Aberrant right subclavian artery: a series of case reports and discussion of morphology and its clinical and surgical implications / Arteria subclavia derecha aberrante, una serie de informes de casos y discusión de la morfología y sus implicaciones clínicas y quirúrgicas

SUMMARY: Aberrant right subclavian artery (arteria lusoria) is a rare embryological abnormality but the most common among aortic arch vascular anomalies. It represents an anatomical variant of right subclavian artery originating as the last branch of aortic arch, passing then retroesophageal to the normal position. It is usually asymptomatic and is found mostly incidentally during imaging examinations. Symptoms are produced when the aberrant artery compresses the nearby structures and the most frequent symptoms are dysphagia and dyspnea. It may be associated with other vascular or heart abnormalities. We are presenting a series of case reports which presented an aberrant right subclavian artery alone or associated with other vascular abnormalities, diagnosed incidentally in adult patients while performing CT examinations for other reasons. Through a literature review we aim to discuss the clinical implications of this vascular anomaly, to point out the importance of being aware of it especially in patients with dysphagia or dyspnea or in patients who undergo operations in the thorax and neck or vascular surgery and endovascular procedures involving the aortic arch and its branches.

RESUMEN: La arteria subclavia derecha aberrante (Arteria lusoria) es una anomalía embriológica rara, pero la más común entre las anomalías vasculares del arco aórtico. Representa una variante anatómica de la arteria subclavia derecha que se origina como la última rama del arco aórtico, pasando luego retroesofágicamente a la posición normal. Por lo general, esta anomalía es asintomática y se encuentra principalmente de manera incidental durante los exámenes de imagen. Los síntomas se producen cuando la arteria aberrante comprime las estructuras cercanas y los síntomas más frecuentes son la disfagia y la disnea. Puede estar asociado con otras anomalías vasculares o cardíacas. Presentamos una serie de informes de casos en los que se presentó una arteria subclavia derecha aberrante única o asociada a otras anomalías vasculares, diagnosticada incidentalmente en pacientes adultos durante la realización de TC por otros motivos. A través de una revisión bibliográfica pretendemos discutir las implicaciones clínicas de esta anomalía vascular, señalar la importancia de conocerla especialmente en pacientes con disfagia o disnea o en pacientes sometidos a operaciones de tórax y cuello o cirugía vascular y procedimientos endovasculares. involucrando el arco aórtico y sus ramas.

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.

Type ii aortopulmonary window with isolated left subclavian artery from left pulmonary artery

Abstract Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

Hybrid treatment of complex aortic arch anomaly with saccular aneurysm

Abstract Aortic arch anomalies are not clinically important unless they cause compression symptoms due to aneurysmatic dilatation. Aortic anomalies need to be treated when they cause complex thoracic aortic diseases, and the treatment approach has evolved over time from open surgical methods, which have high mortality and morbidity rates, to hybrid methods. A case of a 68-year-old male patient with complex aortic arch anomaly treated with hybrid arch repair is reported in this study. Aortic branches were common carotid trunk and aberrant right subclavian artery with a saccular aneurysm.

Vasos subclavios: ¿Qué tan cerca se encuentran de la clavícula? / Subclavian vessels: how close are they to the clavicle?

INTRODUCCIÓN: la lesión de los vasos subclavios durante la cirugía de clavícula es una situación rara, de suceder podría resultar incluso mortal; conocer su ubicación es indispensable para minimizar ese riesgo.OBJETIVO: determinar la ubicación y la distancia de la AS y VS respecto a la clavícula. Secundariamente, identificar las características particulares que influencien la ubicación y la distancia. MATERIALES Y MÉTODO: estudio retrospectivo, AngioTAC de tórax y cuello entre 2012 y 2017; se midió la longitud de la clavícula, distancia y dirección de los vasos subclavios en cada tercio de la clavícula, como también la angulación entre una horizontal y el centro de los vasos subclavios. Resultados: 39 AngioTC, 78 hombros. Distancia AS/clavícula tercio proximal, medio y distal 32,8mm (20,3-46,3), 15,4mm (6,8-28,0) y 62,7mm (37,0-115,4) respectivamente. La distancia VS/clavícula tercio proximal, medio y distal fue: 7,4mm (1,0-19,2), 16,2mm (6,7-34,7) y 67,1mm (29,7-117,0) respectivamente. La ubicación de AS y VS con respecto a la clavícula es posterosuperior en el tercio proximal, posteroinferior en el tercio medio e inferior en el tercio distal. CONCLUSIÓN: En el tercio proximal la vena puede estar solo a 1mm de la clavícula y la arteria a 6mm en dirección antero-posterior, resultando esa zona la más peligrosa. En el tercio medio la distancia es mayor, pudiendo estar arteria y vena a solo 6mm, la dirección de brocado más peligrosa es antero-inferior con una inclinación promedio de 45° caudal. El tercio distal es el más seguro, los vasos están al menos a 30mm de distancia hacia caudal. Nivel de evidencia III.

