1 mSv.55
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While the diagnosis of CoA is usually straightforward, there are several discrete entities that can simulate the process, including pseudocoarctation and interrupted aortic arch. Pseudocoarctation is a rare congenital anomaly in which
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the aorta is angulated and potentially mildly narrowed at the level of the
ligamentum arteriosus (Fig. 16); however, there is no significant gradient across the lesion.63 Interrupted aortic arch, in which there is true discontinuity rather than
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narrowing of the thoracic aorta, is rarely seen as a primary finding in adults but has been reported.64
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Initial and pre-interventional CT reports in patients with known or suspected CoA should describe the morphology of the narrowing: whether it is discrete or elongated, and the diameter of the coarcted segment. Diameter of the ascending aorta, the proximal and distal transverse arch, the aortic isthmus just
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above the coarctation, and diameter of the thoracic aorta at the level of the diagram should all be reported.40 The relationship with and any anomalies of the aortic arch vessels should be described, and, when present, a “Gothic” or
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triangular shaped high cervical arch should be reported (Fig. 12, Fig. 16), as this finding has been associated with increased vascular thickening and increased
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late-onset hypertension compared to the “Romanesque” or rounded arch.65 The imager should evaluate for any associated cardiac or vascular anomalies, particularly left-sided obstructive lesions, including bicuspid aortic valve. Finally, collateral vessels should be described, as they can affect the surgical approach and will be monitored for change on follow-up imaging. The most commonly
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involved vessels include the internal mammary arteries, intercostal arteries, thyrocervical and thoracoacromial trunks.66 Follow-up imaging should focus on the potential complications described
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above, which will be in part dependent upon the type of repair.
Hemorrhage/aortic rupture will be most common in the immediate postoperative period. Recoarctation and aneurysm formation can occur at any time. Small
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pseudoaneurysms can be very difficult to see, especially when located adjacent to a stent. Note that in cases repaired via the left subclavian artery flap
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technique, the left subclavian artery will not be visible and perfusion to the left upper extremity will occur primarily via retrograde flow through the vertebral artery. Stents should be evaluated for fracture, migration, or in-stent stenosis/thrombosis. The size and extent of collateral vessels (Fig. 11) should be
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serially followed, as significant residual or worsening collateralization could indicate a significant residual gradient across the coarcted site. At least one study demonstrated a significant decrease in left and right ventricular mass and
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increased left ventricular function following stenting67; this information may be helpful for referring clinicians. One must keep in mind, however, that functional
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imaging will require at minimum dose modulated retrospective gating, which will increase radiation burden, and the benefits versus risks of higher dose protocols should be analyzed on a per-patient basis.
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8. Conclusion Both primary and repaired CoA are increasingly encountered in older children and adults, necessitating an understanding of potential associations and
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complications. There are several options for repair, which are subject to different complications and will manifest with varying imaging findings. The imager should be aware of the advantages and disadvantages of available imaging modalities
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complications of CoA before and after repair.
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and capable of reporting the relevant morphology, associations, and
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References:
1.
Backer CL, Mavroudis C. Congenital Heart Surgery Nomenclature and
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Database Project: patent ductus arteriosus, coarctation of the aorta,
interrupted aortic arch. The Annals of thoracic surgery. 2000;69:S298-307. 2.
Report of the New England Regional Infant Cardiac Program. Pediatrics.
3.
SC
1980;65:375-461.
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease:
M AN U
prevalence at livebirth. The Baltimore-Washington Infant Study. American journal of epidemiology. 1985;121:31-36. 4.
Hutchins GM. Coarctation of the aorta explained as a branch-point of the ductus arteriosus. Am J Pathol. 1971;63:203-214.
Krediet P. AN HYPOTHESIS OF THE DEVELOPMENT OF
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5.
COARCTATION IN MAN. Acta morphologica Neerlando-Scandinavica. 1965;6:207-212.
Boon AR, Roberts DF. A family study of coarctation of the aorta. Journal
EP
6.
of medical genetics. 1976;13:420-433. Arora SS, Plato BM, Sattenberg RJ, Downs RK, Remmel KS, Heidenreich
AC C
7.
JO. Adult presentation of PHACES syndrome. Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences. 2011;17:137-146.
20
ACCEPTED MANUSCRIPT
8.
