Interrupted aortic arch

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Interrupted aortic arch (IAA) is an uncommon congenital cardiovascular anomaly where there is a separation between the ascending and descending aorta. It can either be complete or connected by a remnant fibrous band. An accompanying large ventricular septal defect (VSD) and/or patent ductus arteriosus (PDA) is frequently present.

Epidemiology

It may account for ~1.5% of congenital cardiac anomalies.

Pathology

Faulty embryological development of the aortic arch (thought to occur during the 5^th to to 7^th ~~week~~ week of intrauterine life).

Classification

According to the Celoria-Patton classification, IAA can be classified into three types according to the location of the anomaly:

type A: second most common, the interruption occurs distal to the left subclavian arterial origin
type B: ~~most~~ most common (>50%), the break occurs between the left common carotid and left subclavian arterial origins
type C: rare, interruption occurs proximal to the left common carotid arterial origin

Each type is divided into three subtypes ⁷:

subtype 1: normal subclavian artery
subtype 2: aberrant subclavian artery
subtype 3: isolated subclavian artery that arises from the ductus arteriosus

Associations

DiGeorge syndrome¹
- found commonly in those with a type B interruption
- almost always associated if there is a right-sided descending aorta
truncus arteriosus ⁸
aortopulmonary septal defect (aortopulmonary window)
transposition of the great arteries
double outlet right ventricle
functional single ventricle

Radiographic features

Plain radiograph

Plain film features are often non-specific³:

the aortic knuckle may be absent
may show cardiomegaly

Antenatal ultrasound/Echocardiography

The right ventricle may appear a lot larger than the left, although this is a non-specific finding. The ascending aorta may also appear more vertical than usual. These modalities may not allow differentiation of IAA from severe aortic coarctation with a hypoplastic arch ⁹.

CT

Allows visualisation of the interrupted aortic arch and associated anomalies.

MRI

non-visualisation of the portion of interruption
great vessels may show a "V" configuration on coronal imaging ²

Treatment and prognosis

If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days. Prostaglandin E₁may be given to initial management to keep the ductus open. Surgical correction (either single- or multistage) is the definitive treatment.

Differential diagnosis

General differential considerations include:

short segment severe aortic coarctation

-<p><strong>Interrupted aortic arch (IAA)</strong> is an uncommon <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> where there is a separation between the ascending and descending aorta. It can either be complete or connected by a remnant fibrous band. An accompanying large <a href="/articles/ventricular-septal-defect-1">ventricular septal defect (VSD)</a> and/or <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus (PDA)</a> is frequently present.</p><h4>Epidemiology</h4><p>It may account for ~1.5% of congenital cardiac anomalies. </p><h4>Pathology</h4><p>Faulty embryological development of the aortic arch (thought to occur during the 5<sup>th</sup> to 7<sup>th</sup> week of intrauterine life).</p><h5>Classification</h5><p>According to the Celoria-Patton classification, IAA can be classified into three types according to the location of the anomaly:</p><ul>
~~-<li>~~
~~-<strong>type A:</strong> second most common, the interruption occurs distal to the left subclavian arterial origin </li>~~
~~-<li>~~
~~-<strong>type B:</strong> most common (>50%), the break occurs between the left common carotid and left subclavian arterial origins</li>~~
~~-<li>~~
~~-<strong>type C:</strong> rare, interruption occurs proximal to the left common carotid arterial origin</li>~~
+<p><strong>Interrupted aortic arch (IAA)</strong> is an uncommon <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> where there is a separation between the ascending and descending aorta. It can either be complete or connected by a remnant fibrous band. An accompanying large <a href="/articles/ventricular-septal-defect-1">ventricular septal defect (VSD)</a> and/or <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus (PDA)</a> is frequently present.</p><h4>Epidemiology</h4><p>It may account for ~1.5% of congenital cardiac anomalies. </p><h4>Pathology</h4><p>Faulty embryological development of the aortic arch (thought to occur during the 5<sup>th</sup> to 7<sup>th</sup> week of intrauterine life).</p><h5>Classification</h5><p>According to the Celoria-Patton classification, IAA can be classified into three types according to the location of the anomaly:</p><ul>
+<li><p><strong>type A</strong>: second most common, the interruption occurs distal to the left subclavian arterial origin </p></li>
+<li><p><strong>type B</strong>: most common (>50%), the break occurs between the left common carotid and left subclavian arterial origins</p></li>
+<li><p><strong>type C</strong>: rare, interruption occurs proximal to the left common carotid arterial origin</p></li>
~~-<li>~~
~~-<strong>subtype 1:</strong> normal subclavian artery</li>~~
~~-<li>~~
~~-<strong>subtype 2:</strong> aberrant subclavian artery</li>~~
~~-<li>~~
~~-<strong>subtype 3:</strong> isolated subclavian artery that arises from the ductus arteriosus</li>~~
+<li><p><strong>subtype 1</strong>: normal subclavian artery</p></li>
+<li><p><strong>subtype 2</strong>: aberrant subclavian artery</p></li>
+<li><p><strong>subtype 3</strong>: isolated subclavian artery that arises from the ductus arteriosus</p></li>
~~-<a href="/articles/22q112-deletion-syndrome-1">DiGeorge syndrome</a> <sup>1</sup><ul>~~
~~-<li>found commonly in those with a type B interruption</li>~~
~~-<li>almost always associated if there is a right-sided descending aorta</li>~~
+<p><a href="/articles/22q112-deletion-syndrome-1">DiGeorge syndrome</a> <sup>1</sup></p>
+<ul>
+<li><p>found commonly in those with a type B interruption</p></li>
