Utilizing Single-Branched Stent in Combination With Fenestration or Chimney for Endovascular Repair of Aortic Arch Lesions With Aberrant Subclavian Artery.

OBJECTIVE: The study was to figure out the feasibility, efficacy, and safety of a single-branched stent graft, namely Castor, in combination with fenestration or chimney in the context of aortic arch lesions presenting with aberrant subclavian artery (ASA) and/or Kommerell's diverticulum (KD). METHODS: All consecutive patients with aortic arch lesions and ASA and/or KD receiving Castor from June 2018 to June 2023 were investigated. RESULTS: Incorporating 18 patients, the study encompassed 11 cases with KD, 3 cases with dysphagia; 2 cases of right-sided aortic arch with left-sided aberrant left subclavian artery (ALSA), and 16 cases of left-sided aortic arch with right-sided aberrant right subclavian artery (ARSA). The mean operation time was 132±23 minutes. The mean measured proximal aortic diameter was 30.9±1.6 mm, and proximal diameter of Castor stent was 34 (32, 34.5) mm, with oversize of 9.1±1.6%; the mean measured branch diameter was 8.8±0.97 mm, and branch diameter of Castor stent was 10 (8, 10) mm, with oversize of 0.86±0.57 mm. Technical success rate was 100%, and no in-hospital mortality, no stroke, and no endoleak were identified. One (5.6%) case with spinal cord ischemia and one (5.6%) case with poor healing of operative site were identified. During the follow-up period, no aortic-related death or secondary intervention was recorded. The maximal aortic diameter was significantly reduced at the sixth postoperative month (padj=0.031); KD diameter was significantly reduced at the third (padj=0.001) and sixth (padj<0.001) postoperative month. CONCLUSION: Totally endovascular repair of aortic arch lesions with ASA and KD via Castor stent in combination with fenestration or chimney is feasible, effective, and safe, which can achieve an encouraging medium-term outcome and provide excellent remodeling at the lesions. CLINICAL IMPACT: Single branched stent in combination with fenestration or chimney achieved a sufficient proximal landing zone and provided an encouraging medium-term outcome in this retrospective review of 18 patients receiving endovascular treatment of pathological aortic arch with aberrant subclavian artery and/or Kommerell's diverticulum. The authors suggest this time-saving and efficient technique to establish systematic experience for the treatment in this kind of patients.

Lusory dysphagia: Unusual cause of intermittent dysphagia.

INTRODUCTION AND IMPORTANCE: Lusory dysphagia (LD) is a rare disorder resulting from extrinsic vascular compression of the esophagus, is often associated with an aberrant right subclavian artery (ARSA). LD is characterized by abnormal development of the right fourth aortic arch during embryonic stages, leading to posterior coursing of the artery in 80 % of cases. Predominantly presenting as dysphagia, LD can occasionally manifest with laryngeal stridor or dyspnea, yet it often remains asymptomatic and incidentally discovered. CASE PRESENTATION: We present a case of LD in a 24-year-old male with Down syndrome, experiencing prolonged dysphagia and recurrent aspiration episodes. Emergency upper gastrointestinal endoscopy revealed food impactation beyond the upper esophageal sphincter, and subsequent imaging studies confirmed an aberrant right subclavian artery causing extrinsic compression. DISCUSSION: LD is a rare condition. It is a challenging diagnosis often necessitating a combination of endoscopy, contrasted imaging and angiography. Treatment strategies vary, with conservative approaches for mild symptoms involving dietary modifications, and surgical intervention considered for persistent or severe cases. CONCLUSION: Recognizing LD in the context of long-standing dysphagia is crucial for accurate diagnosis and appropriate management. Further research is needed to establish standardized diagnostic and therapeutic guidelines for this uncommon condition.

Hybrid Management of Dysphagia Lusoria with Tevar Implantation and Bilateral Subclavian Arteries Debranching: A Review of the Literature and a Case Report.

