Outcomes of Patients Undergoing Surgery for Complete Vascular Rings.

BACKGROUND: Few studies describe outcomes after complete vascular ring surgery in a comprehensive manner. OBJECTIVES: This study sought to describe the clinical presentation, diagnostic work-up, operative approach, and outcomes in children undergoing surgery for complete vascular rings. METHODS: This single-center retrospective cohort study includes consecutive patients (January 1990 through September 2023) undergoing primary surgery for complete vascular rings, or rerepair after primary surgery elsewhere. The primary outcome of interest was complete (as distinct from partial) symptom resolution at latest clinic follow-up. Our current preference is to pursue a comprehensive initial operation including adjunctive vascular and airway procedures targeting common causes of residual aerodigestive symptoms, such as Kommerell diverticulum resection and tracheobronchopexy. Preoperative work-up routinely involved computed tomographic angiography, dynamic bronchoscopy, and laryngoscopy. RESULTS: Of 515 patients (including 39 rerepairs), the most common diagnoses were right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum (n = 323, 62.7%) and double aortic arch (n = 174, 33.8%). There was no perioperative mortality. Chylothorax occurred in 28 patients (5.4%), vocal cord dysfunction in 22 patients (4.3%), and diaphragm paralysis in 2 patients (0.4%). Follow-up was available on 453 patients (88.0%) with a median duration of 3.0 years (Q1-Q3: 0.6-9.2 years). At latest clinic follow-up, 429 patients (94.7%) reported complete symptom resolution. The risk of reoperation for residual or recurrent aerodigestive symptoms was 9.6% (95% CI: 5.7%-13.5%) at 10 years and 12.4% at 20 years (95% CI: 6.9%-17.8%). CONCLUSIONS: Surgery for complete vascular rings provides good symptomatic relief with low risk of complications, whereas reoperations for aerodigestive symptoms are infrequent.

Outcomes for Children With Vascular Ring Repair Using a Simple Surgical Approach.

INTRODUCTION: Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair-simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a "simple" surgical approach. METHODS: We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair. RESULTS: Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6). CONCLUSIONS: Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms.

Resolution of chronic regurgitation in an 8 month old Babydoll sheep by thoracoscopic treatment of a vascular ring anomaly.

OBJECTIVE: The objective was to describe the successful thoracoscopic treatment of esophageal entrapment resulting from a vascular ring anomaly (VRA) comprising a persistent right aortic arch (PRAA) and left ligamentum arteriosum (LA) in a Babydoll sheep wether. STUDY DESIGN: Case report. ANIMAL: Eight month old Babydoll sheep wether, 13 kg. METHODS: The patient presented with a weight half that of its sibling, persistent regurgitation following eating, and delayed growth noted from the age of approximately 2 months, coinciding with the introduction of solid feed into the diet. Plain thoracic radiographs were within normal limits but computed tomography angiography (CTA) confirmed multiple congenital vascular anomalies. The primary finding was esophageal and tracheal entrapment by a PRAA and left LA. Thoracoscopic transection of the LA was performed with a bipolar vessel sealing device with the aid of transesophageal endoscopy. RESULTS: Immediate improvement in attitude and absence of regurgitation were observed. The patient was discharged and subsequently reintroduced to grazing and long-stem hay, which were previously not tolerated. By 6 months post discharge, the patient's weight was 36 kg, comparable to an age-matched sibling and considered appropriate for the stage of growth. CONCLUSION: Thoracoscopic transection of the LA in sheep is a feasible treatment for esophageal compression resulting from a VRA. Surgical intervention resolved the clinical signs and allowed normal digestive rumination, restoring bidirectional esophageal function in a ruminant.

[Double aortic arch and aortotracheal fistula as cause of fatal massive bleeding: a rare and dangerous association]. / Doble arco aórtico y fistula aortotraqueal como causa de hemorragia masiva fatal: una asociación rara y peligrosa.

