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Kommerell's diverticulum (KD) is a rare aneurysm of the origin of an aberrant subclavian artery. Hybrid aortic arch repair for KD is being performed more often. We report hybrid arch repair for KD in a 63-year-old man with a right aortic arch and aberrant right vertebral artery, an extremely rare variant. We performed total arch replacement to completely reconstruct the five cervical arteries with elephant trunk to create an adequate landing zone, followed by second-stage endovascular stent-grafting from the ascending aorta to the proximal descending aorta.

Background A right-sided aortic arch (RAArch) is present in approximately 0.1% of the population. A Kommerell’s diverticulum (KD), a remnant of the dorsal aortic arch usually refers to an aneurysmal aortic enlargement at the origin of an aberrant left subclavian artery (ALSA) and is associated with an increased risk of aortic dissection. Case summary A 59-year-old female smoker with a history of hypertension and hypercholesterolaemia presented with a 24-hour history of sudden-onset and severe stabbing chest pain radiating to the interscapular region. Physical examination was normal except for bilateral basal crepitations. Computed tomography angiography (CTA) showed a type B aortic dissection in a RAArch with an ALSA arising from KD with a peri-aortic haematoma and haemothorax without any active contrast extravasation. After medical stabilization, a semi-urgent hybrid repair was performed with a right carotid-subclavian bypass, thoracic endovascular aortic repair (TEVAR), a plug in the left subclavian artery, and left carotid-subclavian bypass due to severe ischaemia of the left arm. The postoperative CTA showed patent bypasses, aortic remodelling, and a minimal type IIa endoleak at the level of the ALSA. Discussion In patients with a type B dissection and KD, hybrid repair including TEVAR is feasible after careful pre-operative assessment of the patient’s unique anatomy and may reduce post-surgical morbidity and mortality compared to open surgery. Prophylactic repair may be considered in patients with an asymptomatic RAArch and KD.

Cardiovascular malformations during embryogenesis can lead the inferior laryngeal nerve to branching directly from the cervical vagus nerve and entering the larynx. This rare anatomical variation is known as a non-recurrent inferior laryngeal nerve (NRILN), and increases the risk of accidental injury resulting in postoperative vocal cord paralysis during neck surgery. We report a case of an 83-year-old man who presented with left symptomatic internal carotid artery stenosis with a right-sided aortic arch and aberrant left subclavian artery (ALSCA). We performed carotid endarterectomy (CEA) using intraoperative neuromonitoring to avoid NRILN injury. To the best of our knowledge, this is the first report of searching for a left NRILN by electrophysiology during CEA. Neurovascular surgeons need to understand the variations of the NRILN associated with congenital cardiovascular anomalies and effective use of intraoperative nerve monitoring (IONM). We discuss the embryological origin, IONM, and surgical pitfalls of this case.

DiGeorge’s syndrome is a 22q11.2 deletion leading to abnormal embryogenesis of pharyngeal arches and it is manifesting in a variety of clinical signs and symptoms. The spectrum of anomalies varies from minor facial dysmorphism and cleft palate to a broad spectrum of cardiovascular anomalies, thymic disfunction and immune deficiencies, hypocalcemia due tohypoparathyroidism,growth and developmental delay and speech disturbances. Cardiovascular anomalies might include right sided aortic arch, aberrant vesiclesand vascular ring. Here we present an atypical case of partial DiGeorge’s syndrome with feeding and swallowing difficulties and laryngeal stridor in the neonatal period. Early presentation in this period is usually due to severe hypocalcemia and cardiac disease. Feeding difficulties in a preterm baby needed clinical assessment skills in order to establish the diagnosis and delineate it from feeding difficulties usually seen in preterm babies. Esophagogram (barium X Ray) showed antero-posterior oblique impression towards the right side, the latero- lateral view showed impression on the rare side, suspected to be esophageal sub stenosis due to vascular anomaly, aberrant right subclavian arteryand suspectedthymic hypoplasia. We report a 9-year follow up periodbya team of subspecialists. The child had two surgeries due to aberrant vessel and velopharyngeal deficiency. Optimal management of patients with DiGeorge’s syndrome requires a multidisciplinary teamwhichshould include a cardiologist, immunologist, geneticist, speech/language therapist, endocrinologist and other subspecialists depending on patient`'s phenotype.

