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nor were the ascending thoracic aorta or the descending thoracic aorta&#46; The maximum diameter of the ascending thoracic aorta was 39 mm&#46; Subsequently&#44; aortography &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41; visualized a bulbous origin of the right subclavian artery&#44; diagnostic of KD&#46; Placement of an endoprosthesis at the outlet of the ARSA was ruled out on technical grounds due to the lack of an anchoring zone between the two subclavian arteries&#46; Echocardiography showed a bicuspid aortic valve without flow alterations&#44; aortic root and ascending aorta of normal size&#44; and both ventricles of normal size and contractility&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the patient was stable&#44; elective surgery was performed&#44; through a posterolateral thoracotomy in the fourth left intercostal space&#46; Cardiopulmonary bypass &#40;CPB&#41; was established by femoral artery and vein cannulation&#46; The aortic cross-clamp was placed at the mid-distal arch&#44; proximal to the origin of the left subclavian artery&#44; and on the descending thoracic aorta after the distal margin of the aneurysm&#46; Myocardial arrest was not established&#44; which allowed upper body perfusion with a beating heart through patent supra-aortic trunks&#46; CPB was responsible for lower body perfusion&#44; with the arterial cannula positioned in the descending thoracic aorta&#59; there was no need for deep hypothermic circulatory arrest&#46; The aneurysmal segment was resected and replaced by a 28-mm Dacron tube between the origin of the two subclavian arteries and the descending thoracic aorta distal to the origin of the ARSA&#46; Finally&#44; the proximal segment of the ARSA was ligated with a continuous polypropylene suture in two planes&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient&#39;s postoperative course was favorable with hemodynamic stability and early extubation&#46; There were no clinical or Doppler signs of critical ischemia in either upper extremity&#46; Postoperative CTA &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41; revealed no sign of complications in the thoracic aorta replacement&#46; Both the right subclavian and the internal thoracic artery were perfused by the vertebral artery through the thoracic inlet&#46; Postoperative echocardiography showed the bicuspid aortic valve to be free of flow alterations&#44; and biventricular function remained good&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Clinically&#44; the patient had dysphonia and dysphagia&#46; At discharge&#44; the dysphagia was resolved but dysphonia persisted&#46; At the six-month postoperative visit&#44; the patient was clinically asymptomatic in cardiorespiratory terms&#44; reporting no chest pain&#44; palpitation or dyspnea&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Preoperative dysphonia is usually due to compression of the recurrent laryngeal nerve&#44; and may be associated with Horner syndrome by compression of sympathetic ganglia and efferent fibers&#46; During surgery&#44; it is important to identify the recurrent nerve as it passes through the aortic arch in order to prevent injury or irritation that may result in dysphonia due to vocal cord paralysis&#46; Once the injury has occurred&#44; vocal rehabilitation may lead to functional recovery&#44; depending on the degree of injury&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">The indications for surgical repair in KD have not been established&#44; due to the small number of patients with the condition&#46; Most asymptomatic patients present with rupture&#46; In addition&#44; there are insufficient data to predict rupture based on the size of the diverticulum&#46; However&#44; surgical repair is recommended for symptomatic aneurysms with a diameter of 50 mm or more&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> Our case presented as a complication&#44; and was symptomatic&#44; so surgical repair was recommended&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Some authors recommend surgical repair even in the absence of symptoms due to the risk of aneurysm formation and subsequent dissection&#44; regardless of size at diagnosis&#46; This is related to the embryological origin of this anomaly&#46; In the embryo&#44; the main arterial ducts consist of a right and left dorsal aorta arising from the aortic sac connected to the truncus arteriosus&#46; The six aortic arches give rise to the great arteries&#46; The normal right subclavian artery derives embryologically from the fourth right aortic arch&#44; a portion of the right dorsal aorta and the seventh right intersegmental artery&#46; KD is believed to be a remnant of an isolated residual right dorsal arch&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">KD usually occurs in one of three forms&#46; First&#44; cases with an aberrant left subclavian artery are a consequence of regression in the fourth left aortic arch between the left carotid and the left subclavian arteries&#46; Second&#44; in ARSA&#44; the fourth arch anomaly is the consequence of regression of the right arch between the right subclavian artery and the right and left carotid arteries&#44; and the KD is a remnant of the primitive dorsal right aorta&#46; A third abnormality is a KD in a left aortic arch with a right descending aorta&#44; but this is extremely rare&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Selection of the specific treatment to be adopted is based on patient comorbidities and anatomy&#44; and the surgeon&#39;s preference&#46; Less invasive options are preferred as long as the end result is not compromised&#46; Many patients&#44; however&#44; do not have adequate landing zones for fixation of a stent graft even with adjunctive cervical debranching&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> In the case described&#44; there was no area for stent fixation&#44; so we opted for open surgery instead of an endovascular technique&#46; In addition&#44; no replacement of the ascending thoracic aorta was performed because the ascending aorta measured less than 45 mm with a competent bicuspid aortic valve&#44; although patient follow-up was required&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0060" class="elsevierStylePara elsevierViewall">ARSA is the most frequent anomaly of the aortic arch&#46; In asymptomatic cases there are no well-established guidelines for treatment due to its rarity and heterogeneity of presentation&#44; but when a KD is complicated by aortic dissection the treatment is surgery&#46; Choice of the specific technique used is based on patient comorbidities and anatomy&#44; and the surgeon&#39;s preference&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Funding sources</span><p id="par0065" class="elsevierStylePara elsevierViewall">No sources of funding&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Case report
Dissected aberrant right subclavian artery with Kommerell diverticulum
Artéria subclávia direita aberrante dissecada com divertículo de Kommerell
Carlos Domínguez-Massa
Corresponding author
dominguez.massa@gmail.com

Corresponding author.