Aberrant right subclavian artery (ARSA) causing dysphagia, the so-called "dysphagia lusoria", is a frequent embryologic anomaly of the aortic arch. In symptomatic patients, studies report several management options including surgical, hybrid, and totally endovascular strategies. Hybrid techniques have the advantage of no chest opening with reduced morbidity, but the problem of the ARSA stump causing recurrent or persistent dysphagia remains challenging in some cases. We conducted a literature review on the management strategies of ARSA and presented the case of a 72-year-old female patient with ARSA and dysphagia managed with thoracic endovascular repair of the aorta (TEVAR) and bilateral carotid-subclavian artery bypass. This technique was chosen because of the severe calcifications at the level of ARSA origin that would make surgical ligation difficult, or if an occluder device not suitable. We think that a patient-tailored approach should be considered in cases of dysphagia lusoria, considering that a multitude of strategies are reported.

Technical considerations in robotic aberrant right subclavian artery resection for dysphagia lusoria.

Dysphagia lusoria occurs due to compression of the esophagus as an aberrant right subclavian artery (ARSA) crosses the mediastinum. Surgical management includes open, hybrid, and endovascular techniques, with no consensus gold standard. There are few reports of robotic-assisted ARSA resection. We describe the innovative technique and outcomes for two patients who successfully underwent robotic-assisted transthoracic resection of an ARSA after right carotid-subclavian bypass for dysphagia lusoria. Both patients experienced improvement or resolution of their dysphagia and no major complications. In select patients with a noncalcified origin of the ARSA without aneurysmal degeneration, the robotic-assisted approach represents a viable option.

Repair of thoracic aortic aneurysm with bilateral aberrant subclavian artery.

We present a rare anatomical configuration of a 19-year-old woman, characterized by descending thoracic aortic aneurysm with right aberrant subclavian arteries with a Kommerell's diverticulum in a left aortic arch. The complexity of this vascular anomaly was accompanied by an anomalous origin of left subclavian artery. The patient underwent a single-stage open surgical repair via left thoracotomy under deep hypothermic circulatory arrest. The bilateral aberrant subclavian arteries were separately reconstructed in situ using hand-sewn branched grafts.

Association of left ventricular outflow tract size with arch morphology in interrupted aortic arch.

OBJECTIVES: Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention. METHODS: All surgical patients with IAA (2001-2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention. RESULTS: Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5-9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68-5.80), IAA-B: 4.60 (3.92-5.50), P = 0.007], AAb [AAb: 4.00 (3.70-5.04) versus none: 5.15 (4.30-5.68), P = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70-5.02) versus other: 5.00 (4.30-5.68), P = 0.002]. The likelihood of LVOTO was higher among AAb [N = 6 (25%) vs N = 1 (2%), P = 0.004] and IAA-B + AAbS [N = 1 (2%) vs N = 6 (30%), P = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P = 0.11). CONCLUSIONS: IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair.

Case Report: Kommerell's diverticulum and left aberrant subclavian artery stenosis hybrid treatment with branched aortic stent-graft.

Kommerell's diverticulum in association with left or right aberrant subclavian arteries is a rare finding and is challenging to treat. Contemporary surgical and endovascular techniques provide a broad arsenal of possible treatments. Imaging techniques and modeling technology allow a more personalized strategy for each patient. In this case, we present a symptomatic patient with a Kommerell's diverticulum and a left aberrant subclavian artery complicated by proximal stenosis and poststenotic aneurysm. A hybrid technique using a single-branched thoracic stent-graft (Castor, MicroPort Medical, Shanghai, China) in combination with a surgical left subclavian-carotid bypass and endovascular occlusion of the poststenotic aneurysm using a vascular plug device (Amplatzer Vascular Plug, Abbott, Chicago, United States) was performed. This approach was planned and facilitated by the use of a 3D model. Alternative treatment options and the strengths of this approach are briefly reviewed and discussed.