Circumflex aorta is a rare variant of vascular rings that consists of a right sided aortic arch, posterior crossing aorta from right to left and a left ligamentum arteriosum. While division of the ligamentum may cause partial relief of the aerodigestive tract compression, the ultimate cure for this type of vascular ring is translocation of the posterior crossing aorta anteriorly via what is known as “aortic uncrossing” procedure.

OBJECTIVES To assess the safety and efficacy of the translocation of the aberrant left subclavian artery (LSCA) and resection of the Kommerell diverticulum during the concomitant repair of intracardiac anomalies for paediatric patients who had a right-sided aortic arch. METHODS A retrospective review of paediatric patients who were diagnosed right-sided aortic arch, aberrant LSCA, Kommerell diverticulum and intracardiac anomalies between 2015 and 2019 was conducted. Patients who underwent translocation of the aberrant LSCA, diverticulum resection and concomitant intracardiac repair were included. RESULTS Eight patients underwent translocation of aberrant LSCA, diverticulum resection, ligamentum division and concomitant repair of the associated intracardiac anomalies. All patients were male. The median age was 1.3 years (range 0.4–5.5 years) and the median weight was 10.0 kg (range 6.1–21.0 kg). The most commonly combined intracardiac anomaly was a ventricular septal defect. Seven patients (87.5%) had preoperative respiratory or gastrointestinal symptoms. There was no early mortality and no postoperative complications. During the median follow-up of 23 months (range 4–43 months), no patient had residual respiratory or gastrointestinal symptoms. A postoperative computed tomography scan was performed in 3 patients, all of which showed patent LSCA–left carotid artery anastomosis. CONCLUSIONS Translocation of the aberrant LSCA and resection of the Kommerell diverticulum can be safely performed during the concomitant repair of intracardiac anomalies for paediatric patients. This approach could eliminate residual respiratory and gastrointestinal symptoms, and prevent reintervention in the future.

During the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF), cardiovascular malformations occasionally create a technical challenge. We report a novel case of a 4-day-old girl with EA/TEF and multiple cardiovascular malformations, including right-sided aortic arch (RAA), right ductus arteriosus (RDA), single ventricle with single atrium, common atrioventricular valve, pulmonary atresia, bilateral superior vena cava, and interruption of the inferior vena cava (IVC) with hemiazygos continuation. In this case, a right-sided approach would require the mobilization of the RAA and RDA, which were supplying the pulmonary blood flow due to pulmonary atresia. Alternatively, the left-sided approach would require the mobilization of the hemiazygos vein, which was essential for venous return from the lower body due to IVC interruption. We performed the less intrusive left-sided approach, and the postoperative course was uneventful. Right-sided EA/TEF repair should be avoided because RDA spasm or injury caused by RAA mobilization would be fatal. In cases of interrupted IVC with azygos or hemiazygos vein continuation, care must be taken not to ligate these vessels or block the venous return. Preoperative evaluation is important to prevent complications in such complicated cases. If sufficient information is not available, the left-sided approach may be preferred.

Absztrakt: Az aortaív fejlődési rendellenességei az újszülöttek 1-2%-ában fordulnak elő. E betegcsoportban a bal oldali aortaívből eredő jobb arteria subclavia incidenciája 0,7–2%, míg a jobb oldali aortaívből eredő bal arteria subclavia 0,4%-ra tehető. A rendellenes eredésű arteria subclavia – arteria lusoria – okozta panaszok vagy az oesophagus, vagy a trachea compressiójából, vagy azok kombinációjából származnak. Ritka szövődményként a Kommerell diverticulum B típusú aortadissectióval vagy törmelékes bennékének elsodródásával felső végtagi embolisatiót okozhat. Dolgozatunkban 5 eset ismertetését adjuk közre. Egy beteg felső végtagi embolisatióval, 2 beteg akut B típusú aortadissectióval került észlelésre. Egy beteget krónikus I. típusú thoracoabdominalis aneurysma miatt operáltunk, akinél B típusú aortadissectio állumenének thrombotizált aneurysmáját találtuk, ami Kommerell diverticulum thrombosissal társult. Egy betegünkben jobb oldali aortaívből eredő bal arteria lusoriát transponáltunk a carotis communisba. Évekkel később a centralis csonk növekedő aneurysmája miatt kellett beavatkoznunk. Sternotomiából végzett debranching után thoracalis stentgraft-implantatiót végeztünk.

