A Truncus Bicaroticus and an Aberrant Right Subclavian Artery. A Rare Case and Literature Review / Un Tronco Bicarótico y una Arteria Subclavia Derecha Aberrante. Un Caso Raro y Revisión de la Literatura

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

Artéria subclávia direita aberrante: relato de caso e revisão de literatura / Aberrant right subclavian artery: case report and literature review

Resumo A artéria subclávia direita aberrante, também conhecida como artéria lusória, é a anomalia do arco aórtico mais comum, ocorrendo entre 0,5 e 1% da população. Possui prevalência em mulheres e normalmente está associada a outras variações anatômicas, como o nervo laríngeo não recorrente, presente em 86,7% dos casos. Em sua maioria, a artéria subclávia direita aberrante não apresenta sintomas. Descrevemos essa alteração em uma paciente de 82 anos, hipertensa e assintomática, que havia sido submetida a uma angiotomografia toracoabdominal para a avaliação de uma dissecção crônica tipo III (DeBakey) associada à dilatação de aorta descendente. No achado, a artéria subclávia direita aberrante apresentava percurso retroesofágico associado a um divertículo de Kommerell. Devido à raridade, realizamos revisão bibliográfica integrativa das bases de dados MEDLINE, UpToDate, LILACS, SciELO e Portal CAPES dos últimos 6 anos e discutimos as alterações anatômicas mais frequentes, a sintomatologia e as condutas terapêuticas adotadas.

Abstract The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

Symptomatic aberrant right subclavian artery—A case report and anesthetic implications

Vascular compression of the esophagus by an aberrant right subclavian artery (aRSA) leading to dysphagia is a rare occurrence. There has been a significant advancement in the diagnostic and surgical treatment modalities available for this disorder. Anesthetic management has evolved too and this case report highlights the anesthetic management of a 41?year?old woman presenting with symptoms of dysphagia because of compression of esophagus by an aRSA, who subsequently underwent re?implantation of aRSA into ascending aorta.

Arteria subclavia derecha aberrante: a propósito de un caso pediátrico / Aberrant right subclavia artery: purpose of a pediatric case

Los anillos vasculares son anomalías anatómicas que ocurren durante el desarrollo embrionario del arco aórtico, sus ramas y de la arteria pulmonar. Estas estructuras vasculares pueden conducir a grados variables de sintomatología respiratoria y/o digestiva debido a la formación de un anillo completo o parcial que comprime la tráquea y/o el esófago. La arteria subclavia derecha aberrante es la anomalía más común del arco aórtico, con una incidencia reportada entre 0.5 a 2.5%. Generalmente es asintomática, sin embargo, la disfagia es el síntoma más frecuente porque en la mayoría de los casos transcurre detrás del esófago. El diagnóstico puede ser incidental en casos asintomáticos o como resultado del estudio de síntomas digestivos y/o respiratorios persistentes o recurrentes. Los pacientes sintomáticos requieren intervención quirúrgica. Describimos el caso de una paciente de 3 meses de edad con arteria subclavia derecha aberrante. El esofagograma mostró compresión extrínseca del esófago, y la angiotomografía computada confirmó el diagnóstico. La corrección quirúrgica se realizó exitosamente.

Vascular rings are anatomical abnormalities that occur during embryonic development of the aortic arch, its branches, and the pulmonary artery. These vascular structures can lead to variable degrees of respiratory and/or digestive symptoms by forming a complete or partial ring compressing the trachea and/or the esophagus. The aberrant right subclavian artery is the most common aortic arch anomaly, with reported incidence between 0.5 to 2.5%. It is generally asymptomatic; however, dysphagia is the most recognized symptom because in majority of the cases it crosses behind the esophagus. The diagnosis can be incidental when they are asymptomatic or as a result of the study of persistent or recurrent digestive and/or respiratory symptoms. Symptomatic patients require surgical intervention. We describe the case of a 3-month-old female patient with aberrant right subclavian artery. The barium esophagram showed extrinsic compression of the esophagus, and the computed tomography angiography confirmed the diagnosis. Surgical correction was successfully performed.