Kammache I, Acar P, Kreitmann B, Fraisse A. Williams-Beuren syndrome: an unusual cause of neonatal and infantile coarctation. The Journal of thoracic and cardiovascular surgery. 2010;140:e80-81. Agarwal V, Ramnarine I, Corno AF, Pozzi M. Recurrent coarctation in a
RI PT
9.
patient with Alagille syndrome. Interactive cardiovascular and thoracic surgery. 2005;4:514-516.
Marino B, Digilio MC, Toscano A, Giannotti A, Dallapiccola B. Congenital
SC
10.
heart diseases in children with Noonan syndrome: An expanded cardiac
M AN U
spectrum with high prevalence of atrioventricular canal. The Journal of pediatrics. 1999;135:703-706. 11.
Ho VB, Bakalov VK, Cooley M, et al. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features.
12.
TE D
Circulation. 2004;110:1694-1700.
Aboulhosn J, Child JS. Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and
13.
EP
coarctation of the aorta. Circulation. 2006;114:2412-2422. Elzenga NJ, Gittenberger-de Groot AC. Coarctation and related aortic
AC C
arch anomalies in hypoplastic left heart syndrome. International journal of cardiology. 1985;8:379-393.
14.
Jenkins NP, Ward C. Coarctation of the aorta: natural history and outcome
after surgical treatment. QJM : monthly journal of the Association of Physicians. 1999;92:365-371.
21
ACCEPTED MANUSCRIPT
15.
Benyounes N, Blanc R, Boissonnet H, Piotin M. Subarachnoid hemorrhage revealing aortic coarctation in a young man. Neuroradiology. 2011;53:931-932. Sebastia C, Quiroga S, Boye R, Perez-Lafuente M, Castella E, Alvarez-
RI PT
16.
Castells A. Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 2003;23 Spec No:S79-91.
Matsui H, Adachi I, Uemura H, Gardiner H, Ho SY. Anatomy of
SC
17.
coarctation, hypoplastic and interrupted aortic arch: relevance to
2007;5:871-880. 18.
M AN U
interventional/surgical treatment. Expert review of cardiovascular therapy.
Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown). 2007;8:123-128.
Conte S, Lacour-Gayet F, Serraf A, et al. Surgical management of
TE D
19.
neonatal coarctation. The Journal of thoracic and cardiovascular surgery. 1995;109:663-674; discussion 674-665. Hager A, Schreiber C, Nutzl S, Hess J. Mortality and restenosis rate of
EP
20.
surgical coarctation repair in infancy: a study of 191 patients. Cardiology.
AC C
2009;112:36-41.
21.
Lacour-Gayet F, Bruniaux J, Serraf A, et al. Hypoplastic transverse arch and coarctation in neonates. Surgical reconstruction of the aortic arch: a study of sixty-six patients. The Journal of thoracic and cardiovascular surgery. 1990;100:808-816.
22
ACCEPTED MANUSCRIPT
22.
Thomson JD, Mulpur A, Guerrero R, Nagy Z, Gibbs JL, Watterson KG. Outcome after extended arch repair for aortic coarctation. Heart. 2006;92:90-94. Kimura-Hayama ET, Melendez G, Mendizabal AL, Meave-Gonzalez A,
RI PT
23.
Zambrana GF, Corona-Villalobos CP. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics.
24.
SC
2010;30:79-98.
Elgamal MA, McKenzie ED, Fraser CD, Jr. Aortic arch advancement: the
M AN U
optimal one-stage approach for surgical management of neonatal coarctation with arch hypoplasia. The Annals of thoracic surgery. 2002;73:1267-1272; discussion 1272-1263. 25.
Aoun SG, El Ahmadieh TY, Soltanolkotabi M, et al. Ruptured spinal artery
TE D
aneurysm associated with coarctation of the aorta. World neurosurgery. 2014;81:441 e417-422. 26.
Luo ZQ, Lai YQ, Zhu JM, Li JH, Liu KY, Zhou QW. Intercostal artery
EP
aneurysm associated with coarctation of aorta in an adult. Journal of cardiac surgery. 2010;25:719-720. Campbell M. Natural history of coarctation of the aorta. British heart
AC C
27.
journal. 1970;32:633-640.
28.
Gross RE. Surgical correction for coarctation of the aorta. Surgery. 1945;18:673-678.
29.
Crafoord C, Ejrup B, Gladnikoff H. Coarctation of the aorta. Thorax. 1947;2:121-152.
23
ACCEPTED MANUSCRIPT
30.