Congenital variations and anomalies of the aortic arch are important entity in vascular imaging. Most of them are asymptomatic. About 30-40% presents with tracheo-oesophageal symptoms like dyspnea and dysphagia. A 27 year-old female presented with persistent dysphagia for 6 months duration and cause of persistent dysphagia was the right sided aortic arch with aberrant left subcalvian artery from Kommerell’s diverticulum. The diagnosis was made through combination of chest X-Ray, echocardiography, non-ionic contrast swallow and Computed Tomography angiography. Non-invasive modalities (CT and MR Angiography) play an important role in diagnosis and pre-operative surgical planning providing relationship with the surrounding structures especially trachea and esophagus.

Introduction and importance Congenital vascular rings are rare in adults. The management is challenging when vascular rings present with concomitant aortic aneurysm and Kommerall's diverticulum. Case presentation We present a 68year old patient with a congenital vascular ring in the form of right sided aortic arch, with a retroesophageal left subclavian artery arising from an aneurysmal Kommerell's diverticulum. In addition, the patient had aneurysmal distal aortic arch and proximal descending aorta, causing severe symptomatic tracheal compression. The patient was successfully managed by debranching and reimplantation of head neck vessels to the ascending aorta through a median sternotomy, and concurrent endovascular stenting of the aneurysmal aorta. Clinical discussion A combination of complete vascular rings and aortic arch aneurysm poses a potentially higher surgical risk. The use of the less invasive endovascular stenting of the aorta as well as a limited open surgical approach offers a lesser risk, especially in poor pre-operative states. Conclusion A multidisciplinary team effort is key. Hybrid management of aneurysmal congenital vascular rings in high risk adults offers an attractive management alternative to conventional surgery., Highlights • Aneurysmal congenital vascular rings are rare in adults. • Multidisciplinary team approach is key. • Single stage hybrid management is an alternative to open surgical management.

BACKGROUND A right sided aortic arch (RAA) and an aortic diverticulum (AD) are a rare congenital anomaly associated with an aortic rupture and dissection. Recently, various methods for the surgical management have been described to treat RAAs and ADs. CASE REPORT We describe a case of an RAA with a mirror image branching (RAMI) and AD in a teenager who complained of chest pain. We planned a total arch replacement with the frozen elephant trunk technique. Further, we needed to devise a way to reconnect the cerebral vessels because of his small arch anatomy and limited space in the mediastinum. We performed a right subclavian artery debranching, creating an anastomosis using one graft and diamond anastomosis, and performed the left innominate and right carotid anastomosis in an island fashion. This surgical approach could be a treatment option for young or small patients with RAMIs and ADs, whose arch anatomy is very small.

The right aortic arch with aberrant left subclavian artery is the most common cause of vascular ring and can be either asymptomatic or symptomatic owing to mass effect. Removal of Kommerell diverticulum and division of the ligamentum arteriosum through a left thoracotomy is currently advocated in symptomatic patients and patients with large diverticulum (>5 cm) to avoid inherent complications.

Right aortic arch with mirror image branching (RAMI) is a rare congenital defect of the aorta. The exact incidence of RAMI in the general population is unclear. In RAMI the first branch arising from the arch is the left innominate artery, followed by the right carotid artery and right subclavian arteries. We report a case of an adult female patient with RAMI discovered as an incidental finding during radiological investigations for suspected pulmonary embolism in emergency department. No other congenital malformations were reported. It is important to recognize congenital variants of the aortic arch, as they can have relevant implications for patients’ prognosis and management. Therefore, being aware of these conditions is key to avoid any mistakes or surgical and endovascular complications. Keywords: Congenital vascular anomalies, Right aortic arch, RAMI, Incidental finding

We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process. We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process.