Prenatal Diagnosis of Aberrant Right Subclavian Artery: Association with Genetic Abnormalities / Diagnóstico pré-natal de artéria subclávia direita anômala: associação com anormalidades genéticas

Abstract Objective The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). Methods This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. Results An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed amalformation: one case of hypospadias and 1 case of cleft palate. Conclusion The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers.

Resumo Objetivo O objetivo do presente estudo foi determinar a frequência demalformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). Métodos Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico prénatal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Resultados Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. Conclusão A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

Arteria subclavia derecha aberrante aislada. Reporte de un caso / Isolated aberrant right subclavian artery. A case report

Resumen La arteria subclavia derecha aberrante (ARSA) es la anomalía del arco aórtico más común. La probabilidad de hallar un ARSA durante un estudio ecográfico es de alrededor de 1%. En aquellos fetos con ARSA, la probabilidad de tener otros hallazgos cardiacos y/o extracardiacos o una anomalía cromosómica es elevada. La prevalencia de la relación del ARSA con el síndrome de Down es de aproximadamente 20%, por lo que este marcador puede contribuir al asesoramiento del síndrome de Down en el segundo trimestre y probablemente en el primer trimestre. La recomendación ante el hallazgo de ARSA es realizar un estudio detallado de la anatomía fetal en busca de otros marcadores de aneuploidías y realizar ecocardiografía fetal. La realización de estudios invasivos quedará limitado a aquellas situaciones en donde además del ARSA se encuentren otros marcadores u otras condiciones que aumenten el riesgo de síndrome de Down. Sin embargo, ante el hallazgo de ARSA aislado, el incremento en el riesgo es igual a cero, pudiendo considerarse una variante de la normalidad.

Abstract An aberrant right subclavian artery (ARSA) is the most common branch abnormality of the aortic arch. It can be identified by ultrasound scan in 1% of cases. The probability of association with cardiac and/or extracardiac anomalies, as well as chromosomal abnormality, is high. Prevalence of ARSA with Down syndrome is approximately 20%, and this marker may contribute to counseling on Down syndrome during the second trimester and maybe in the first trimester. When ARSA is found, recommendations include a detailed study of the fetal anatomy for other markers of aneuploidy and to obtain a fetal echocardiogram. Invasive studies will be limited to those situations where, in addition to ARSA, other markers or other conditions that increase the risk of Down syndrome are found. However, the finding of an isolated ARSA increases the risk to zero and is considered a normal variant.

Unique Five Vessel Pattern Of Human Aortic Arch: A Case Report

We report here a unique vascular pattern of left sided aortic arch, detected incidentally in a 55 year old female,who underwent CECT chest for symptom of chronic cough. There were five vessels arising from the convex aspectof the arch with sequence as right common carotid artery (RCCA), left common carotid artery (LCCA), left vertebralartery (LVA), left subclavian artery (LSA) and aberrant right subclavian artery (ARSA).

Aberrant right subclavian artery (arteria lusoria) aneurysm with a Kommerell's diverticulum / Aneurisma de artéria subclávia direita aberrante (artéria lusória) com divertículo de Kommerell

The treatment options for aberrant right subclavian artery vary depending on the presence of Kommerell's diverticulum. Because there is a tendency not to report mortalities of these rare cases in the literature, it is hard to reach a conclusion on treatments from the limited data on post-interventional results in these patients. We report our experience with a 67-year old patient with an aberrant right subclavian aneurysm with Kommerell's diverticulum, diagnosed by chance

As opções de tratamento para artéria subclávia direita aberrante variam dependendo da presença de divertículo de Kommerell. Como há uma tendência a não relatar mortalidade nos raros casos descritos na literatura, é difícil chegar a uma conclusão sobre tratamentos tendo em vista os dados limitados sobre resultados pós-intervenção nesses pacientes. Relatamos aqui nossa experiência com um paciente de 67 anos de idade com aneurisma de artéria subclávia aberrante direita com divertículo de Kommerell diagnosticado ao acaso