Arteria subclavia derecha retroesofágica: reporte de caso / Right retroesophageal subclavian artery: case report

RESUMEN: Durante el desarrollo embrionario suelen ocurrir anomalías a nivel del arco aórtico primordial. Una de estas es la arteria subclavia retroesofágica derecha (ASDR) la cual es un defecto frecuente de los arcos aórticos embriológicos y se debe a la interrupción del cuarto arco aórtico (AO) derecho entre las depresiones para la arteria carótida común y la arteria subclavia. Las variaciones anatómicas y morfológicas del AO y sus ramas son significativas para los procedimientos diagnósticos y quirúrgicos en el tórax y el cuello. Reportar una ASDR encontrada durante disección, correlacionando esta con sus implicancias clínicas. De un cadáver de sexo femenino, de edad y causa de muerte desconocida, fijado con formaldehído al 10 % y aguada destilada. Se realizó disección del corazón y los grandes vasos, en especial el AO, desarrollado según la técnica convencional, para remover el órgano luego de disecar el pericardio y disecar la arteria aorta ascendente y el AO, preservando plenamente su configuración externa y sus ramas colaterales, donde se encontró la ASDR. La ASDR presentó un diámetro en su origen de 12,13 mm. La longitud existente entre la ASDR y la Arteria carótida común Derecha (ACCD) fue de 43,84 mm. El diámetro del esófago a nivel cefálico y caudal de la ASDR alcanzó valores de 17,59 mm y 13,82 mm respectivamente. Por su parte los diámetros de la tráquea a nivel cefálico y caudal a la ASDR, fueron respectivamente 22,12 mm y 13,30 mm. El conocimiento de esta variante anatómica resulta de gran interés a la hora de interpretar y orientar el diagnóstico de las posibles causas de una disfagia asociada a una arteria subclavia retroesofágica y fundamenta aún más la importancia del estudio mediante disección.

SUMMARY: During embryonic development, anomalies usually occur at the primordial aortic arch (AA) level. One of these is the right retroesophageal subclavian artery (RSA). The anatomical and morphological variations of AA and its branches are significant for diagnostic and surgical procedures in the thorax and neck. The objective of the study was to report an RSA found during the dissection, correlating this with the corresponding clinical implications. A human female cadaver of unknown age and cause of death was used and conserved in 10 % formaldehyde and distilled wash. The dissection of the heart and large vessels was performed. There was particular emphasis one the AA, and development according to conventional techniques, removing the organ after dissecting the pericardium and dissecting the ascending aorta and the AA. External function and its collateral branches were fully preserved, where the RSA was located. The RSA presented a diameter at its origin of 12.13 mm. The length between the RSA and the common right carotid artery (CRCA) was 43.84 mm. The diameter of the esophagus at the cephalic and caudal level of the RSA has values of 17.59 mm and 13.82 mm respectively. Furthermore, the diameters of the trachea at cephalic and caudal level to the RSA, respectively, were 22.12 mm and 13.30 mm. Knowledge of this anatomical variant is of great interest when interpreting and guiding the diagnosis of potential causes of a dysphagia associated with a retroesophageal subclavian artery, and is even more important during dissection studies.

Anillos vasculares: Serie de 28 pacientes atendidos en un Servicio de Endoscopía Respiratoria Pediátrica / Vascular rings: Series of 28 patients of a Pediatrie Respiratory Endoscopy Department

Los anillos vasculares tienen una incidencia del 1 % entre las enfermedades cardiovasculares congénitas. Constituyen una malformación embriológica en la que el arco aórtico, sus ramas o las arterias pulmonares provocan presión sobre la tráquea y/o el esófago. Anatómicamente, se dividen en dos grupos, dependiendo de cómo rodean la tráquea y el esófago: completo o incompleto. La sintomatología suele iniciarse en los primeros meses de vida con estridor bifásico o espiratorio que aumenta con el llanto y la alimentación, tos traqueal de tonalidad metálica, infecciones respiratorias recurrentes, episodios de apnea refleja y cianosis, sibilancias, hiperextensión cervical, retracción esternal e intercostal, y dificultad en la alimentación. Se realizó un análisis retrospectivo de 28 pacientes con diagnóstico de anillo vascular que concurrieron a la consulta en el Servicio de Endoscopía Respiratoria del Hospital Garrahan entre enero de 2015 y septiembre de 2017.