Singer MI, Rowen M, Dorsey TJ. Transluminal aortic balloon angioplasty for coarctation of the aorta in the newborn. Am Heart J. 1982;103:131132. O'Laughlin MP, Perry SB, Lock JE, Mullins CE. Use of endovascular
RI PT
31.
stents in congenital heart disease. Circulation. 1991;83:1923-1939. 32.
Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the
correction. Circulation. 1989;80:840-845.
Hager A, Kanz S, Kaemmerer H, Schreiber C, Hess J. Coarctation Long-
M AN U
33.
SC
aorta. Long-term follow-up and prediction of outcome after surgical
term Assessment (COALA): significance of arterial hypertension in a cohort of 404 patients up to 27 years after surgical repair of isolated coarctation of the aorta, even in the absence of restenosis and prosthetic
TE D
material. The Journal of thoracic and cardiovascular surgery. 2007;134:738-745. 34.
Seirafi PA, Warner KG, Geggel RL, Payne DD, Cleveland RJ. Repair of
EP
coarctation of the aorta during infancy minimizes the risk of late hypertension. The Annals of thoracic surgery. 1998;66:1378-1382. Forbes TJ, Kim DW, Du W, et al. Comparison of surgical, stent, and
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35.
balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). Journal of the American College of Cardiology. 2011;58:2664-2674.
24
ACCEPTED MANUSCRIPT
36.
Fruh S, Knirsch W, Dodge-Khatami A, Dave H, Pretre R, Kretschmar O. Comparison of surgical and interventional therapy of native and recurrent aortic coarctation regarding different age groups during childhood.
RI PT
European journal of cardio-thoracic surgery : official journal of the
European Association for Cardio-thoracic Surgery. 2011;39:898-904. 37.
McGuinness JG, Elhassan Y, Lee SY, et al. Do high-risk infants have a
SC
poorer outcome from primary repair of coarctation? Analysis of 192 infants over 20 years. The Annals of thoracic surgery. 2010;90:2023-2027. Zannini L, Gargiulo G, Albanese SB, et al. Aortic coarctation with
M AN U
38.
hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options. The Annals of thoracic surgery. 1993;56:288294.
Barreiro CJ, Ellison TA, Williams JA, Durr ML, Cameron DE, Vricella LA.
TE D
39.
Subclavian flap aortoplasty: still a safe, reproducible, and effective treatment for infant coarctation. European journal of cardio-thoracic
EP
surgery : official journal of the European Association for Cardio-thoracic Surgery. 2007;31:649-653. Godart F. Intravascular stenting for the treatment of coarctation of the
AC C
40.
aorta in adolescent and adult patients. Archives of cardiovascular diseases. 2011;104:627-635.
41.
Harris KC, Du W, Cowley CG, Forbes TJ, Kim DW. A prospective
observational multicenter study of balloon angioplasty for the treatment of
25
ACCEPTED MANUSCRIPT
native and recurrent coarctation of the aorta. Catheter Cardiovasc Interv. 2014;83:1116-1123. 42.
Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines
RI PT
for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the
SC
Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart
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Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Journal of the American College of Cardiology. 2008;52:e143-263.
Ala-Kulju K, Heikkinen L. Aneurysms after patch graft aortoplasty for
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43.
coarctation of the aorta: long-term results of surgical management. The Annals of thoracic surgery. 1989;47:853-856. Knyshov GV, Sitar LL, Glagola MD, Atamanyuk MY. Aortic aneurysms at
EP
44.
the site of the repair of coarctation of the aorta: a review of 48 patients.
AC C
The Annals of thoracic surgery. 1996;61:935-939.
45.
Sealy WC. Paradoxical hypertension after repair of coarctation of the aorta: a review of its causes. The Annals of thoracic surgery. 1990;50:323-
329.
26
ACCEPTED MANUSCRIPT
46.
van Gameren M, Witsenburg M, Takkenberg JJ, et al. Early complications of stenting in patients with congenital heart disease: a multicentre study. European heart journal. 2006;27:2709-2715. Balaji S, Oommen R, Rees PG. Fatal aortic rupture during balloon
RI PT
47.
dilatation of recoarctation. British heart journal. 1991;65:100-101. 48.
Fletcher SE, Cheatham JP, Froeming S. Aortic aneurysm following
SC
primary balloon angioplasty and secondary endovascular stent placement in the treatment of native coarctation of the aorta. Catheterization and
49.
M AN U
cardiovascular diagnosis. 1998;44:40-44.