Total Arch Replacement with Open Stent Grafting for Aberrant Right Subclavian Artery in Two Cases / 日本心臓血管外科学会雑誌

We report two cases of total arch replacement with open stent graft for the aberrant right subclavian artery (ARSA). Case 1 was a thoracic artery aneurysm with an ARSA. We thought it would be difficult to perform in-situ reconstruction of ARSA via median sternotomy, so we performed total arch replacement with the open stent-grafting technique. Therefore the right axillary artery was reconstructed by extra-anatomical bypass and coil embolization of the ARSA proximal to the vertebral artery to achieve complete thrombosis of the ARSA. The postoperative course was uneventful. Case 2 was a Stanford type A acute aortic dissection involving an ARSA with the entry located near the ARSA. Total arch replacement was performed using the open stent-grafting technique to close the entry site and origin of the ARSA. Then the right axillary artery was reconstructed by extra-anatomical bypass and coil embolization of the ARSA. The postoperative course was uneventful. The open stent-grafting technique might be an effective alternative management of thoracic aortic disease with ARSA.

Coronary Artery Bypass Grafting Combined with Total Arch Replacement for Severe Coronary Disease Complicated with Multiple Arch Vessel Stenosis / 日本心臓血管外科学会雑誌

A 72-year-old man was referred to our hospital for coronary artery bypass grafting (CABG) due to asymptomatic severe coronary artery disease. A preoperative CT revealed 99% stenosis of the aberrant right subclavian artery, 90% stenosis at the origin of right common carotid artery, and 75% stenosis at the origin of the left subclavian artery. As he had high risk of a perioperative cerebral ischemic event for CABG, it was difficult to perform a carotid artery stent. We performed a total aortic arch replacement combined with CABG. The postoperative course was uneventful, and he was discharged on the 12th postoperative day.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.

Clinical discussion of the arteria lusoria: a case report / Discussão clínica a respeito da artéria lusória: um relato de caso

Abstract The right subclavian artery may originate from the left portion of the aortic arch. This aberrant vessel is known as the arteria lusoria. Its course to its usual site runs behind the esophagus, which may cause a disease known as dysphagia lusoria, responsible for symptoms of discomfort. This artery is often associated with other anomalies, such as the non-recurrent laryngeal nerve and the bicarotid trunk, and with diseases such as aneurysms, congenital heart defects, and even genetic syndromes. During routine dissection of a male cadaver fixed in 10% formalin solution, an arteria lusoria was found. This article reports the variation and discusses its embryological, clinical and surgical aspects.

Resumo Em alguns casos, a artéria subclávia direita pode se originar da porção esquerda do arco aórtico. Esse vaso aberrante é conhecido como artéria lusória. Para chegar em sua região, essa artéria passa posteriormente ao esôfago, e pode ser, portanto, causa de uma doença conhecida como disfagia lusória, desencadeando sintomas desconfortantes. A artéria lusória está frequentemente associada com outras anomalias, como o nervo laríngeo não recorrente e o tronco bicarotídeo, assim como pode estar em associação com aneurismas, defeitos cardíacos congênitos e até síndromes genéticas. Durante dissecação cadavérica de rotina, foi observada a presença dessa artéria em um cadáver do sexo masculino fixado em uma solução de formalina a 10%. O objetivo deste trabalho é relatar a variação conhecida como artéria lusória e trazer destaque para seus aspectos embriológicos, clínicos e cirúrgicos.

Prenatal diagnosis of aberrant right subclavian artery in an unselected population

PURPOSE: The purpose of this study was to determine the frequency of aberrant right subclavian artery (ARSA) among unselected fetuses and to evaluate its association with chromosomal abnormalities and other congenital anomalies. METHODS: In all, 7,547 fetuses (gestational age, 20 to 34 weeks) were examined using routine antenatal sonography at our institution between April 2014 and September 2015. The right subclavian artery was assessed using grayscale and color Doppler ultrasonography in the transverse 3-vessel and tracheal view, and confirmed in the coronal plane. RESULTS: ARSA was found in 28 fetuses (0.4%). Further, 27 of these 28 fetuses were euploid (96.4%). Trisomy 18 was the only chromosomal anomaly (3.6%) found in the study sample. ARSA was an isolated finding in 23 of the 28 cases (82.1%). In the remaining three cases (10.7%), ARSA was accompanied with extracardiac anomalies. Other cardiac defects were present in three cases (10.7%). CONCLUSION: Isolated ARSA does not seem to be associated with a significantly increased risk of aneuploidy. However, the possibility of fetal karyotyping, which is a more invasive procedure, should be discussed in the light of the overall risk of the fetus.

