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<article article-type="case-report" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xml:lang="EN">
<front>
<journal-meta>
<journal-id journal-id-type="publisher-id">Front. Surg.</journal-id>
<journal-title>Frontiers in Surgery</journal-title><abbrev-journal-title abbrev-type="pubmed">Front. Surg.</abbrev-journal-title>
<issn pub-type="epub">2296-875X</issn>
<publisher>
<publisher-name>Frontiers Media S.A.</publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="doi">10.3389/fsurg.2022.1063224</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Surgery</subject>
<subj-group>
<subject>Case Report</subject>
</subj-group>
</subj-group>
</article-categories>
<title-group>
<article-title>Subclavian steal syndrome associated with right aortic arch: A case report</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author"><name><surname>Cai</surname><given-names>Jing</given-names></name>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib>
<contrib contrib-type="author"><name><surname>Xie</surname><given-names>Chao</given-names></name>
<xref ref-type="aff" rid="aff2"><sup>2</sup></xref><uri xlink:href="https://loop.frontiersin.org/people/2086311/overview"/></contrib>
<contrib contrib-type="author" corresp="yes"><name><surname>Wang</surname><given-names>Xianwei</given-names></name>
<xref ref-type="aff" rid="aff3"><sup>3</sup></xref>
<xref ref-type="corresp" rid="cor1">&#x002A;</xref><uri xlink:href="https://loop.frontiersin.org/people/2041512/overview" /></contrib>
</contrib-group>
<aff id="aff1"><label><sup>1</sup></label><addr-line>Department of General Practice</addr-line>, <institution>Xiangya Hospital, Central South University</institution>, <addr-line>Changsha</addr-line>, <country>China</country></aff>
<aff id="aff2"><label><sup>2</sup></label><addr-line>Department of Cardiovascular Surgery</addr-line>, <institution>The Second Xiangya Hospital, Central South University</institution>, <addr-line>Changsha</addr-line>, <country>China</country></aff>
<aff id="aff3"><label><sup>3</sup></label><addr-line>Department of General Surgery</addr-line>, <institution>Xiangya Hospital, Central South University</institution>, <addr-line>Changsha</addr-line>, <country>China</country></aff>
<author-notes>
<fn fn-type="edited-by"><p><bold>Edited by:</bold> Jieqing Wan, Shanghai Jiao Tong University, China</p></fn>
<fn fn-type="edited-by"><p><bold>Reviewed by:</bold> Anshuman Darbari, All India Institute of Medical Sciences, India Mizar D&#x0027;Abramo, Umberto 1 Hospital, Italy</p></fn>
<corresp id="cor1"><label>&#x002A;</label><bold>Correspondence:</bold> Xianwei Wang Email <email>xwwangxyyy@163.com</email></corresp>
<fn fn-type="other" id="fn001"><p><bold>Specialty Section:</bold> This article was submitted to Vascular Surgery, a section of the journal Frontiers in Surgery</p></fn>
</author-notes>
<pub-date pub-type="epub"><day>06</day><month>01</month><year>2023</year></pub-date>
<pub-date pub-type="collection"><year>2022</year></pub-date>
<volume>9</volume><elocation-id>1063224</elocation-id>
<history>
<date date-type="received"><day>06</day><month>10</month><year>2022</year></date>
<date date-type="accepted"><day>21</day><month>11</month><year>2022</year></date>
</history>
<permissions>
<copyright-statement>&#x00A9; 2023 Cai, Xie and Wang,</copyright-statement>
<copyright-year>2023</copyright-year><copyright-holder>Cai, Xie and Wang</copyright-holder><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/4.0/">
<p>This is an open-access article distributed under the terms of the <ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">Creative Commons Attribution License (CC BY)</ext-link>. The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.</p></license>
</permissions>
<abstract>
<p>The right aortic arch (RAA) is a rare congenital vascular variant disease. We reported a case of subclavian steal syndrome associated with RAA. The primary clinical symptoms were vertigo and ischemic symptoms of the left upper extremity. We diagnosed the condition using aortic computed tomography angiography and digital subtraction angiography. The patient underwent carotid-subclavian bypass surgery.</p>
</abstract>
<kwd-group>
<kwd>right aortic arch</kwd>
<kwd>subclavian steal syndrome</kwd>
<kwd>bypass surgery treatment</kwd>
<kwd>case</kwd>
<kwd>report</kwd>
</kwd-group>
<counts>
<fig-count count="3"/>
<table-count count="0"/><equation-count count="0"/><ref-count count="15"/><page-count count="0"/><word-count count="0"/></counts>
</article-meta>
</front>
<body>
<sec id="s1"><title>Case presentation</title>