Boshoff DE, Eyskens B, Gewillig M. Late redilation of a stent in the aorta crossing the subclavian artery complicated with a cerebellar infarction. Acta cardiologica. 2007;62:295-297.
Hoffman JL, Gray RG, LuAnn Minich L, et al. Screening for aortic
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50.
aneurysm after treatment of coarctation. Pediatr Cardiol. 2014;35:47-52. 51.
Padang R, Dennis M, Semsarian C, et al. Detection of serious
EP
complications by MR imaging in asymptomatic young adults with repaired coarctation of the aorta. Heart, lung & circulation. 2014;23:332-338. Chakrabarti S, Kenny D, Morgan G, et al. Balloon expandable stent
AC C
52.
implantation for native and recurrent coarctation of the aorta--prospective computed tomography assessment of stent integrity, aneurysm formation and stenosis relief. Heart. 2010;96:1212-1216.
53.
Chen X, Qu YJ, Peng ZY, Lu JG, Ma XJ. Diagnosis of congenital aortic arch anomalies in chinese children by multi-detector computed
27
ACCEPTED MANUSCRIPT
tomography angiography. Journal of Huazhong University of Science and Technology. Medical sciences = Hua zhong ke ji da xue xue bao. Yi xue Ying De wen ban = Huazhong keji daxue xuebao. Yixue Yingdewen ban.
54.
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2013;33:447-451.
Nie P, Wang X, Cheng Z, et al. The value of low-dose prospective ECGgated dual-source CT angiography in the diagnosis of coarctation of the
55.
SC
aorta in infants and children. Clinical radiology. 2012;67:738-745.
Xu J, Zhao H, Wang X, et al. Accuracy, Image Quality, and Radiation
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Dose of Prospectively ECG-Triggered High-Pitch Dual-Source CT Angiography in Infants and Children with Complex Coarctation of the Aorta. Academic radiology. 2014. 56.
Turkvatan A, Akdur PO, Olcer T, Cumhur T. Coarctation of the aorta in
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adults: preoperative evaluation with multidetector CT angiography. Diagnostic and interventional radiology (Ankara, Turkey). 2009;15:269274.
Gopal A, Loewinger L, Budoff MJ. Aortic coarctation by cardiovascular CT
EP
57.
angiography. Catheter Cardiovasc Interv. 2010;76:551-552. Hu XH, Huang GY, Pa M, et al. Multidetector CT angiography and 3D
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58.
reconstruction in young children with coarctation of the aorta. Pediatr
Cardiol. 2008;29:726-731.
59.
Therrien J, Thorne SA, Wright A, Kilner PJ, Somerville J. Repaired coarctation: a "cost-effective" approach to identify complications in adults. Journal of the American College of Cardiology. 2000;35:997-1002.
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ACCEPTED MANUSCRIPT
60.
Kenny DP, Hamilton M, Martin R. CT or MRI for post-procedural aortic stenting? Heart. 2011;97:164; author reply 165.
61.
Salam S, Myerson S, Wilson N. Artefactual limitations of magnetic
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resonance imaging in the diagnosis of recoarctation of the aorta. Heart. 2008;94:753. 62.
Gopal A, Mao SS, Karlsberg D, et al. Radiation reduction with prospective
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ECG-triggering acquisition using 64-multidetector Computed Tomographic
2009;25:405-416. 63.
Atalay MK, Kochilas LK. Familial pseudocoarctation of the aorta. Pediatr Cardiol. 2011;32:692-695.
64.
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angiography. The international journal of cardiovascular imaging.
Gordon EA, Person T, Kavarana M, Ikonomidis JS. Interrupted aortic arch
65.
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in the adult. Journal of cardiac surgery. 2011;26:405-409. Ou P, Celermajer DS, Mousseaux E, et al. Vascular remodeling after "successful" repair of coarctation: impact of aortic arch geometry. Journal
66.
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of the American College of Cardiology. 2007;49:883-890. Ho SY, Anderson RH. Coarctation, tubular hypoplasia, and the ductus
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arteriosus. Histological study of 35 specimens. British heart journal. 1979;41:268-274.
67.
Babu-Narayan SV, Mohiaddin RH, Cannell TM, Muhll IV, Dimopoulos K, Mullen MJ. Cardiovascular changes after transcatheter endovascular stenting of adult aortic coarctation. International journal of cardiology.
2011;149:157-163.