Transposition of the great vessels (TGV) is a common congenital heart defect that is difficult to diagnose before birth. Antenatal diagnosis is associated with increased survival. Unusual features such as anomalous pulmonary artery origin may delay cyanosis, decreasing clinical suspicion. A three-week old female infant who had never been cyanotic presented for forensic autopsy due to onset of unresponsiveness at home. History included risk factors for TGV and signs of heart enlargement that were not recognized during life. Cardiac pathology consultation identified D-type TGV with additional rare anomalies. TGV may present as sudden unexplained infant death (SUID) for forensic autopsy if variant features prevent development of cyanosis. Cardiac pathology consultation is helpful in clarifying these features.

Background: Kommerell’s diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian artery, a Kommerell’s diverticulum and persisting ductus arteriosus or ductal ligament enclose the lateral side, forming a vascular ring which may result in (symptomatic) esophageal or tracheal compression. Spontaneous rupture of an aneurysmatic Kommerell’s diverticulum has also been reported. Due to the rarity of this condition and underreporting in the literature, the clinical implications of a Kommerell’s diverticulum are not well defined. Case summary: We describe seven consecutive adult patients with a right-sided aortic arch and an aberrant course of the left subclavian artery (arteria lusoria), and a Kommerell’s diverticulum, diagnosed in our tertiary hospital. One patient had severe symptoms related to the Kommerell’s diverticulum and underwent surgical repair. In total, two of the patients experienced mild non-limiting dyspnea complaints and in four patients the Kommerell’s diverticulum was incidentally documented on a computed tomography (CT) scan acquired for a different indication. The size of the Kommerell’s diverticulum ranged from 19 × 21 mm to 30 × 29 mm. In the six patients that did not undergo surgery, a strategy of periodic follow-up with structural imaging was pursued. No significant growth of the Kommerell’s diverticulum was observed and none of the patients experienced an acute aortic syndrome to date. Discussion: Kommerell’s diverticulum in the setting of a right-sided aortic arch with an aberrant left subclavian artery is frequently associated with tracheal and esophageal compression and this may result in a varying range of symptoms. Guidelines on management of Kommerell’s diverticulum are currently lacking. This case series and literature overview suggests that serial follow-up is warranted in adult patients with a Kommerell’s diverticulum with small dimensions and no symptoms, however, that surgical intervention should be considered when patients become symptomatic or when the diameter exceeds 30 mm in the absence of symptoms.

Kommerell diverticulum (KD) combined with right-sided aortic arch (RAA) and aberrant left subclavian artery (ALSA) are rare and limited to a few case reports and small series. Thoracic endovascular aortic repair (TEVAR), which is mini-invasive, is widely utilized in complicated aortic disease. We performed a systematic review of the literature to identify all patients who underwent endovascular repair for KD in terms of technical feasibility and procedural outcomes. Published and accepted studies only in English as well as article reference lists were searched and extracted to assess case series reporting solely TEVAR in KD patients. There were 28 patients with KD/RAA identified from 19 studies. All of them underwent endovascular technique for KD exclusion and the median age was 69 years (range 39–83 years). Hypertension (n=17) was the most common comorbidity in this cohort, followed by diabetes mellitus (n=3), hyperlipidemia (n=3), and smoking (n=3). The presenting symptoms were dysphagia (n=8, 29%), intermittent back pain (n=4, 14%), and acute aortic dissection (n=6, 21%), while asymptomatic was found in 9 patients (n=9, 32%). A technical success rate of 100% was reported associated with various managements of ALSA, proximal embolization (n=19, 68%), in-situ revascularization (n=3, 11%), and left carotid-subclavian bypass (n=3, 11%). All patients survived without severe complications and were discharged home within less than 14 days. The mean follow-up time was 9.3 months, patency was found in all patients, thrombosis and distinct shrinkage of KD aneurysm as indicated by CT-scans were noted (n=20, 71%), and type II endoleak was found in only 4 patients (n=4, 14%). TEVAR appears to be safe and offers favorable results, but it still needs substantial evidence to support routine use in KD. TEVAR is an alternative to open repair in selected cases, but it needs further investigation in a large cohort.