Vascular rings are unusual congenital malformations. Among them, double aortic arch (DAA) is often difficult to diagnose due to its low incidence of symptoms. DAA can be associated with tracheal or esophageal compression and, in severe cases, could require tracheal intubation or chronic use of a nasogastric tube. This scenario favors the development of aortotracheal fistulas (ATF) or aortoe-sophageal fistulas (AEF). OBJECTIVE: To present a clinical case with an unusual association of DAA with ATF and to reinforce the importance of maintaining high diagnostic suspicion in patients with massive aerodigestive bleeding without an obvious source. CLINICAL CASE: A 32-week preterm newborn who required prolonged mechanical ventilation and presented intermittent episodes of massive oropharyngeal bleeding with hemodynamic compromise associated with lower airway obstruction without pulmonary hemorrhage. The patient underwent upper endoscopy and exploratory laparotomy without evidence of bleeding. Flexible nasopharyngolaryngoscopy and direct laryngoscopy also showed no abnormalities. A CT angiography showed complete DAA with indentation of the left dominant arch over the trachea, without severe stenosis or evidence of a fistula. AEF was suspected, so exploratory surgery was considered. However, the patient died before surgery due to a massive pulmonary hemorrhage. The autopsy revealed the presence of ATF. CONCLUSIONS: In patients with massive aerodigestive bleeding without an obvious source, the presence of DAA and possible AEF/ ATF should be considered. Imaging studies have a poor performance for this diagnosis, so surgery should be considered for diagnosis and treatment in these patients.

[Right Middle Lung Cancer with Double Aortic Arch:Report of a Case].

Double aortic arch is an embryological abnormality of the aortic arch forming a vascular ring. It has been noted that the right recurrent nerve travels differently in patients with a duplicated aortic arch and may be in close proximity to the area of superior mediastinal lymph node dissection in lung cancer. We report a surgical case of a patient with right middle lung cancer associated with a duplicated aortic arch. A 64-year-old man was referred to our hospital because of a nodular shadow in the right lung field noted on chest X-ray during a medical checkup. A transbronchial needle biopsy revealed a diagnosis of adenocarcinoma, and right middle lobe resection and lymph node dissection were performed. When dissecting the superior mediastinal lymph nodes in a patient with an overlapping aortic arch, it was necessary to carefully perform the operation, paying attention to the running of the right recurrent nerve.

Variations in the Evaluation and Management of Vascular Rings: A Survey of American Clinicians.

Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf's alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients.

Contralateral Thoracotomy With Extracorporeal Circulation for Reoperative Resection of a Kommerell Diverticulum.

Reoperative vascular ring surgery is uncommon. Standard redo ipsilateral thoracotomy may present technical challenges and risks. We describe a patient with right aortic arch, aberrant left subclavian artery, and a Kommerell diverticulum in whom previous vascular ring division via left thoracotomy did not relieve dysphagia. Three years after the unsuccessful operation, left subclavian-carotid transposition via supraclavicular incision followed by resection of the Kommerell diverticulum via right thoracotomy with extracorporeal circulation relieved symptoms. Contralateral thoracotomy with extracorporeal circulation provides a safe, alternative approach to redo ipsilateral thoracotomy for resection of a symptomatic Kommerell diverticulum. We review the literature on the incidence, surgical indications, and operative approaches to manage symptoms from a Kommerell diverticulum.

Perioperative and Anesthetic Considerations in Vascular Rings and Slings.

Vascular rings represent an increasingly prevalent and diverse set of congenital malformations in which the aortic arch and its primary branches encircle and constrict the esophagus and trachea. Perioperative management varies significantly based on the type of lesion, its associated comorbidities, and the compromise of adjacent structures. Multiple review articles have been published describing the scope of vascular rings and relevant concerns from a surgical perspective. This review seeks to discuss the perioperative implications and recommendations of such pathology from the perspective of an anesthesia provider.

Prenatal diagnosis of right aortic arch: associated anomalies and fetal prognosis according to different subtypes.

OBJECTIVES: Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes. METHODS: This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA). RESULTS: A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery. CONCLUSIONS: The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus.

Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography.

INTRODUCTION: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery. The first branch of the mRAA is the left brachiocephalic artery, which appears to be the same as that of an incomplete DAA due to blood flow interruption. The present retrospective study aimed to evaluate the differences between DAA and mRAA by fetal echocardiography. METHODS: This single retrospective cohort study included all patients diagnosed with complete DAA, incomplete DAA, or mRAA at our facility between 2010 and 2022. The patients were diagnosed with complete DAA, incomplete DAA, or mRAA after birth and remaining fetal echocardiograms. The patients were divided into the DAA (complete DAA: n = 4, incomplete DAA: n = 3) and mRAA (n = 4) groups. The following three outcomes were compared: (1) angle between the right aortic arch and first branch (RF angle), (2) ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta, and (3) maximum tracheal diameter on a three-vessel trachea view. RESULTS: The incomplete DAA cases were difficult to diagnose via fetal echocardiography. On fetal echocardiography, the RF angle was significantly steeper in the DAA group than in the mRAA group (median 57° [36°-69°] vs. 75° [62°-94°]; p < 0.05). The DAA and RAA groups showed no significant differences in the ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta (median 0.57 [0.17-0.68] vs. 0.73 [0.56-1.0]) and maximum tracheal diameter (median 2.5 [1.4-3.3] vs. 3.2 [2.8-3.5] mm). The cut-off value for the presence of DAA was an RF angle <71°. CONCLUSION: The DAA group (complete and incomplete DAA) had a significantly steeper RF angle than the mRAA group. Therefore, RF angle measurement could improve the fetal diagnosis and postnatal prognosis of DAA.

