Dysplastic aberrant left subclavian artery originating from a thoracic intersegmental artery associated with a right aortic arch.

PURPOSE: A right aortic arch (RAA) is a rare vascular anomaly that often coexists with an aberrant left subclavian artery (ALSA). Due to the rarity of RAA, the development of an ALSA is not well understood. METHOD: We describe a case in which a 58-year-old man who was scheduled to undergo posterior decompression and fusion surgery for thoracic ossification of the posterior longitudinal ligament from Th1 to Th3 was found to have a RAA and an ALSA. RESULTS: Preoperative computed tomography angiography demonstrated a RAA and an ALSA. The ALSA was extremely tortuous and ran in the paraspinal muscles behind the thoracic laminae, which meant it was in the surgical field. The ALSA arose from the descending aorta and bifurcated into the left segmental arteries of Th1 and Th2, and also bifurcated into the left vertebral artery, which had a normal subsequent course. The dysplastic ALSA was considered to have developed from the thoracic intersegmental artery. Based on preoperative examination findings, we performed spinal surgery without vessel injury. CONCLUSION: We report a rare case of a dysplastic ALSA that developed from the thoracic intersegmental artery with a RAA. The knowledge of this anomaly provides safety in spinal surgery of the cervicothoracic junction.

[Hybrid treatment of Kommerell diverticulum and giant aneurysm of intra-thoracic segment of aberrant left subclavian artery]. / Klinicheskii sluchai gibridnogo lecheniya divertikula Kommerelya i gigantskoi anevrizmy vnutrigrudnogo segmenta aberrantnoi levoi podklyuchichnoi arterii.

Kommerell's diverticulum causes compression of the esophagus, trachea and laryngeal nerve between the aberrant mouth of the left subclavian artery and ascending aorta. This leads to dysphagia or shortness of breath. We describe hybrid treatment of the right aortic arch with Kommerell's diverticulum and giant aneurysm of the aberrant left subclavian artery.

Expanded phenotype of primary ciliary dyskinesia related to DRC1 pathogenic variant with dysmorphisms and vascular anomalies.

We present a case of a female diagnosed with primary ciliary dyskinesia (PCD) type 21 with non-previously reported extrapulmonary symptoms, including facial features and congenital vascular anomalies. Whole genome sequencing in our patient revealed a homozygous pathogenic variant in the DRC1 gene and no other notable structural nor punctual variants. This case demonstrates a unique clinical manifestation of PCD, which is possibly associated with the presence of a homozygous pathogenic DRC1 variant. Therefore, we suggest that analysis of DRC1 be considered with PCD type 21 when such features are present.

Retroesophageal left brachiocephalic vein with an ectatic aberrant left subclavian artery: A rare double vascular loop.

We hereby present a rare variant course of left brachiocephalic vein coexisting with an aberrant right subclavian artery in a patient with a ventricular septal defect with pulmonary atresia. This case highlights the importance of CT angiography in diagnosing associated aortic arch and systemic venous anomalies in complex congenital heart diseases.

Atretic anomalous left subclavian artery as part of an unusual vascular ring.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

High incidence of progressive stenosis in aberrant left subclavian artery with right aortic arch.

Right aortic arch with aberrant left subclavian artery (RAA/aLSCA) is a rare aortic arch anomaly. The clinical association of aLSCA stenosis with RAA/aLSCA has not yet been fully elucidated. The aim of this study was to investigate the diagnosis, incidence, management and outcome of aLSCA stenosis in infants with prenatally diagnosed RAA/aLSCA. Ten fetuses who were diagnosed as having RAA/aLSCA in Kyushu University Hospital between January 2011 and December 2014 were enrolled. The maternal and child medical records were reviewed to investigate sex, gestational age at the fetal diagnosis, gestational age and body weight at birth, the findings of computed tomography (CT), Doppler ultrasonography of the vertebral artery and angiography, and the complications and outcomes of aLSCA stenosis. In 8 of 10 patients, aLSCA stenosis was identified on the first CT examination after birth. No patients had dysphagia or respiratory distress. The stenosis spontaneously resolved in 3 patients. In 4 of the 5 remaining patients, aLSCA stenosis progressed, including one case in which complete occlusion occurred-the case was associated with retrograde flow from the left vertebral artery supplying the distal LSCA. Balloon angioplasty was successfully used to treat stenosis in two cases. The subclavian steal phenomenon and developmental problems were not observed in any patients. aLSCA stenosis was identified in 80% of patients with RAA/aLSCA after birth. The early detection and elective treatment of stenotic lesions may be required to prevent complete occlusion during the development of the cardiovascular and cerebrovascular systems.

Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct.

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.

Aortic arch anomalies detected in foetal life by echocardiography.