Open Stent Grafting for Stanford Type A Acute Aortic Dissection Originating from an Aberrant Right Subclavian Artery / 日本心臓血管外科学会雑誌

<p>An aberrant right subclavian artery (ARSA) is a relatively rare congenital anomaly of arch branches, occurring in 0.5-2.0% of the population. Stanford type A acute aortic dissection involving an ARSA is rare, and is associated with difficult surgical planning in an emergency situation. We report a case of Stanford type A acute aortic dissection originating from an ARSA in a 50-year-old man. He was referred to our hospital with a chief complaint of chest and back pain. Contrast enhanced CT scan revealed type A aortic dissection involving an ARSA, with the entry located near the ARSA. Given the possible difficulty of performing distal anastomosis over the ARSA and ARSA reconstruction, total arch replacement was performed using the open stent-grafting technique. The postoperative course was uneventful, and a CT scan revealed a thrombosed false lumen and ARSA. The false lumen of the aorta next to the stent graft eventually disappeared at 1 year postoperatively. The open stent-grafting technique might be an effective alternative in the management of Stanford type A acute aortic dissection with ARSA.</p>

Aberrant right subclavian artery-esophageal fistula: massive upper gastrointestinal hemorrhage secondary to prolonged intubation / Fístula de artéria subclávia direita anômala com esôfago - hemorragia digestiva alta maciça secundária a intubação gástrica prolongada

ABSTRACT Aberrant right subclavian artery-esophageal fistula is a rare but potentially fatal complication. It may be associated with procedures, such as tracheostomy and tracheal or esophageal intubation, and yields massive upper gastrointestinal bleeding difficult to identify and to control. A high index of suspicion is essential for early diagnosis and better prognosis. We report a rare case of a patient who survived after emergent surgical procedure for massive upper gastrointestinal bleeding secondary to aberrant right subclavian artery-esophageal fistula after prolonged intubation.

RESUMO A fístula de artéria subclávia direita anômala com o esôfago é uma complicação rara, mas potencialmente fatal. Pode estar associada a procedimentos como traqueostomia e intubação traqueal ou esofágica e originar hemorragia digestiva alta maciça, de difícil identificação e controle. Um elevado índice de suspeição é essencial para o diagnóstico precoce e a melhoria do prognóstico. Relatamos caso raro de doente que sobreviveu após intervenção cirúrgica emergente por hemorragia digestiva alta maciça secundária a fístula de artéria subclávia direita anômala com esôfago, após intubação gástrica prolongada.

Dysphagia Lusoria: A Case Report

Aberrant right subclavian artery is a rare cause of dysphagia. This is a congenital anomaly with the right subclavian artery originating from the dorsal part of the aortic arch and coursing through the mediastinum between the esophagus and the vertebral column. We report a case of a patient with chronic dysphagia caused by this condition. We further discuss the case with regards to its clinical features and options of management.

Clinical value of scanning the initial segment of right subclavian artery routinely in carotid artery ultrasonography / 中国基层医药