Narrowing Down the Symptomatology of Isolated Vascular Rings in Children.

Vascular rings may cause respiratory or gastrointestinal symptoms due to compression of the trachea and/or esophagus. Advances in imaging have enabled early detection in asymptomatic patients posing new management dilemmas. Surgery is expected to relieve symptoms, although this has not been well studied. We sought to evaluate the presence and pattern of symptoms associated with vascular rings before surgical intervention and to detail symptom resolution after surgery. A 10-year retrospective review of patients diagnosed with an isolated vascular ring was performed between January 2010 and December 2019. 100 patients were identified; 35 double aortic arch (DAA) and 65 right aortic arch and left ligamentum arteriosum (RALL). 73 patients were symptomatic on presentation; 47 had respiratory, 5 had gastrointestinal, and 21 had both types of symptoms. Surgical repair was performed in 75 patients; 74 were symptomatic. Respiratory symptoms were more likely in patients with preoperative tracheal narrowing (p < 0.001). Moderate-severe respiratory symptoms led to surgery in RALL patients (OR 10.6, p = 0.0001). DAA patients were more likely to undergo surgery (p < 0.001) irrespective of symptom severity. At a median post-surgical follow-up of 4 months, there was a significant reduction in symptom burden (p < 0.001), except for asthma symptoms (p = 0.131). Symptom resolution was not dependent on the vascular ring anatomy (p = 0.331) or the age at operation (p = 0.158). Vascular rings are typically accompanied by respiratory symptoms and less commonly GI symptoms, both of which resolve in most patients after surgery. Those who present predominantly with asthma-like symptoms may not achieve resolution after surgery.

Management of esophageal cancer in patients with a right aortic arch or double aortic arch: a case series of 34 cases.

BACKGROUND: Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported. METHODS: We analyzed RAA and DAA cases treated with esophagectomy in our center's database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA. RESULTS: We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts. CONCLUSIONS: Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.

Confusion from prenatal diagnosis: Type C persistent fifth aortic arch or right-side ductus arteriosus.

We described a case of a double aortic arch (DAA) with a subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC). We experienced uncertainty regarding this interesting case despite the diagnosis of right-sided ductus arteriosus. The ductus arteriosus originates from the right pulmonary artery (PA) and converges into the descending aorta (DAO), whereas the vessel originated from the PA and converged into the ascending aorta (AAO). Therefore, we assumed that the vessel connecting the PA to AAO may be a type-C persistent fifth aortic arch (PFAA).

Persistence of aerodigestive symptoms after vascular ring repair.

OBJECTIVE: Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair are considered excellent, the long-term effect of tracheal and esophageal remodeling and the persistence of symptoms have scarcely been reported. Our study aims to evaluate the respiratory and swallowing outcomes of vascular ring repair. STUDY DESIGN AND METHODS: A retrospective cohort study of children who underwent vascular ring repair between 2010 and 2022 in a tertiary-care children's hospital. RESULTS: There were 108 patients enrolled: sixty-three patients (57.41 %) with a right aortic arch, 42 patients (38.89 %) with a double aortic arch, and 3 patients (2.78 %) with other vascular rings. Forty-three (39.81 %) patients were diagnosed prenatally. Of the 65 patients (60.19 %) diagnosed postnatally, 35/65 (53.85 %) had either respiratory or swallowing symptoms as the indication for diagnostic workup. Persistent respiratory and swallowing symptoms were noted in 34/108 (31.48 %) and 30/108 (27.78 %) patients, respectively, within a year of surgical repair. Fourteen patients underwent repeated laryngoscopy and bronchoscopy that demonstrated residual tracheomalacia; however, only 2/14 (1.9 %) patients required tracheostomy tube placement, and 6-out-of-7 patients were weaned off positive pressure airway support. Persistent respiratory symptoms were significantly more common in patients with a double aortic arch compared to a right aortic arch. No differences were noted in demographics, comorbidities, and preoperative aerodigestive symptoms between patients with residual symptoms and patients with no residual symptoms. CONCLUSIONS: Persistent respiratory and swallowing symptoms after vascular ring repair are not uncommon. Postoperative evaluation should be pursued by a dedicated team, and treatment considered as appropriate.