<p>A 60-year-old female patient was admitted to the hospital after she reported experiencing frequent nausea and dizziness for more than 1 year. Vertigo did not reveal a significant relationship with the body position and spontaneously resolved after a while. For a year, the appeal symptoms had recurred. She presented with a 3-year history of hypertension and coronary heart disease. The patient&#x0027;s mother and three sisters had a history of identical undiagnosed vertigo. Upon admission, physical examination revealed that the blood pressures of the left upper limb and the right upper limb were 113/67&#x2005;mmHg and 157/85&#x2005;mmHg, respectively, and the systolic pressure difference between the two upper limbs was 44&#x2005;mmHg. Although the right brachial and radial arteries were normal, the pulse rates of the left brachial and radial arteries were low. The left hand and forearm had slightly lower skin temperature than the right hand and forearm. The sensory muscle strength of both forearms was normal. The right aortic arch and aberrant great branch of the aortic arch were detected by thoracic aortic computed-tomography angiography (CTA) (<xref ref-type="fig" rid="F1">Figure&#x00A0;1A</xref>). The left subclavian artery was not visible. as revealed by the aortic arc digital subtraction angiography (DSA) (<xref ref-type="fig" rid="F1">Figure&#x00A0;1B</xref>). The left vertebral artery (LVA) supplied the left subclavian artery (LSA) (<xref ref-type="fig" rid="F1">Figure&#x00A0;1C</xref>).</p>
<fig id="F1" position="float"><label>Figure 1</label>
<caption><p>Preoperative CTA of the aorta showed a right-sided aortic arch with an abnormal great branch (<bold>A</bold>). Aortic DSA showed that the left subclavian artery was not visible (<bold>B</bold>). Left subclavian artery supplied by the left vertebral artery (<bold>C</bold>). LSA, left subclavian artery; LCCA, left common carotid artery.</p></caption>
<graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="fsurg-09-1063224-g001.tif"/>
</fig>
</sec>
<sec id="s2"><title>Diagnosis and treatment</title>
<p>The patient underwent a left common carotid artery-subclavian bypass surgery (<xref ref-type="fig" rid="F2">Figure&#x00A0;2</xref>). The patient&#x0027;s vertigo improved following surgery. The throb of the left brachial artery and radial artery was intensified. The skin temperature of the left hand and forearm was elevated; however, there was no significant difference observed in the blood pressure of the two upper limbs. A postoperative review of cervical vascular CTA (<xref ref-type="fig" rid="F3">Figure&#x00A0;3</xref>) revealed effective bypass vessel angiography. Our patient had no symptoms associated with subclavian steal syndrome at 3-month and 6-month follow-up.</p>
<fig id="F2" position="float"><label>Figure 2</label>
<caption><p>Intraoperative dissociation of the left subclavian artery and its branches, proximal narrowing of the lumen of the left subclavian artery is seen (<bold>A</bold>). Common carotid artery&#x2014;left subclavian graft (<bold>B</bold>). PLSA, proximal left subclavian artery; DLSA, distal left subclavian artery; VA, vertebral artery; ITA, internal thoracic artery; TCT, thyroid trunk; BVP, artificial vessel; LCCA, left common carotid artery; LIJV, left internal jugular vein.</p></caption>
<graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="fsurg-09-1063224-g002.tif"/>
</fig>
<fig id="F3" position="float"><label>Figure 3</label>
<caption><p>Postoperative CTA showed that good angiography of bypass vessels (<bold>A,B</bold>). LSA, left subclavian artery; LCCA, left common carotid artery.</p></caption>
<graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="fsurg-09-1063224-g003.tif"/>
</fig>
</sec>
<sec id="s3"><title>Discuss</title>
<p>With a global prevalence of 0.1&#x0025;, RAA is a rare vascular malformation that can either be a mirror image of normal branches or a combination of various abnormalities (<xref ref-type="bibr" rid="B1">1</xref>, <xref ref-type="bibr" rid="B2">2</xref>). Cases of subclavian steal syndrome associated with RAA are quite rare. One report showed symptoms of left arm ischemia and vertebrobasilar insufficiency years after left subclavian artery ligation (<xref ref-type="bibr" rid="B3">3</xref>). There are reports of a man with Peutz-Jeghers syndrome who had his first episode of vertigo at the age of 29 due to subclavian steal syndrome (<xref ref-type="bibr" rid="B4">4</xref>).</p>
<p>The ductus arteriosus is usually on the left side. However, the ductus arteriosus may also be present on the right side, possibly with bilateral arterial development associated with aortic arch malformations (<xref ref-type="bibr" rid="B3">3</xref>). The patient included in our study belonged to the third category. The separation of the left subclavian artery from the right aortic arch may be related to 22q11 deletion in embryological development (<xref ref-type="bibr" rid="B5">5</xref>, <xref ref-type="bibr" rid="B6">6</xref>).</p>