Objective To explore the clinical value of scanning the initial segment of right subclavian artery routinely in carotid artery ultrasonography, thus to increase the detectable rate of arteriosclerosis.Methods 2 896 subjects who had undergone carotid artery ultrasonography were analyzed retrospectively from November 2010 to June 2015 in Shuishang Park Hospital,Tianjin.The initial segment of right subclavian artery had been examined rou-tinely in carotid artery ultrasonography from February 2013.The detectable rate of arteriosclerosis at the carotid artery was compared in the two groups.The detectable rate of arteriosclerosis at the bifurcation of common carotid artery and the initial segment of right subclavian artery was analyzed and compared in different age -groups from February 2013 to June 2015 ( group B ) .Results The detectable rate of arteriosclerosis at the carotid artery in group B (74.21%) was significantly higher than 57.34%of group A(χ2 =91.82,P=0.000).The detectable rate of arterio-sclerosis at the carotid artery in group B was increased by age ( the initial segment of right subclavian artery χ2 =181.829,P=0.000;the bifurcation of common carotid arteryχ2 =635.298,P=0.000).The detectable rate of arteri-osclerosis at the initial segment of right subclavian artery was higher than that of the bifurcation of common carotid artery in group of age 36-45 years and >45-55 years (the group of age 36-45 years χ2 =8.988,P=0.003;the group of age >45-55 yearsχ2 =19.507,P=0.000).Conclusion It is necessary that examining the initial seg-ment of right subclavian artery routinely in carotid artery ultrasonography to increase the detectable rate of arterioscle-rosis,and the initial segment of right subclavian artery is the important part to find the arteriosclerosis of carotid artery early,it is of guiding significance to prevent cardiovascular and cerebrovascular disease.

A Patient with an Aberrant Right Subclavian Artery Who Underwent Endovascular Aortic Repair for Stanford Type B Acute Aortic Dissection / 日本心臓血管外科学会雑誌

<p>Case : A 75-year-old man was brought to our hospital by ambulance with dorsal pain. Contrast-enhanced computed tomography (CT) revealed acute communicating aortic dissection (Stanford type B) complicated by an aberrant right subclavian artery (ARSCA). Under a diagnosis of type B dissection, conservative treatment by hypotensive therapy and resting was performed. One month after onset, contrast-enhanced CT showed the expansion of the false lumen, and intermittent abdominal pain persisted. To close the entry of the distal arch and reconstruct the ARSCA route, right common carotid artery-right axillary artery bypass, thoracic endovascular aortic repair (TEVAR), and coil embolization of the ARSCA were performed. Five days after surgery, contrast-enhanced CT revealed the expansion of a false abdominal lumen. Abdominal endovascular aortic repair (EVAR) was additionally conducted, leading to the disappearance of false lumen blood flow. ARSCA is a congenital arch vessel abnormality. It may cause obstruction of the esophagus/trachea, aortic aneurysm/dissection of an adjacent area, or aneurysmal changes/rupture of the ARSCA. Various techniques have been reported ; reconstruction of the ARSCA route and closure of the false lumen by de-branch TEVAR may be effective for acute communicating aortic dissection with an ARSCA.</p>

Selective use of superficial temporal artery cannulation in infants undergoing cardiac surgery.

Arterial cannulation is routinely performed in children undergoing cardiac surgery to aid the intraoperative and intensive care management. Most commonly cannulated peripheral site in children is radial artery, and alternatives include posterior tibial, dorsalis pedis, and rarely superficial temporal artery (STA). Two specific situations in cardiac surgery where STA cannulation and monitoring was useful during the surgical procedure are reported. To our knowledge, such selective use of STA pressure monitoring has not been reported in the literature previously. Our experience suggests that STA monitoring can be useful and reliable during repair of coarctation of aorta or administration of anterograde cerebral perfusion in patients having associated aberrant origin of the right subclavian artery.

A Chronic Aortic Dissection with Aberrant Right Subclavian Artery and Kommerell's Diverticulum / 日本心臓血管外科学会雑誌

A 65-year-old woman suffered from left-side paralysis and dysarthria after sudden chest pain, and we diagnosed cerebral infarction caused by type A acute aortic dissection in the Stanford classification. At that time, the aberrant right subclavian artery with Kommerell's diverticulum was found on enhanced computed tomography. The acute aortic dissection with closed false lumen was treated conservatively. Because the ulcer-like projection (ULP) expanded during the course, we performed surgery. Ascending aorta and arch replacement, patch closure of Kommerell's diverticulum and reconstruction of right subclavian artery were performed simultaneously. The postoperative course was good.