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies. Foetal echocardiograms, clinic and genetic histories of 33 patients who had been diagnosed with aortic arch anomaly from 2007 to 2015 were reviewed. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. In a patient with isolated right aortic arch, 22q11 microdeletion had been revealed. Given this data, we strongly suggest foetal karyotype analysis when aortic arch anomalies are identified. Impact Statement What is already known on this subject: The data about the prenatal diagnosis of aortic arch anomalies are limited. What the results of this study add: In our study, 653 patients were examined by foetal echocardiography during the study period. Thirty three patients who had been diagnosed with aortic arch anomaly prenatally and confirmed after delivery were enrolled in the study. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. Trisomy 18 was detected in the patient with bilateral ductus arteriosus and Di George syndrome (22q11 microdeletion) was determined in two patients with right aortic arch. While in the first patient, there were no other intracardiac anomalies; in the second patient with 22q11 microdeletion, Fallot tetralogy accompanied the right aortic arch. What the implications are of these findings for clinical practice and/or further research: This results showed that aortic arch anomalies can be associated with genetic anomalies even when they are found without other congenital heart disease. Given these data, we strongly suggest foetal karyotype analysis and genetic testing when aortic arch anomalies are identified.

Kommerell's diverticulum: A rare aortic arch anomaly.

Anomalies of the aortic arch associated with diverticulum are rare. We present a case of incidentally detected right-sided aortic arch with Kommerell's diverticulum and aberrant left subclavian artery.

Navigating the Unexpected: Dysphagia Lusoria Complicates Severe Pneumonia With Parapneumonic Effusion.

Managing pneumonia, especially when complicated by underlying anatomical anomalies, presents unique challenges that require a nuanced and multidisciplinary approach. Dysphagia lusoria, a rare vascular anomaly where the right subclavian artery originates aberrantly, can coexist with other thoracic conditions, complicating both diagnosis and treatment. Understanding the interplay between such anomalies and common infections like pneumonia is crucial for optimal patient outcomes. This case report describes a 33-year-old male with a history of recurrent pneumonia in the past who presented to the emergency department (ED) with right flank pain and dyspnea persisting for one week. Initial investigations revealed moderate parapneumonic pleural effusion and right lower lobe pneumonia. At the time, an aberrant origin of the right subclavian artery (ARSA) (dysphagia lusoria) was incidentally detected on imaging. The patient's management included antibiotic therapy tailored for pneumonia and the placement of a chest tube for pleural effusion drainage. Despite intermittent dysphagia, surgical intervention for dysphagia lusoria was deferred due to its minimal impact on daily functioning. The patient improved significantly with supportive care and antibiotics, highlighting the complexity of managing pneumonia complicated by anatomical anomalies. This case underscores the importance of multidisciplinary management and tailored treatment strategies in addressing intricate clinical scenarios.

Right-sided Aortic Arch with Aberrant Left Subclavian Artery arising from Kommerell's Diverticulum: A case report.

Right-sided aortic arch, first documented by Fioratti and Aglietti in 1763, is a rare variant of the thoracic vascular anatomy present in about 0.1% of the adult population. In half of these cases the left subclavian artery is also aberrant. The aberrant left subclavian artery usually originates from a conical dilatation near its origin from the aorta also known as "Kommerell's diverticulum." Fewer than 80 of these cases have been reported in the literature as far as our web search is concerned. It is usually asymptomatic and diagnosed incidentally during adulthood. We are presenting a 56 years old male patient presented with right side chest and shoulder pain of 1 week duration. The pain exacerbated with motion of the right upper extremity and radiates to his lower back. However, he had no history of cough, shortness of breath, syncope, and dysphagia. The vital signs were in normal range. Pulmonary and cardiovascular exam were unremarkable. The complete blood count (CBC), electrocardiogram (EKG), and echocardiography showed no abnormality. In the adult population a right-sided aortic arch with an aberrant left subclavian artery arising from Kommerell's diverticulum is a rare occurrence often asymptomatic unless aneurysmal disease or compression of mediastinal structures ensues. Even though it is rare and at times an incidental finding, the condition is clinically relevant because of the morbidity caused by the complications. We report a case of Kommerell's Diverticulum of an aberrant left subclavian artery in an adult patient with a right-sided aortic arch. Right-sided aortic arch with aberrant left subclavian artery arising from Kommerell's Diverticulum is quite rare and may remain asymptomatic. On times it may cause symptoms in adulthood often as a result of early atherosclerotic changes of the anomalous vessels, dissection, or aneurysmal dilatation with compression of adjacent structures causing dysphagia, dyspnea, cough, or chest pain. Even though there are no general guidelines for the management of this condition patients need to be informed about the nature and possible outcomes of their condition. Close follow up of asymptomatic patients is one option of management until there are situations which require consideration of surgical intervention.

A Unique Association of Right-Sided Aortic Arch With Kommerell's Diverticulum, Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, and Typical Carcinoid: A Case Report.