Persistent right aortic arch and aberrant left subclavian artery in a dog / Persistência de arco aórtico direito e artéria subclávia esquerda aberrante em um cão

A 1.5-year-old male German Shepherd dog was referred to a Teaching Veterinary Hospital with a chronic history of regurgitation and a previous presumptive diagnosis of megaesophagus. An esophagogram showed partial esophageal dilation, suggesting one vascular ring anomaly. Computed tomography identified a persistent right aortic arch (PRAA) and an aberrant left subclavian artery (ALSA). The patient underwent thoracotomy, ligamentum arteriosum ligation, and debridement of the periesophageal region. The ligation of the ALSA was not carried out because the esophagus was observed to be released entirely during the surgical intervention. Therefore, intervention on the subclavian artery was not necessary. Clinical follow-up occurred on seven, 14, and 30 postoperative days. The dog improved, showing only sporadic regurgitations. Clinical history and complementary exams were essential to establish a diagnosis. The liberation of the esophageal transit during surgery contributed to the decision not to perform the ALSA ligation.(AU)

Um cão pastor alemão, macho, de 1,5 anos de idade, foi atendido em um Hospital Veterinário Universitário com história crônica de regurgitação e diagnóstico presuntivo prévio de megaesôfago. Um esofagograma mostrou dilatação parcial do esôfago sugerindo uma anomalia de anel vascular. A tomografia computadorizada identificou persistência do arco aórtico direito (PAAD) e artéria subclávia esquerda aberrante (ALSA). O paciente foi submetido à toracotomia, ligadura do ligamento arterioso e desbridamento da região periesofágica. A ligadura da ALSA não foi realizada, pois, durante a intervenção cirúrgica, observou-se que o esôfago estava completamente liberado, não sendo necessária intervenção na artéria subclávia. O acompanhamento clínico ocorreu aos sete, 14 e 30 dias de pós-operatório. O cão evidenciou boa recuperação, apresentando apenas regurgitações esporádicas. A história clínica associada aos exames complementares foi essencial para o diagnóstico. A liberação do trânsito esofágico durante a cirurgia contribuiu para a decisão de não realizar a ligadura da ALSA.(AU)

Incomplete vascular ring of the aortic arch presenting with dysphagia in an adult: case report.

Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the anomalies are asymptomatic and often discovered as incidental findings. In the great majority of the symptomatic cases, the presentation may be either with breathlessness or dysphagia or both. This is in addition to the nature of the intrinsic arterial disease of the aberrant vessel, especially in adult patients; and unless borne in mind, the diagnosis is often missed leading to delays and wrong treatment. In this report we present a case of dysphagia in an adult male Nigerian initially diagnosed as œsophageal stricture from herbal potion ingestion but review of his imaging investigations gave a final diagnosis of dysphagia lusoria from an aberrant right subclavian artery. The difficulty in making a diagnosis and the need for a multidisciplinary review of the imaging investigations are highlighted. The patient was successfully treated by a combined trans-thoracic and cervical approach with division and re-implantation of the aberrant vessel unto the right common carotid artery. He has remained symptom-free for 2 years after surgery. Although the great majority of these anomalies are often asymptomatic, it is important they are borne in mind both in imaging investigations as well as in procedures involving structures in the upper visceral mediastinum. Various surgical approaches have been documented in the management of symptomatic ones; it is however recommended that options that ensure revascularization of the affected limb be selected.

Vascular ring anomaly with a right patent ductus arteriosus and a left aortic arch in a juvenile cat.

This paper reports the clinical findings and surgical treatment of feline right patent ductus arteriosus (RPDA) with a left aortic arch. A two-month-old female Maine Coon was referred for an investigation of regurgitation after weaning. RPDA with a left aortic arch was diagnosed based on the echocardiographic and computed tomography (CT) findings. A right-fourth intercostal thoracotomy was found to be an appropriate approach to the duct. Preoperative diagnosis is crucial and diagnostic imaging, including radiography, echocardiography, and cardiac CT examination, is essential for determining if the aortic arch is right or left.