BACKGROUND: injury to the subclavian vessels during clavicle surgery is a rare situation, if it happens it could even be fatal; knowing their location is essential to minimize that risk. OBJECTIVE: determine location and distance of the AS and VS with respect to the clavicle. Secondarily identify particular characteristics that influence location and distance. MATERIAL AND METHODS: retrospective study, AngioTAC of thorax and neck between 2012 and 2017; it was measured the length of the clavicle, distance and direction of the subclavian vessels in each third of the clavicle and angulation between a horizontal and the center of the subclavian vessels were measured. Results: 39 AngioTC, 78 shoulders. AS / clavicle third proximal, middle and distal distance 32.8mm (20.3-46.3), 15.4mm (6.8-28.0) and 62.7mm (37.0-115.4) respectively. Distance VS / clavicle third proximal, middle and distal was: 7.4mm (1.0-19.2), 16.2mm (6.7-34.7) and 67.1mm (29.7-117.0) respectively. The location of AS and VS with respect to the clavicle is posterosuperior in the proximal third, posteroinferior in the middle third and inferior in the distal third. CONCLUSION: In the proximal third the vein can be only 1mm from the clavicle and the artery to 6mm in the anterior-posterior direction, this zone is the most dangerous. In the middle third the distance is greater, artery and vein can be only to 6mm, the most dangerous drilling direction is antero-inferior with an average inclination of 45° caudal. The distal third is the safest, the vessels are at least 30mm away from the vessels. Level of evidence III.

Arteria subclavia aberrante izquierda, hallazgo infrecuente / Aberrant left subclavian artery, an uncommon variation

Objetivo: La arteria subclavia aberrante (ASA) o arteria lusoria, es una de las malformaciones más comunes del arco aórtico; en especial su variante derecha. Por otro lado, la arteria subclavia aberrante izquierda es más rara. Presentamos un caso infrecuente de un paciente con síndrome deleción 22q11.2 y ASA izquierda con síntomas en extremidad superior izquierda. La ASA izquierda es una condición muy poco frecuente y hay escasa información sobre su tratamiento quirúrgico. La mayoría de las veces, el ASA es asintomática; especialmente en adultos. Si presenta clínica, sus síntomas son disfagia, tos, disnea, claudicación de la extremidad superior. La presencia de sintomatología es una indicación quirúrgica. El tratamiento quirúrgico de una ASA es discutido. Éste va a depender de la anatomía, comorbilidades y experiencia del cirujano.

Objective: The Aberrant Right Subclavian Artery (ARSA) or Lusoria Artery is one of the most common aortic arch malformations. Aberrant Left Subclavian Artery (ALSA) on the other hand, is a much rarer condition. We present an uncommon case of ALSA in a patient with 22q11.2 deletion syndrome with upper limb symptoms and review the treatment options. ALSA is an exceedingly rare condition and information on its surgical treatment is scarce. In most cases the presence of an ALSA is asymptomatic, especially in adults. Some of the most typical symptoms are dysphagia (dysphagia lusoria), cough, dyspnea, claudication of the upper limb extremity. When symptomatic, the patient has indication of surgery. The treatment of an ALSA is still debatable and it depends on the anatomy, comorbidities and surgeon´s experience.

O papel da ecografia vascular no manejo das complicações associadas ao acesso venoso central para hemodiálise em pacientes oncológicos: relato de dois casos e revisão da literatura / The role of vascular echography in the management of complications associated with central venous access for hemodialysis in cancer patients: two case reports and literature review

O implante de cateter venoso central para hemodiálise é comumente realizado em grandes centros e suas complicações estão, por vezes, associadas ao treinamento insuficiente de quem o realiza, mas também às condições clínicas do próprio doente. O presente estudo relata dois casos de intercorrências relacionadas ao uso do cateter de curta permanência para hemodiálise. No primeiro caso, houve inserção inadvertida da cânula na artéria subclávia esquerda e consequente trombose arterial, que foi conduzida conservadoramente, documentando-se uma boa perfusão colateral com ecografia vascular. O segundo caso ilustra o achado incidental de uma trombose venosa séptica central em paciente que havia feito uso do cateter por uma semana, optando-se por tratá-la com antibioticoterapia, anticoagulação plena e controle ecográfico. Em ambos os casos, a intervenção cirúrgica seria de alto risco devido ao prognóstico reservado dos doentes. A ultrassonografia vascular permitiu o monitoramento das situações clínicas e o emprego de terapêutica menos agressiva