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented.

Tratamiento híbrido de arteria subclavia derecha aberrante / Hybrid treatment aberrant right subclavian artery

Introduction: An aberrant right subclavian artery is the most frequent aortic arch malformation. It is frequently an incidental finding of imaging studies and serious complications may arise if left untreated. Clinical case: We present a case of a young woman with a dilated aberrant right subclavian artery that was successfully treated by a hybrid approach.

Introducción: La arteria subclavia derecha aberrante es la malformación arterial más frecuente del arco aórtico. Su diagnóstico es habitualmente un hallazgo de estudios de imágenes solicitados por otras causas pero puede tener serias complicaciones si se obtiene en forma tardía. Caso clínico: Presentamos el caso de una mujer joven con diagnóstico de arteria subclavia lusoria dilatada sometida exitosamente a tratamiento híbrido electivo.

Arteria subclavia aberrante / Aberrant subclavian artery

Se estudiaron las variaciones en el origen y trayecto de la arteria subclavia derecha aberrante, observada en un cadáver utilizado para docencia de anatomía humana. En este caso, el arco aórtico da origen a cuatro ramas separadas, que de derecha a izquierda son: arteria carótida común derecha, arteria carótida común izquierda, arteria subclavia izquierda y arteria subclavia derecha. Desde este origen, la arteria subclavia derecha transcurre por detrás de la tráquea y del esófago, a la altura del cuerpo vertebral de T III, para luego retomar su trayecto normal hacia la raíz del cuello. Se midieron los diámetros del arco aórtico y de sus ramas. Asimismo, se consignaron los díametros y la distancia de origen de ambas arterias vertebrales, desde las arterias subclavias. Esto se realizó con el objeto de analizar posibles asimetrías importantes relacionadas con la aparición de esta variación. En el texto se discuten las probables razones embriológicas que permiten explicar el origen anómalo de la arteria subclavia derecha.

The anatomical variations involving the origin and passage of the right aberrant subclavian artery were studied, based on findings in a cadaver used for human anatomy teaching. In this case, the aortic arch gives rise to four separated branches, from right to left: right common carotid artery, left common carotid artery, left subclavian artery and right subclavian artery. From its origin, the right subclavian artery runs behind the trachea and esophagus, at the level of the third thoracic vertebrae, afterwards it reassumes its normal passage to the neck. The diameter of the aortic arch and its four branches were measured. In both vertebral arteries the diameter and distance from its origin in the subclavian arteries to the origin of the subclavian arteries were measured, with the purpose of analyzing potential important asymmetries. The probable embryologic explanations for this anomaly are discussed.

Disfagia lusoria: Reporte de un caso y revisión de literatura / Dysphagia lusoria: A case report and review of the literature

La compresión extrínseca del esófago por estructuras vasculares es una causa poco frecuente de disfagia; sin embargo, su diagnóstico es de gran importancia para ofrecer un adecuado manejo y disminuir la repercusión sobre la calidad de vida de los pacientes. Se presenta un caso ilustrativo de disfagia lusoria y posteriormente una revisión sobre su etiología, enfoque diagnóstico y manejo.

Extrinsic esophagus compression produced by vascular structures is a rare cause of dysphagia. Nevertheless, its diagnosis is critical to allow an appropriate management and to lower the impact on the patients’ quality of life. Here, an illustrative case of dysphagia lusoria is presented followed by a review of its etiology, diagnosis approach and its treatment.