Se presenta caso de un paciente con historia de 2 meses de evolución de presentar disfagia, astenia, debilidad generalizada y deposiciones melénicas asociado a dolor abdominal tipo cólico en mesogastrio/epigastrio no irradiado, con dispepsia ocasional exacerbado con la ingesta de alimentos. La placa de tórax revela opacidad paratraqueal derecha con ausencia del boton aórtico izquierdo, con diagnóstico probable de masa mediastínica. La tomografía de torax contrastata muestra variante anatómica del arco aórtico de ubicación del lado derecho. AbstractWe present a case of a patient with a history of 2 months of evolution of having dysphagia, asthenia, generalized weakness and melanin depositions associated with colic-like abdominal pain in non-irradiated mesogastrium / epigastrium, with occasional dyspepsia exacerbated with food intake. The Chest X rays reveals band opacity paratracheal right with absence of the left aortic button, with probable diagnostic of mediastinal mass. The thoracic contrast tomography shows an anatomical variant of the aortic arch located on the right side.

Echocardiographic findings in the most common type of cyanotic congenital heart disease, tetralogy of Fallot, consists of ventricular septal defect (VSD), overriding of aorta, right ventricular hypertrophy, and right ventricular outflow tract obstruction. It should be kept in mind not to miss other associated anomalies including right sided aortic arch, abnormal coronary arteries particularly if crossing the right ventricular outflow tract, aortopulmonary collaterals, persistent left superior vena cava, additional VSDs, and other anomalies.

Anomalies of the aortic arch and its branches rarely develop. The surgery for esophageal cancer may be challenging with the presence of these anomalies. This study is aimed to analyze the influence of these variations during the esophagectomy. A total of 21 patients with aortic arch and brachiocephalic vessel variations were retrospectively identified from 2013 to 2019. Anomalies were distributed: 15 patients with left-sided aortic arch combined with aberrant right subclavian artery (LAA + ARSA), 2 right-sided aortic arch with mirror-image arch branches (RAA + MIAB), and 4 right-sided aortic arch combined with aberrant left subclavian artery (RAA + ALSA). Perioperative characteristics and long-term survival were analyzed. Tumors were mostly located in the upper and middle thorax (42.9% and 47.6%, respectively). Of the 15 patients with LAA + ARSA, 13 underwent McKeown esophagectomy and 2 via transhiatal approach. Left thoracotomy was performed on all 6 patients with RAA. The R0 resection rate was 90.5% (19/21). Recurrent laryngeal nerve (RLN) injury occurred in 2 patients. Two patients died within 30 days postoperatively. Lymph node yield was 23.6 ± 1.2, with a metastasis rate of 38.1% (8/21). The median follow-up time was 18 months. Recurrence occurred in 6 patients (11–35 months) and 4 patients died after recurrence with a median time to death of 21 months (8–47 months). For resectable esophageal cancer combined with aortic arch and its branches anomalies, satisfactory surgical results can be obtained under careful preoperative evaluation and reasonable surgical approach selection.

Spontaneous ruptured aneurysm involving an aberrant subclavian artery with a right-sided aortic arch and Kommerell’s diverticulumis a rare life-threatening condition that can be treated successfully if promptly identified. Multidetector Computed Tomography angiography is the first line imaging modality of thoracic vascular anomalies diagnosis. We report the case of a 74-year-old man suffering from this emergency ondition with mediastinal hematoma mostly extending to the left-side extrapleural cavity. The patient underwent successful emergency thoracic endovascular aortic repair and an Amplatzer vascular plug was placed into the first segment of the ALSA. Post-procedural imaging showed complete exclusion of the aneurysm. Emergency endovascular repair can be effective in such cases.