Our case presents a unique occurrence marking the first documentation of a connection between a typical carcinoid in the context of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and vascular anomalies, including a right-sided aortic arch with Kommerell's diverticulum. Kommerell's diverticulum is a rare congenital anomaly. The lusoria subclavian artery, another developmental anomaly, arises from the right aortic arch instead of the typical left side. Neuroendocrine cells may contribute to lung disease pathogenesis by altering their physiology before clinical symptoms appear. A 56-year-old woman with an unyielding chronic cough underwent diagnostic evaluation, unveiling rare vascular anomalies alongside a pulmonary nodule. Radiological investigations disclosed a solid nodule in the middle lobe, accompanied by proximal right-sided aortic arch ectasia and an aberrant left subclavian artery. Following multidisciplinary deliberation, thoracic and vascular surgeons elected for surgical nodule resection. Utilizing uniportal video-assisted thoracoscopic surgery, the procedure revealed the anomaly of the right-sided aortic arch. Preliminary histological examination indicated a low-grade pulmonary carcinoid, obviating the need for further lymphadenectomy due to its low malignancy potential. Subsequent histological analysis confirmed a well-differentiated neuroendocrine tumor G1 consistent with typical carcinoid within a DIPNECH framework. Currently, the patient is in follow-up. This case underscores the importance of multidisciplinary evaluation and tailored surgical approaches for managing patients with rare vascular anomalies and pulmonary nodules, emphasizing the requisite comprehensive preoperative assessment and collaborative efforts among diverse medical specialties to optimize outcomes.

Interarm Blood Pressure Difference Revealing a Right-Sided Aortic Arch and Occluded Aberrant Left Subclavian Artery.

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies as Aberrant Right Subclavian Artery, Right Aortic Arch and Double Aortic Arch.

We aimed to evaluate retrospectively associated anomalies and outcome in prenatal aortic arch anomalies (AAAs). We included ninety patients with aberrant right subclavian artery (ARSA), right aortic arch (RAA) with mirror image branching (RAA-mirror) or aberrant left subclavian artery (RAA-ALSA) and double aortic arch (DAA) between 2011 and 2020. In total, 19/90 (21.1%) had chromosomal anomalies, the highest rate being within the ARSA subgroup (17/46, 37%). All (13/13) of the RAA-mirror subgroup, 10/27 (37.0%) of RAA-ALSA, 13/46 (28.3%) of ARSA and 0/4 within the DAA subgroup had additional intracardiac anomaly. The rate of extracardiac anomalies was 30.7% in RAA-mirror, 28.3% in ARSA, 25.0% in DAA and 22.2% in the RAA-ALSA subgroup. A total of 42/90 (46.7%) had isolated AAAs: three (7.1%) with chromosomal anomalies, all trisomy 21 (3/26, 11.5%) within the ARSA subgroup. Out of 90, 19 (21.1%) were lost to follow-up (FU). Two (2.2%) intrauterine deaths occurred, and six (6.7%) with chromosomal anomalies terminated their pregnancy. In total, 63 (70.0%) were liveborn, 3/63 (4.8%) with severe comorbidity had compassionate care and 3/60 (5.0%) were lost to FU. The survival rate in the intention-to-treat cohort was 53/57 (93%). Forty-one (77.4%) presented with vascular ring/sling, two (4.9%) with RAA-ALSA developed symptoms and one (2.4%) needed an operation. We conclude that intervention due to vascular ring is rarely necessary. NIPT could be useful in isolated ARSA cases without higher a priori risk for trisomy 21 and after exclusion of other anomalies.

Single-incision, off-pump repair of a right aortic arch with an aberrant left subclavian artery and diverticulum of Kommerell in an adult.

Repairing a complete vascular ring in adults can be challenging. A right aortic arch with an aberrant retro-oesophageal left subclavian artery and persistent diverticulum of Kommerell represents one of the most common variants seen in adults, and the ring is completed by the left-sided ligamentum arteriosum. Most presentations in adults occur secondary to oesophageal compression, resulting in varying degrees of dysphagia. Owing to the difficulty and challenges associated with exposure in adults, it is not unusual for surgeons to offer a two-incision approach or to stage the procedure. We present a detailed surgical technique for a single-incision repair of a right aortic arch with an aberrant retro-oesophageal left subclavian artery via a left posterolateral thoracotomy.

Rare Combination of Right Aortic Arch, Aberrant Left Subclavian Artery and Absent Left Pulmonary Artery in a Boy with Tetralogy of Fallot.