+<li><p>almost always associated if there is a right-sided descending aorta</p></li>
~~-<li>~~
~~-<a href="/articles/truncus-arteriosus">truncus arteriosus</a> <sup>8</sup>~~
~~-</li>~~
~~-<li><a href="/articles/aortopulmonary-septal-defect-1">aortopulmonary septal defect (aortopulmonary window)</a></li>~~
~~-<li><a href="/articles/transposition-of-the-great-arteries">transposition of the great arteries</a></li>~~
~~-<li><a href="/articles/double-outlet-right-ventricle">double outlet right ventricle</a></li>~~
~~-<li>~~
~~-<a href="/articles/single-ventricle">functional single ventricle</a> </li>~~
+<li><p><a href="/articles/truncus-arteriosus">truncus arteriosus</a> <sup>8</sup></p></li>
+<li><p><a href="/articles/aortopulmonary-septal-defect-1">aortopulmonary septal defect (aortopulmonary window)</a></p></li>
+<li><p><a href="/articles/transposition-of-the-great-arteries">transposition of the great arteries</a></p></li>
+<li><p><a href="/articles/double-outlet-right-ventricle">double outlet right ventricle</a></p></li>
+<li><p><a href="/articles/single-ventricle">functional single ventricle</a> </p></li>
~~-<li>the <a href="/articles/aortic-knob">aortic knuckle</a> may be absent</li>~~
~~-<li>may show cardiomegaly</li>~~
-</ul><h5>Antenatal ultrasound/Echocardiography</h5><p>The right ventricle may appear a lot larger than the left, although this is a non-specific finding. The ascending aorta may also appear more vertical than usual. These modalities may not allow differentiation of IAA from severe aortic coarctation with a hypoplastic arch <sup>9</sup>.</p><h5>CT</h5><p>Allows visualisation of the interrupted aortic arch and associated anomalies. </p><h5>MRI</h5><ul>
~~-<li>non-visualisation of the portion of interruption</li>~~
~~-<li>great vessels may show a "V" configuration on coronal imaging <sup>2</sup>~~
~~-</li>~~
-</ul><h4>Treatment and prognosis</h4><p>If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days. Prostaglandin E<sub>1 </sub>may be given to initial management to keep the ductus open. Surgical correction (either single- or multistage) is the definitive treatment.</p><h4>Differential diagnosis</h4><p>General differential considerations include:</p><ul><li>short segment severe <a href="/articles/coarctation-of-the-aorta">aortic coarctation</a>
~~-</li></ul>~~
+<li><p>the <a href="/articles/aortic-knob">aortic knuckle</a> may be absent</p></li>
+<li><p>may show cardiomegaly</p></li>
+</ul><h5>Antenatal ultrasound/Echocardiography</h5><p>The right ventricle may appear a lot larger than the left, although this is a non-specific finding. The ascending aorta may also appear more vertical than usual. These modalities may not allow differentiation of IAA from severe aortic coarctation with a hypoplastic arch <sup>9</sup>.</p><h5>CT</h5><p>Allows visualisation of the interrupted aortic arch and associated anomalies. </p><h5>MRI</h5><ul>
+<li><p>non-visualisation of the portion of interruption</p></li>
+<li><p>great vessels may show a "V" configuration on coronal imaging <sup>2</sup></p></li>
+</ul><h4>Treatment and prognosis</h4><p>If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days. Prostaglandin E<sub>1 </sub>may be given to initial management to keep the ductus open. Surgical correction (either single- or multistage) is the definitive treatment.</p><h4>Differential diagnosis</h4><p>General differential considerations include:</p><ul><li><p>short segment severe <a href="/articles/coarctation-of-the-aorta">aortic coarctation</a></p></li></ul>