, Alberto Berbel-Bonillo, Manuel Pérez-Guillen, José A. Montero-Argudo
Department of Cardiovascular Surgery, Hospital Universitario y Politécnico La Fe, Valencia, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Aberrant right subclavian artery &#40;ARSA&#41; is the most frequent anomaly of the aortic arch&#44; with a reported prevalence ranging between 0&#46;2&#37; and 2&#46;5&#37; of the population&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1&#8211;3</span></a> and is associated with Kommerell diverticulum &#40;KD&#41; in 60&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1&#44;2</span></a> KD is one of the vascular ring group of congenital heart defects and consists of a dilatation of the origin of the subclavian arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> It is usually asymptomatic&#44; but when there are symptoms the most frequent is dysphagia due to compression of the esophagus&#44; followed by asthma due to tracheal compression&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> The diagnosis is made by computed tomography angiography &#40;CTA&#41;&#44; magnetic resonance imaging&#44; or the reference technique&#44; arteriography&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">When KD is complicated by aortic dissection the treatment is surgery&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> However&#44; in asymptomatic cases there are no well-established guidelines for treatment due to its rarity and heterogeneity of presentation&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> In general&#44; surgical intervention is recommended in symptomatic cases&#44; or when the diverticulum is large &#40;in some cases reaching 50 mm&#41;&#44; due to the risk of rupture or embolization&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">A 54-year-old man arrived at the hospital with symptoms of abrupt chest pain radiating to the neck&#44; not relieved by nitrates&#46; The patient had a history of ARSA diagnosed in another hospital&#44; with dysphagia of unknown duration&#46; Due to the symptoms of acute aortic syndrome&#44; CTA was performed &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#44; findings of which were compatible with a dissecting aneurysm of an ARSA compressing the esophagus&#46; The origin of the left subclavian&#44; left carotid and right carotid arteries were not dissected&#44; nor were the ascending thoracic aorta or the descending thoracic aorta&#46; The maximum diameter of the ascending thoracic aorta was 39 mm&#46; Subsequently&#44; aortography &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41; visualized a bulbous origin of the right subclavian artery&#44; diagnostic of KD&#46; Placement of an endoprosthesis at the outlet of the ARSA was ruled out on technical grounds due to the lack of an anchoring zone between the two subclavian arteries&#46; Echocardiography showed a bicuspid aortic valve without flow alterations&#44; aortic root and ascending aorta of normal size&#44; and both ventricles of normal size and contractility&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the patient was stable&#44; elective surgery was performed&#44; through a posterolateral thoracotomy in the fourth left intercostal space&#46; Cardiopulmonary bypass &#40;CPB&#41; was established by femoral artery and vein cannulation&#46; The aortic cross-clamp was placed at the mid-distal arch&#44; proximal to the origin of the left subclavian artery&#44; and on the descending thoracic aorta after the distal margin of the aneurysm&#46; Myocardial arrest was not established&#44; which allowed upper body perfusion with a beating heart through patent supra-aortic trunks&#46; CPB was responsible for lower body perfusion&#44; with the arterial cannula positioned in the descending thoracic aorta&#59; there was no need for deep hypothermic circulatory arrest&#46; The aneurysmal segment was resected and replaced by a 28-mm Dacron tube between the origin of the two subclavian arteries and the descending thoracic aorta distal to the origin of the ARSA&#46; Finally&#44; the proximal segment of the ARSA was ligated with a continuous polypropylene suture in two planes&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient&#39;s postoperative course was favorable with hemodynamic stability and early extubation&#46; There were no clinical or Doppler signs of critical ischemia in either upper extremity&#46; Postoperative CTA &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41; revealed no sign of complications in the thoracic aorta replacement&#46; Both the right subclavian and the internal thoracic artery were perfused by the vertebral artery through the thoracic inlet&#46; Postoperative echocardiography showed the bicuspid aortic valve to be free of flow alterations&#44; and biventricular function remained good&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Clinically&#44; the patient had dysphonia and dysphagia&#46; At discharge&#44; the dysphagia was resolved but dysphonia persisted&#46; At the six-month postoperative visit&#44; the patient was clinically asymptomatic in cardiorespiratory terms&#44; reporting no chest pain&#44; palpitation or dyspnea&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Preoperative dysphonia is usually due to compression of the recurrent laryngeal nerve&#44; and may be associated with Horner syndrome by compression of sympathetic ganglia and efferent fibers&#46; During surgery&#44; it is important to identify the recurrent nerve as it passes through the aortic arch in order to prevent injury or irritation that may result in dysphonia due to vocal cord paralysis&#46; Once the injury has occurred&#44; vocal rehabilitation may lead to functional recovery&#44; depending on the degree of