Background: A 57-year-old woman was diagnosed with Kommerell’s diverticulum in the setting of a right aortic arch on computed tomography. Case report: Although asymptomatic, the maximum diameter of the aneurysm was 55 mm; thus, she underwent surgery to prevent rupture of the aneurysm. A bypass was constructed from the left common carotid artery to the left subclavian artery. A stent-graft was deployed from the distal right subclavian artery, and coil embolization of the diverticulum was performed via the left subclavian artery. She was discharged after 12 days of surgery. The postoperative four-month follow up showed a smaller aneurysm. Conclusion: Thoracic endovascular aortic repair is feasible and effective for Kommerell’s diverticulum.

Right-sided aortic arch with aberrant left subclavian artery is a rare variant of vascular anatomy. Three types of right-sided aortic arches are described and classified based on the arrangement of the aortic arch vessels, the presence or absence of congenital heart abnormalities, the relationship of the aortic arch to the trachea and esophagus, as well as the presence or absence of a complete or incomplete vascular ring. On review of the existing literature, 31 case reports were found with a spectrum of clinical presentation sand outcomes. In this case report, we highlight a case of a young female in her early 20’swho presented with choking spells, shortness of breath along with intermittent dysphagia since childhood. She was otherwise healthy and pregnant at 26 weeks gestational age. A Computed tomography scan with angiography (CTA) of the thorax was performed to rule out a pulmonary embolism (PE) however surprisingly, a right-sided aortic arch with aberrant left subclavian artery was revealed instead. Subsequently, an extensive literature review was carried out to better understand clinical presentation sand treatment strategies for this rather rare disorder.

Non-recurrent inferior laryngeal nerve (NRILN) is rare but one of the important anatomical variations in thyroid and parathyroid surgery. Almost all cases were observed on the right side with aberrant right subclavian artery and left NRILN have been reported in only five cases so far. Here, we reported a 38 year-old Japanese male with left NRILN accompanying adenomatous goiter. He was referred to our hospital for the surgical treatment of left thyroid goiter. Preoperative computed tomography revealed right-sided aortic arch and aberrant left subclavian artery with no signs of complete situs inversus viscerum, suggesting possible left NRLN. Left hemithyroidectomy was performed using nerve monitoring system. Intraoperatively, left recurrent laryngeal nerve was not identified along tracheoesophageal groove, but directly originated from vagal nerve and was running horizontally to larynx. Mobility of vocal cords were not impaired and postoperative course was uneventful. During thyroid surgery for the patients with right-sided aortic arch, meticulous care should be taken using nerve monitoring system to avoid nerve injury.

Kommerell’s diverticulum is a rare, congenital aortic arch anomaly, usually associated with other vascular abnormalities. When present with a concurrent right-sided aortic arch and an aberrant subclavian artery, this triad can form a vascular ring that encompasses the trachea and esophagus. This anatomical variant is usually asymptomatic but can present with respiratory symptoms due to compression of the trachea. In this report, we discuss a case of a Kommerell’s diverticulum, which presented as frequent and recurring asthma exacerbations in a pediatric patient.

Dysphagia is an underestimated health problem. At the same time, it is a potentially life-threatening condition. Dysphagia in young adults is rare and thus it is rarely discussed in the literature. Vascular anomalies are much less frequent causes of dysphagia than structural pathologies of the digestive system, iatrogenic lesions or neurological causes. This paper presents a case of a 21-year-old woman with escalating dysphagia in the course of a congenital vascular anomaly in the form of a right-sided aortic arch with retroesophageal left subclavian artery (left arteria lusoria) and compression. The paper highlights delayed symptomatology of the congenital defect, reasons behind the long-lasting diagnostic process, and the role of the laryngological – phoniatric examination in order to exclude oral and pharyngeal causes. The differential diagnostics and treatment options are discussed thereinafter.