Introduction: Right aortic arch is associated with an aberrant subclavian artery and absence of left pulmonary artery in patients with Tetralogy of Fallot. However, the occurrence of a combination of the right aortic arch, an aberrant left subclavian artery, and an absent left pulmonary artery in a single patient is rare. Therefore, the purpose of this case report was to discuss the rare association between a right aortic arch, the absence of a left pulmonary artery, and an aberrant left subclavian artery in an 8-year-old Ethiopian boy with Tetralogy of Fallot. Case Presentation: An 8-year-old male child who presented with easy fatigability, dizziness, and intermittent difficulty in swallowing solid food over the past two years. He had grade clubbing, cyanosis of the lips, and an ejection systolic murmur at the left lower sternal border. Tetralogy of Fallot with absent left pulmonary and right aortic lesions was diagnosed using echocardiography. CT tomography revealed a detailed vascular anatomy and confirmed the diagnosis of an aberrant left subclavian artery. Conclusion: In the evaluation of patients with Tetralogy of Fallot, it is important to pay close attention to vascular abnormalities such as right aortic arch, aberrant left subclavian artery, and absent left pulmonary artery for not missing them.

Total arch replacement with frozen elephant trunk technique for Kommerell's diverticulum with a right-sided aortic arch and aberrant left subclavian artery.

BACKGROUND: Kommerell's diverticulum with a right-sided aortic arch and aberrant left subclavian artery is uncommon. We perforemed a single-stage procedure with the frozen elephant trunk technique. CASE PRESENTATION: A 62-year-old man underwent aortic dissection a year ago, and computerized tomographic angiography performed at that time revealed a right aortic arch, Kommerell's diverticulum (42 mm), and an aberrant left subclavian artery. We performed one-stage repair through median sternotomy. The cervical branches were exposed during the operation, and a deep hypothermic circulatory arrest with antegrade cerebral perfusion was established. The aorta was transected distally to the origin of the left carotid artery. We inserted a stent graft into the aorta, followed by peripheral anastomosis using a premade 5-branch Dacron graft. The right subclavian artery and the aorta were reconstructed, and the remaining cervical branches were reconstructed after the cross-clamp had been released. CONCLUSIONS: Total arch replacement through median sternotomy was performed for the right aortic arch, Kommerell's diverticulum, and aberrant left subclavian artery. The frozen elephant trunk technique is allowed to perform a one-stage operation safely.

Accidental central venous catheter cannulation into aberrant arterial anatomy requiring endovascular intervention.

Central venous catheter placement continues to be an extremely common procedure throughout hospital systems. Although ultrasound guidance can mitigate some placement risks, misplacement of lines into neighboring structures, such as arteries, remains an unfortunate complication. In this report, we will discuss an 83-year-old female with aberrant left subclavian artery and right sided arch, which provided for successful stent graft coverage of arterial injury secondary to accidental subclavian artery cannulation with a central venous catheter with preservation of the right common carotid artery and avoidance of a potentially morbid sternotomy.

Carotid-subclavian bypass and endovascular aortic repair of Kommerell's diverticulum with aberrant left subclavian artery: A case report.

BACKGROUND: Kommerell's diverticulum (KD) with aberrant left subclavian artery is a rare congenital deformity and also has very little research literature about it (35% of case study). There are three types of aortic arch diverticulum. Even literature concerning the treatment options are limited. CASE SUMMARY: We present a case report of a 50-year-old male with KD in the right aortic arch with aberrant left subclavian artery. We conducted a total endovascular repair procedure, which is innovative and will spread more light in the medical world. Our patient has no past medical history and is a non-smoker and non-alcoholic. Patient presented with shortness of breath, chest pain and dizziness for six months. Blood tests were done and computerized tomography (CT) angiogram of the chest confirmed the diagnosis, illustrating showed a 3.9 cm KD. On Day 1, the CT angiogram showed mild dilatation of the thoracic aorta, adjacent esophagus, trachea was compressed and displaced. Surgery was planned as the treatment modality. Carotid-Subclavian artery bypass and endovascular aortic repair was conducted. We used prolene 5-0 C1 sutures to precisely anastomose a 6-mm Dacron graft to the left subclavian artery. Haemostasis was secured and wounds were closed. Protamine was administered and patient was shifted to intensive care unit. Post-operative, patient responded favorably and was discharged. Regular follow-up is done. CONCLUSION: The procedure we performed is novel. This will help the cardio-thoracic surgeons a better insight about the full procedures we conducted, thereby bringing more light and better treatment options in managing KD with aberrant subclavian artery.

Post-operative Outcomes for Vascular Rings: A Systematic Review and Meta-analysis.

OBJECTIVES: Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms. METHODS: PubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms. RESULTS: Nineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0-1.0], post-surgical complication rate 18% [95% CI: 12.0-23.0], prevalence of reintervention 3% [95% CI: 1.0-5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0-52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0-0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0-23.0], reintervention rate was 2% [95% CI: 0.0-4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0-55.0]. CONCLUSIONS: While surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.