References changed:

1. Yu T, Zhu X, Tang L, Wang D, Saad N. Review of CT Angiography of Aorta. Radiol Clin North Am. 2007;45(3):461-83, viii. <a href="https://doi.org/10.1016/j.rcl.2007.04.010">doi:10.1016/j.rcl.2007.04.010</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/17601503">Pubmed</a>
2. Dillman J, Yarram S, D'Amico A, Hernandez R. Interrupted Aortic Arch: Spectrum of MRI Findings. AJR Am J Roentgenol. 2008;190(6):1467-74. <a href="https://doi.org/10.2214/AJR.07.3408">doi:10.2214/AJR.07.3408</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18492893">Pubmed</a>
3. Neye-Bock S & Fellows K. Aortic Arch Interruption in Infancy: Radio- and Angiographic Features. AJR Am J Roentgenol. 1980;135(5):1005-10. <a href="https://doi.org/10.2214/ajr.135.5.1005">doi:10.2214/ajr.135.5.1005</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/6778140">Pubmed</a>
4. Roche K, Krinsky G, Lee V, Rofsky N, Genieser N. Interrupted Aortic Arch: Diagnosis with Gadolinium-Enhanced 3D MRA. J Comput Assist Tomogr. 1999;23(2):197-202. <a href="https://doi.org/10.1097/00004728-199903000-00006">doi:10.1097/00004728-199903000-00006</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10096325">Pubmed</a>
5. Hernandez R, Aisen A, Foo T, Beekman R. Thoracic Cardiovascular Anomalies in Children: Evaluation with a Fast Gradient-Recalled-Echo Sequence with Cardiac-Triggered Segmented Acquisition. Radiology. 1993;188(3):775-80. <a href="https://doi.org/10.1148/radiology.188.3.8351346">doi:10.1148/radiology.188.3.8351346</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/8351346">Pubmed</a>
6. Frank L, Dillman J, Parish V et al. Cardiovascular MR Imaging of Conotruncal Anomalies. Radiographics. 2010;30(4):1069-94. <a href="https://doi.org/10.1148/rg.304095158">doi:10.1148/rg.304095158</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20631369">Pubmed</a>
7. Goo H, Park I, Ko J et al. CT of Congenital Heart Disease: Normal Anatomy and Typical Pathologic Conditions. Radiographics. 2003;23 Spec No(suppl_1):S147-65. <a href="https://doi.org/10.1148/rg.23si035501">doi:10.1148/rg.23si035501</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/14557509">Pubmed</a>
8. Bohuta L, Hussein A, Fricke T et al. Surgical Repair of Truncus Arteriosus Associated with Interrupted Aortic Arch: Long-Term Outcomes. Ann Thorac Surg. 2011;91(5):1473-7. <a href="https://doi.org/10.1016/j.athoracsur.2010.12.046">doi:10.1016/j.athoracsur.2010.12.046</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/21524457">Pubmed</a>
9. Kimura-Hayama E, Meléndez G, Mendizábal A, Meave-González A, Zambrana G, Corona-Villalobos C. Uncommon Congenital and Acquired Aortic Diseases: Role of Multidetector CT Angiography. Radiographics. 2010;30(1):79-98. <a href="https://doi.org/10.1148/rg.301095061">doi:10.1148/rg.301095061</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20083587">Pubmed</a>
1. Yu T, Zhu X, Tang L et-al. Review of CT angiography of aorta. Radiol. Clin. North Am. 2007;45 (3): 461-83, viii. <a href="http://dx.doi.org/10.1016/j.rcl.2007.04.010">doi:10.1016/j.rcl.2007.04.010</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17601503">Pubmed citation</a><div class="ref_v2"></div>
2. Dillman JR, Yarram SG, D'Amico AR et-al. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 2008;190 (6): 1467-74. <a href="http://dx.doi.org/10.2214/AJR.07.3408">doi:10.2214/AJR.07.3408</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18492893">Pubmed citation</a><div class="ref_v2"></div>
3. Neye-bock S, Fellows KE. Aortic arch interruption in infancy: radio- and angiographic features. AJR Am J Roentgenol. 1980;135 (5): 1005-10. <a href="http://www.ajronline.org/cgi/content/abstract/135/5/1005">AJR Am J Roentgenol (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/6778140">Pubmed citation</a><div class="ref_v2"></div>
4. Roche KJ, Krinsky G, Lee VS et-al. Interrupted aortic arch: diagnosis with gadolinium-enhanced 3D MRA. J Comput Assist Tomogr. 23 (2): 197-202. <a href="http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0363-8715&volume=23&issue=2&spage=197">J Comput Assist Tomogr (link)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/10096325">Pubmed citation</a><div class="ref_v2"></div>
5. Hernandez RJ, Aisen AM, Foo TK et-al. Thoracic cardiovascular anomalies in children: evaluation with a fast gradient-recalled-echo sequence with cardiac-triggered segmented acquisition. Radiology. 1993;188 (3): 775-80. <a href="http://radiology.rsna.org/content/188/3/775.abstract">Radiology (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/8351346">Pubmed citation</a><div class="ref_v2"></div>
6. Frank L, Dillman JR, Parish V et-al. Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30 (4): 1069-94. <a href="http://dx.doi.org/10.1148/rg.304095158">doi:10.1148/rg.304095158</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/20631369">Pubmed citation</a><div class="ref_v2"></div>
7. Goo HW, Park IS, Ko JK et-al. CT of congenital heart disease: normal anatomy and typical pathologic conditions. Radiographics. 2003;23 Spec No : S147-65. <a href="http://dx.doi.org/10.1148/rg.23si035501">doi:10.1148/rg.23si035501</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/14557509">Pubmed citation</a><div class="ref_v2"></div>
8. Bohuta L, Hussein A, Fricke TA et-al. Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes. Ann. Thorac. Surg. 2011;91 (5): 1473-7. <a href="http://dx.doi.org/10.1016/j.athoracsur.2010.12.046">doi:10.1016/j.athoracsur.2010.12.046</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21524457">Pubmed citation</a><span class="auto"></span>
9. Kimura-Hayama ET, MeléNdez G, MendizáBal AL et-al. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 2010;30 (1): 79-98. <a href="http://radiographics.rsna.org/content/30/1/79.full">Radiographics (full text)</a> - <a href="http://dx.doi.org/10.1148/rg.301095061">doi:10.1148/rg.301095061</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/20083587">Pubmed citation</a><span class="ref_v3"></span>

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