Purpose Right-sided aortic arch is a rare vessel anomaly with an incidence of 0.1% worldwide. Supra-aortic branches form a mirror image of the left-sided aortic arch or an aberrant left subclavian artery associated with Kommerell diverticulum. Most patients are diagnosed by a difference in blood pressure in each upper extremity or by the presence of left subclavian steal syndrome in their younger age. The diagnosis of onset of ischemic stroke in middle age is rare. Methods We present the case of a female patient who presented with an ischemic stroke in the left posterior circulation area. She had no history of congenital heart malformation. We performed head magnetic resonance imaging, cerebral angiography, and enhanced computed tomography of the aortic arch and major branches. Results The patient had a right-sided aortic arch and an aberrant left subclavian artery. The left subclavian artery was occluded at the proximal portion with a fibrous string. Collateral flow in the anterior cervical subcutaneous area supported left limb perfusion. Conclusion An atheromatous change reduced shunt flow via collateral networks at the anterior cervical region. Congenital subclavian steal supported the ischemic stroke.

Bicuspid aortic valve (BAV) is a common congenital cardiac malformation, frequently combined with ascending aorta dilation. However, isolated abnormalities of the aortic arch are less frequent in BAV patients. Here, we present a rare case of BAV combined with right-sided aortic arch aneurysm, aberrant left subclavian artery, and Kommerell's diverticulum, diagnosed by echocardiography and computed tomography angiography. The patient was followed-up regularly because of the life-threatening risks of aortic arch aneurysms.

Kommerell diverticulum (KD) with aberrant left subclavian artery (ALSA) is a rare congenital variation of vascular structure. We reported a case of 3-year-old boy with KD and ALSA, which was preliminarily diagnosed by transthoracic echocardiography and verified by computed tomography angiography (CTA). The patient was treated successfully with KD resection and anastomosed the left subclavian artery directly to the left carotid artery under cardiopulmonary bypass. Our case illustrates that echocardiography and CTA should be used to comprehensively assess all the internal and external cardiac structures in order to determine the appropriate surgical plan.

Background Vascular rings (VRs) are classified as true rings or partial rings. The complete VRs include double aortic arch (DAA) and right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum. The incomplete (VRs) include innominate artery compression and pulmonary artery sling. Methods We retrospectively analyzed the data from 90 patients with VRs and pulmonary artery slings diagnosed at Mansoura University Children’s Hospital between 2004 to 2016. The initial diagnostic clue might be a right sided aortic arch on a plain x-ray chest. Lateral views were evaluated for retro tracheal opacity, anterior tracheal bowing, and posterior indentation. In our series, bronchoscopy was used for patients with respiratory tract symptoms. Bronchoscopy was performed in 21 patients (23.3%), with finding of external tracheal wall pulsatile compression. Echocardiography was used initially in most cases as the first diagnostic tool followed by Multislice CT scan (MSCT). Results The age of operation was significantly earlier in patients with complete VRs versus incomplete vascular rings. By far the commonest variant was the DAA which constituted in our series 57.8%. The second common type was RAA with aberrant left subclavian and left ligamentum which constituted 30%. The third most common type was pulmonary artery sling constituting 6.7%. In our series, Echocardiography was done in 78 cases (85.6%). It was useful not only in initially diagnosing VR anomaly but also it detected associated cardiac anomalies in 4 cases. MSCT was an excellent noninvasive diagnostic modality that was used in 75 cases (83.3%). We operated 90 cases; 79 via left thoracotomy, 10 cases via median sternotomy and one case via right thoracotomy. Among the 90 cases that were operated, there was a single mortality, no cases were complicated by recurrent laryngeal nerve injury and five cases had postoperative chylothorax. Conclusions In our series, Echocardiography and MSCT were essential for proper diagnosis and planing for surgery. Left thoracotomy or median sternotomy provided good exposure in the operated cases. Surgery was done with low mortality and morbidity.

A 30-year-old diesel-engine-repair worker presented with cough and nasal regurgitation while swallowing liquids for last 10 years, impaired balance while walking and hand tremors for last 5 years, and mild exertional dyspnoea for last 1 year. General examination was normal and respiratory

Kommerell diverticulum is a rare developmental anomaly of aortic arch. It is most frequently seen with right-sided aortic arch and aberrant left subclavian artery or ligamentum arteriosum, which have a significant role in completing a vascular ring. However, aberrant origin of neck vessels along with it is not commonly seen. The signs and symptoms vary depending on the severity. The paediatric patients usually present early due to compression of mediastinal structures such as trachea or oesophagus.