<p>Contorni was the first to define subclavian steal syndrome, a condition in which blood flow is reversed in branches of the subclavian artery (<xref ref-type="bibr" rid="B7">7</xref>). The criteria for the diagnosis of subclavian steal syndrome are as follows: (1) Occlusion or marked stenosis of the subclavian or innominate arteries; (2) retrograde vertebral arterial flow; (3) patency of both the vertebral and basilar arteries (<xref ref-type="bibr" rid="B8">8</xref>). Our case met these criteria. Owing to the inadequate blood supply to the brain arteries, some patients with subclavian steal syndrome may experience symptoms of temporary ischemia (<xref ref-type="bibr" rid="B9">9</xref>). The symptoms of this disease include vertigo, dizziness, headache, diplopia, and a wide spectrum of symptoms involving the brain stem (<xref ref-type="bibr" rid="B1">1</xref>). It is caused by posterior circulation (brainstem vestibular system) ischemia, and vertigo generally lasts for several minutes and subsides on its own and thus can be distinguished from pseudovertigo, which is caused by hypertension (<xref ref-type="bibr" rid="B10">10</xref>). To accurately diagnose the subclavian syndrome, it is imperative to ascertain the blood pressure (BP) differences between retrograde blood flow in the arm and vertebral arteries (<xref ref-type="bibr" rid="B11">11</xref>, <xref ref-type="bibr" rid="B12">12</xref>). More than 95&#x0025; of patients have been documented in the literature to have overtly visible clinical symptoms, including dizziness, vomiting, headache, and syncope. The external carotid artery, the thyrocervical artery, the external carotid artery-occipital artery, the muscular branches of the vertebral artery systems, and a crossover flow between the internal mammary arteries are all collateral pathways for blood to enter the subclavian artery; however, the main anastomotic network is the vertebrobasilar system (<xref ref-type="bibr" rid="B10">10</xref>).</p>
<p>The primary imaging tests used to diagnose this disease were transthoracic echocardiography (TTE), magnetic resonance imaging (MRI), and angiography. Eighty-four percent of the cases were suspected based on the initial TTE; however, in many cases, echocardiography often indicated a suspicion albeit did not confirm the diagnosis. To determine the details of vascular connections, further imaging is generally required. In 38&#x0025; of the cases, cardiac catheterization was used, allowing for hemodynamic assessment. A cardiac CT scan is preferred if the airway needs to be evaluated (<xref ref-type="bibr" rid="B13">13</xref>). Moreover, compared to other modalities, a cardiac CT scan can detect a smaller or stenotic patent ductus arteriosus (<xref ref-type="bibr" rid="B14">14</xref>). MRI provides the advantage of assessing blood flow through vessels with small diameters and into the heart chambers (<xref ref-type="bibr" rid="B15">15</xref>).</p>
<p>The patient underwent left common carotid artery-subclavian bypass surgery, which reduced the stress on the vertebral artery by diverting blood flow from the left common carotid artery to the subclavian artery. The patient&#x0027;s vertigo improved following surgery. The throb of the left brachial artery and radial artery was enhanced. The bypass surgery treatment proved to be an effective method of surgery for the patient.</p>
</sec>
</body>
<back>
<sec id="s4" sec-type="data-availability"><title>Data availability statement</title>
<p>The original contributions presented in the study are included in the article/Supplementary Material, further inquiries can be directed to the corresponding author/s.</p>
</sec>
<sec id="s5"><title>Ethics statement</title>
<p>Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.</p>
</sec>
<sec id="s6"><title>Author contributions</title>
<p>JC performed the data analyses and wrote the manuscript. XW contributed to the conception of the study; CX contributed to the manuscript preparation; All authors contributed to the article and approved the submitted version.</p>
</sec>
<sec id="s7" sec-type="COI-statement"><title>Conflict of interest</title>
<p>The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.</p>
</sec>
<sec id="s8" sec-type="disclaimer"><title>Publisher&#x0027;s note</title>
<p>All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.</p>
</sec>
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</article>