Central venous catheter implantation for hemodialysis is commonly performed in large centers and its complications are sometimes associated with insufficient training of those who perform it, but may also be related to the patient's clinical condition. The present study reports two cases of complications related to use of a short-stay catheter for hemodialysis. In the first case, the cannula was inadvertently inserted into the left subclavian artery, causing arterial thrombosis, which was conservatively managed and good collateral perfusion was documented with vascular echography. The second case illustrates an incidental finding of Central Venous Septic Thrombosis in a patient who had used a catheter for a week, which was treated with antibiotic therapy, anticoagulation, and ultrasound control. In both cases, surgical intervention would have been high risk because of the patients' poor prognosis. Vascular ultrasonography enabled monitoring of these clinical situations and use of less aggressive treatments

Mujer joven con tumoración supraclavicular izquierda / Young woman with left supraclavicular tumor

Paciente femenina, de 20 años de edad. Antecedentes de padecer durante su niñez de cuadros convulsivos a repetición, con sepsis nasofaríngea frecuente y sepsis urinaria, para las que ha llevado diferentes tratamientos médicos. En agosto presentó crisis respiratoria con fiebre alta y malestar general sin tratamiento especifico. Comienza a presentar dolor intenso en el hombro y brazo izquierdo notando aumento de volumen circunscrito a la región supraclavicular irradiado al precordio...

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Avaliação da artéria subclávia direita na ecografia do primeiro trimestre / Right subclavian artery evaluation during first trimester ultrasound scan

OBJETIVO: Avaliar a artéria subclávia direita durante a ecografia do primeiro trimestre, descrever a técnica para a sua avaliação e, em caso de identificação de artéria subclávia direita aberrante (ARSA), determinar sua associação com alterações cromossómicas e/ou malformações cardíacas e sua orientação. MÉTODOS: Estudo prospectivo que consistiu na avaliação da artéria subclávia direita no primeiro trimestre (comprimento crânio caudal entre 45 e 84 milímetros), em todas as gestações únicas, consecutivas, por um único examinador, ecógrafo Voluson E8 (GE Healthcare, Zipf, Áustria) com sonda transabdominal RAB 4-8-D, 2 a 8 MHz, por um período não superior a 2 minutos, numa população geral de baixo risco. Com a ajuda do power/color Doppler, a artéria subclávia direita foi classificada como normal ou aberrante. Foi utilizada análise de regressão estatística (IBM SPSS Statistics for Windows, versão 20.0) para estudar o grau de associação entre a falha na avaliação/classificação da artéria subclávia direita e o comprimento crânio-caudal fetal e o índice de massa corporal materno. RESULTADOS: A mediana da idade materna foi de 30 anos (variando entre 17 e 43 anos) e a mediana do tempo de gestação no momento da avaliação da artéria subclávia direita foi de 12 semanas de gestação (variando entre 11 e 13 semanas de gestação). A avaliação da artéria subclávia direita foi possível em 138/176 (78,4%) dos casos. ARSA foi diagnosticada em um único caso (0,7%). Esse feto com ARSA também apresentou um foco hiperecogênico no ventrículo cardíaco esquerdo. Foi realizada ecocardiografia fetal às 16 semanas de gestação, que confirmou o diagnóstico de ARSA e foco hiperecogênico. A amniocentese revelou cariótipo normal, 46, XX. CONCLUSÃO: É possível fazer o diagnóstico de ARSA na ecografia do primeiro trimestre. O nosso único caso de ARSA apresentou um cariótipo normal sem malformações cardíacas associadas. .

PURPOSE: To determine the feasibility of evaluation of the right subclavian artery during the first trimester ultrasound scan, as well as to describe the technique for its evaluation and, in case of aberrant right subclavian artery (ARSA) identification, to determine its association with chromosomal abnormalities and/or cardiac malformations and its management. METHODS: A prospective study for evaluation of the right subclavian artery during the first trimester ultrasound scan (crown-to-rump length between 45 and 84 mm), in all consecutive single pregnancies, by a single examiner, using a Voluson E8 system (GE Healthcare, Zipf, Austria) with a 2 to 8 MHz RAB 4-8-D transabdominal probe, within a short period of time (less than 2 minutes), in a general low risk population. Color and/or power Doppler flow mapping was used to classify the right subclavian artery as normal or aberrant. Regression analysis with the IBM SPSS Statistics software for Windows, version 20.0 was used to determine the significance of the association between failure to examine/classify the right subclavian artery and both fetal crown-rump length and maternal body mass index. RESULTS : Median maternal age was 30 years (range: 17-43 years) and median gestational age at the time of evaluation of the right subclavian artery was 12 weeks (range: 11-13 weeks). The evaluation of the right subclavian artery was successful in 138/176 (78.4%) of the cases. ARSA was diagnosed in a single case (0.7%). This fetus with ARSA also presented a hyperechogenic focus on the left cardiac ventricle. Fetal echocardiography at 16 weeks of gestation was performed and confirmed ARSA and the hyperechogenic focus. Amniocentesis revealed a normal 46, XX karyotype. CONCLUSION: ARSA can be identified during a routine first trimester ultrasound scan. Our single ARSA case had a normal karyotype and no associated cardiac malformations. .