Right-sided aortic arch is a rare anatomical variant present in about 0.1% of the adult population.1,2 Half of the cases are associated with an aberrant left subclavian artery (0.05%-0.1%). Right-sided aortic arch with aberrant left subclavian artery is less common than left-sided aortic arch with aberrant right subclavian artery (0.5-2.0%).3,4 A rightsided aortic arch is an anatomic variant resulting from persistence of the right fourth aortic arch and involution of the left. It can be associated with an aberrant left subclavian artery arises from Kommerell’s diverticulum. It is usually asymptomatic and diagnosed incidentally during adult age. A 40-year-old male presented with cough and a hump in the back. The patient was evaluated for scoliosis and plain CT thorax was done.

OBJECTIVE: For fetuses with a diagnosis of right aortic arch and normal cardiac anatomy, we aimed to establish the frequency of chromosomal anomaly diagnosed with single nucleotide polymorphism microarray analysis, particularly focusing on microduplications or microdeletions which would have gone undetected by conventional karyotyping and six-probe fish (13,18,21, X,Y, TUPLE). METHOD: Retrospective study of fetal ultrasounds between 2011 and 2016 in an Australian tertiary referral centre. Outcomes of interest were survival and postnatal surgery for vascular ring. RESULTS: Thirty patients were identified; 24 were apparently isolated. Chromosomal anomalies were identified in eight fetuses (32%) of 25 who had chromosomal testing. The rate in isolated cases was 11% and 56% in non-isolated cases. The 22q11.2 deletion was identified in three fetuses (12%). Microarray identified copy number variants of potential clinical significance in four additional fetuses (16%). Long continuous stretches of homozygosity were identified in one fetus with cerebellar hypoplasia potentially identifying the loci for recessive mutations. Surgery for vascular ring was performed on seven infants (25%) CONCLUSION: Microarray detected clinically significant chromosomal anomalies in fetuses with right aortic arch that would not be detected with conventional karyotyping. Prenatal counselling should include the chance of postnatal surgery and the importance of long-term follow-up. © 2017 John Wiley & Sons, Ltd.

The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

Background: Right sided aortic arch is slightly rare variant of aortic arch which requires more description and elaboration for the management in clinician point view. This may remain asymptomatic or present with various vague symptomatology. It is mandatory for the clinician to be aware of the underlying anatomical details which may sometimes be responsible for the presenting complaints. Furthermore, this anomaly may also be associated with other abnormalities. Case presentation: We present 60-years old male who complained of headache and vague chest pain of two weeks duration and underwent Contrast Enhanced Computerized Tomography (CECT) chest for further evaluation. There was an incidental finding of right sided aortic arch with Aberrant Left Subclavian Artery (ALSA). This anomaly was not responsible for any of the symptoms as per the CECT findings. Conclusion: It is important to know the detailed relationship of right sided aortic arch with ALSA to the other neighboring anatomical structures. MDCT and MRI are always helpful tools to appreciate and apprise the clinician about the details of the anomaly in view of the management if required. Sometimes the symptoms may be present because of the compression by the vascular rings.

Right-sided aortic arch is a rare anomaly (incidence 0.05%), commonly associated with an aberrant subclavian artery (35% of cases) with a Kommerell diverticulum at the origin. This aberrant artery passes posterior to the esophagus and can cause compression syndrome. We present such a case, treated with a hybrid endovascular repair. (Level of Difficulty: Advanced.)

Absence of a common carotid artery (CCA) is an extremely rare congenital anomaly. The first documented case was in 1787 during a postmortem examination. The incidence of agenesis of the CCA is unknown with fewer than 25 cases reported in literature. Agenesis of the CCA is commonly associated with separate origins of the internal carotid artery (ICA) and external carotid artery (ECA) and is usually an incidental finding on imaging studies and can pass unnoticed or misdiagnosed. Such an anomaly is typically asymptomatic unless associated with an accompanying arterial lesion that results in a work-up for symptomatic intracerebral pathology, a focal neurological deficit, or during autopsy. Although absence of the CCA is usually asymptomatic, transient ischemic attacks, hemiplegia, and intracranial hemorrhage may occur.