injury&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">The indications for surgical repair in KD have not been established&#44; due to the small number of patients with the condition&#46; Most asymptomatic patients present with rupture&#46; In addition&#44; there are insufficient data to predict rupture based on the size of the diverticulum&#46; However&#44; surgical repair is recommended for symptomatic aneurysms with a diameter of 50 mm or more&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> Our case presented as a complication&#44; and was symptomatic&#44; so surgical repair was recommended&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Some authors recommend surgical repair even in the absence of symptoms due to the risk of aneurysm formation and subsequent dissection&#44; regardless of size at diagnosis&#46; This is related to the embryological origin of this anomaly&#46; In the embryo&#44; the main arterial ducts consist of a right and left dorsal aorta arising from the aortic sac connected to the truncus arteriosus&#46; The six aortic arches give rise to the great arteries&#46; The normal right subclavian artery derives embryologically from the fourth right aortic arch&#44; a portion of the right dorsal aorta and the seventh right intersegmental artery&#46; KD is believed to be a remnant of an isolated residual right dorsal arch&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">KD usually occurs in one of three forms&#46; First&#44; cases with an aberrant left subclavian artery are a consequence of regression in the fourth left aortic arch between the left carotid and the left subclavian arteries&#46; Second&#44; in ARSA&#44; the fourth arch anomaly is the consequence of regression of the right arch between the right subclavian artery and the right and left carotid arteries&#44; and the KD is a remnant of the primitive dorsal right aorta&#46; A third abnormality is a KD in a left aortic arch with a right descending aorta&#44; but this is extremely rare&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Selection of the specific treatment to be adopted is based on patient comorbidities and anatomy&#44; and the surgeon&#39;s preference&#46; Less invasive options are preferred as long as the end result is not compromised&#46; Many patients&#44; however&#44; do not have adequate landing zones for fixation of a stent graft even with adjunctive cervical debranching&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> In the case described&#44; there was no area for stent fixation&#44; so we opted for open surgery instead of an endovascular technique&#46; In addition&#44; no replacement of the ascending thoracic aorta was performed because the ascending aorta measured less than 45 mm with a competent bicuspid aortic valve&#44; although patient follow-up was required&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0060" class="elsevierStylePara elsevierViewall">ARSA is the most frequent anomaly of the aortic arch&#46; In asymptomatic cases there are no well-established guidelines for treatment due to its rarity and heterogeneity of presentation&#44; but when a KD is complicated by aortic dissection the treatment is surgery&#46; Choice of the specific technique used is based on patient comorbidities and anatomy&#44; and the surgeon&#39;s preference&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Funding sources</span><p id="par0065" class="elsevierStylePara elsevierViewall">No sources of funding&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Aberrant right subclavian artery is the most frequent anomaly of the aortic arch&#44; and in 60&#37; of cases is associated with Kommerell diverticulum&#46; It is usually asymptomatic but may present with dysphagia or asthma due to esophageal and tracheal compression&#46; Indications for surgical repair have not been established&#59; however&#44; when Kommerell diverticulum is complicated by aortic dissection the treatment is surgery&#46; We present the case of a 54-year-old man with thoracic pain due to dissection of an aberrant right subclavian artery associated with Kommerell diverticulum&#46; Elective surgical treatment was performed&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A art&#233;ria subcl&#225;via direita aberrante &#233; a anomalia mais frequente do arco a&#243;rtico&#44; em 60&#37; dos casos &#233; associada ao divert&#237;culo de Kommerell&#46; &#201; geralmente assintom&#225;tica ou pode apresentar disfagia ou dispneia&#44; devido &#224; compress&#227;o do es&#243;fago ou traqueia&#46; As indica&#231;&#245;es para a repara&#231;&#227;o cir&#250;rgica n&#227;o foram estabelecidas&#59; no entanto&#44; quando o divert&#237;culo de Kommerell &#233; complicado por dissec&#231;&#227;o a&#243;rtica&#44; o tratamento &#233; cir&#250;rgico&#46; Apresentamos um caso de um homem de 54 anos&#44; com dor tor&#225;cica devido &#224; dissec&#231;&#227;o de uma art&#233;ria subcl&#225;via direita aberrante associada ao divert&#237;culo de Kommerell&#46; &#201; feito um tratamento cir&#250;rgico preferencial&#46;</p></span>"
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                      "titulo" => "Arco a&#243;rtico derecho junto con arteria subclavia izquierda aberrante asociada con divert&#237;culo de Kommerell"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
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                            0 => "M&#46;J&#46; Valle-Caballero"
                            1 => "M&#46;&#193;&#46; Guti&#233;rrez-Mart&#237;n"
                            2 => "C&#46; Caparr&#243;s-Escudero"
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Revista Portuguesa de Cardiologia (English edition)
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