Imaging of Congenital Subclavian Artery to Subclavian Vein Fistula in a Newborn.

The purpose of this paper is to report a congenital subclavian artery to subclavian vein fistula in a three weeks old male infant who presented with congestive heart failure and pulmonary hypertension. He had been scanned by various imaging modalities including echocardiography, cardiac computed tomography, diagnostic cardiac catheterization, cardiac magnetic resonance angiocardiography and cranial computed tomoangiography. He had successful surgical ligation after failure of device occlusion trial at age of 9 months.

Thoracic Endovascular Aortic Repair with the Chimney Technique for Blunt Traumatic Pseudoaneurysm of the Aortic Arch in a No-Option Patient

A 42-year-old man was involved in a motor vehicle collision. Imaging studies revealed the presence of a post-traumatic aortic pseudo-aneurysm (about 34x26 cm) arising from the descending thoracic aorta at the level of the left subclavian artery (LSA), prone to rupture. Thoracic endovascular aneurysm repair (TEVAR) was the only feasible option due to his poor overall medical status. In this case, LSA needed to be covered in order to extend the proximal landing zone. Eventually, modified TEVAR was successfully performed by means of the chimney technique to preserve flow to the LSA and to prevent flow into the pseudoaneurysmal sac.

Multivessel cutting balloon angioplasty in a patient with type III nonspecific aortoarteritis.

A 23-year-old female patient with type III nonspecific aortoarteritis (Takayasu's arteritis) presented with multiple obstructive lesions and severe congestive heart failure. Large, cutting balloons 5-8 mm in diameter were used to dilate lesions in the abdominal aorta, both renal arteries, right common carotid artery, proximal left subclavian artery, and ostium of the left vertebral artery. Wide luminal expansion without residual stenosis, substantial dissection or need for adjunctive stenting was achieved at all six angioplasty sites. The use of cutting balloons appears suitable for treating obstructive lesions in aortoarteritis.

A decade of subclavian angioplasty: aortoarteritis versus atherosclerosis.

The therapeutic outcome of percutaneous transluminal angioplasty (PTA) for subclavian steno-occlusive disease performed over the last 10 years was assessed in 60 consecutive patients of aortoarteritis (n = 35) and atherosclerosis (n = 25). Sixty lesions were stenotic and 6 short segment total occlusions. Twenty-five lesions were prevertebral and 41 postvertebral. PTA was successful in 56 (primary success rate 93.3%) stenotic lesions and 3 (50%) total occlusions. In comparison to atherosclerosis, patients with aortoarteritis were younger (27.7 +/- 9.1 versus 54.7 +/- 10.7 years; p < 0.001), more often female (75% versus 20%; p < 0.001), gangrene was uncommon (0% versus 16%; p < 0.05) and diffuse involvement was more often seen (42.9% versus 4.0%; p < 0.001). The luminal diameter stenosis was similar before PTA (88.6 +/- 9.7% versus 89.0 +/- 9.1%; p = NS); however, aortoarteritis group had more residual stenosis (15.7 +/- 12.5% versus 8.3 +/- 9.6%; p < 0.05) after PTA. Higher balloon inflation pressure was required to dilate the lesions of aortoarteritis (9.9 +/- 4.6 versus 5.5 +/- 1.0 atm; p < 0.001). Three (5%) patients had complications which could be effectively managed nonsurgically. There were no neurological sequelae, even in PTA of prevertebral lesions. On clinical follow-up over a period of 4-120 (43.7 +/- 29.6) months in 45 of the 60 (75%) patients, higher restenosis rate (20.8% versus 4.8%; p = NS) was observed in aortoarteritis group, particularly in those patients with diffuse arterial narrowing. These lesions could be effectively redilated. Successful PTA resulted in marked improvement in symptoms on long-term follow-up. In conclusion, subclavian PTA is safe and can be as effectively performed in aortoarteritis as in atherosclerosis with good long-term results.