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Figure Legends
Figure 1. 26-year-old male with a history of CoA status post repair with resection
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and end-to-end anastomosis (arrowhead in A). The patient also has a bicuspid
aortic valve (arrow in B) and associated dilatation of the ascending aorta (arrow
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in A).
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Figure 2. Typical location of CoA at the aortic isthmus (arrow).
Figure 3. Discrete CoA in a 68-year-old male. Curved multiplanar reconstructions demonstrate a localized, shelflike lesion at the aortic isthmus (arrow in A). There is smooth ectasia of the descending aorta distal to the coarctation (arrow in B).
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Anatomic relationships are nicely demonstrated using 3D volume rendering (C), where we also see a tiny residual ductal diverticulum (arrow in C).
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Figure 4. 20-year-old male with CoA (arrow in A) initially presented emergently with acute aortic dissection. He underwent emergent surgery with aortic valve
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and ascending aortic replacements seven months prior to this study. A residual dissection is present in the transverse arch (arrow in B). Also note significant dilatation of the internal mammary arteries secondary to collateralization (C). He subsequently underwent CoA dilatation and covered stent placement.
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Figure 5. 48-year-old male with known CoA (arrowhead in A) was asymptomatic but hypertensive (systolic blood pressure >200mmHg) despite multiple medications. Patient had initially refused surgery. CT imaging shows a
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pseudoaneurysm at the coarctation site (arrows in B and C).
without (B) an interposition graft (arrow in A).
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Figure 6. Resection with end-to-end anastomosis of a discrete CoA with (A) and
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Figure 7. Left subclavian artery flap anastomosis. The left subclavian artery is ligated just proximal to the origin of the vertebral artery. An aortotomy is made below the coarctation and extends across the isthmus and left subclavian artery. The subclavian is divided just proximal to the ligature and the flap is moved
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caudad (curved arrow) for anastomosis over the aortotomy.
Figure 8. CoA treated with an extra-anatomic conduit from the ascending aorta to
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the descending thoracic aorta (arrow).
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Figure 9. Angioplasty and stent placement for CoA. A wire is passed across the stenosis (arrow), balloon angioplasty is performed (arrowhead), and bare metal or covered stent is placed across the coarctation (double arrows).
Figure 10. 55-year-old male with previously unknown CoA (arrow in A) with an associated left subclavian artery aneurysm (arrowhead in A and B). Patient
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underwent dilatation and stenting (arrow in B), which is nicely demonstrated on 3D volume-rendered image.
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Figure 11. Examples of collateral vessels involving the thyrocervical trunks and intercostal arteries.
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Figure 12. Small pseudoaneurysm (arrow) extending posteriorly from a stent in an 18 year-old male with prior patch angioplasty that was followed by stent
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placement for recoarctation. Also note the high (“Gothic”) arch, which has been associated with increased late-onset hypertension.
Figure 13. 16-year-old female with CoA initially underwent resection with end-to-
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end anastomosis as an infant; recoarctation necessitated angioplasty and stenting. Follow-up examination demonstrates a fracture through the stent (arrow in A), which was subsequently treated with an additional covered stent (arrows in
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B and C).
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Figure 14. 12-year-old male who presented with previously unknown CoA of the transverse arch, just distal to the origin of the left common carotid artery (arrows in A and B), associated with significant collateralization of the right internal mammary artery (C). The patient was treated with resection and interposition graft placement with reimplantation of the left subclavian artery; post-operative imagine revealed no early complications (D). Follow-up imaging one year after
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surgery demonstrated occlusion of the left subclavian artery (arrows in E and F) with retrograde filling via the vertebral artery.
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Figure 15. 46-year-old female with history of CoA repaired with resection and
interposition graft placement demonstrates several irregular pseudoaneurysms
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(arrows in A-E).
Figure 16. 36-year-old male initially presented to an outside emergency
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department with chest pain and left arm tingling; chest CT raised the possibility of CoA. Dedicated aortic imaging revealed a high (“Gothic”) aortic arch (arrow in A) with kinking (arrowhead in A) of the aortic isthmus but without significant narrowing (arrow in B). The patient had no hypertension or diminished lower
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extremity pulses on serial follow-up visits, and imaging findings were attributed to
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aortic pseudocoarctation.
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Coarctation of the aorta is increasingly seen in older patients.
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Imaging plays a crucial role in diagnosis, surgical planning, and follow-up of these patients. Imagers should be familiar with the various available surgical techniques.
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CT angiography is a fast, reliable method in the diagnosis and follow-up of
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these patients.