A 4-year old boy with a history of poor feeding, dyspnea and irritability while feeding that started since infancy, was referred to our center. Long-term examination of the disorder was started by echocardiography since he was one-month old and was performed when he was 2 years old. Although in all those evaluations, right sided aortic arch (RSAA) and increased right ventricle (RV) and pulmonary artery (PA) pressures were noticed, the definite diagnosis was not reached at that occasion. The patient was referred to our radiographic center and preceeded to undergo CT angiography with multislice spiral thin section scans plus maximum intensity projection (MIP), multiplanar reconstruction (MPR) and volume rendering techniques. The computed tomography (CT) angiographic findings revealed type C aortic arch interruption with right sided aortic arch which is a very rare condition and only one similar case has been previously reported in the literature. Interestingly, almost all types of right sided aortic arch interruptions reported so far were of type B. The reason why our case is type C could be explained from an embryologic point of view. Explanation of this case could be helpful and attractive since echocardiographic and angiographic findings were not definitive enough for final diagnosis of this case. The symptoms intensified after repairing the patient’ s ventricular septal defect (VSD); however, definite diagnosis was obtained by CT angiography. What makes this case report more important is that the authors believe that diagnostic methods in developed countries are able to diagnose this congenital heart disease sooner in neonatal or even in prenatal periods, resulting in a lower incidence of presentation of similar cases in childhood.

Background :Congenital aortic arch anomalies encompass various malformations, which can range from asymptomatic to presenting with severe respiratory or gastrointestinal issues. These anomalies may occur alone or alongside other congenital heart defects. Imaging is crucial for identifying these conditions and guiding accurate preoperative decisions.Objectives :This study aimed to assess the frequency of these abnormalities in patients with a right aortic arch, explore the reasons for their referral, and evaluate patient outcomes. Methods :This cross-sectional study was conducted on children under the age of 20 who visited the Congenital Heart Diseases Clinic at Imam Reza Hospital, Iran. We collected data from 277 records of patients with right aortic arch from 2002 to 2022. Data were gathered using a checklist, including echocardiographic results, angiography findings, and other diagnostic modalities for the patients. Statistical analysis was performed using IBM SPSS software, Version 25. Results :In this study, 118 (29.8%) patients had heterotaxia, of which 106 (26.8%) had dextrocardia and were excluded [82 (20.7%) with situs inversus, 21 (5.3%) with situs solitus, and 15 (3.7%) with situs ambiguous]. Among the 277 patients with a right aortic arch, 7 (2.3%) had an aberrant left subclavian artery branch. There were 141 (50.9%) female patients. The trachea and esophagus were compressed. Respiratory symptoms were commonly present in patients with a compressive right arch and aberrant left subclavian artery (Kommerell’s diverticulum), which showed a significant relationship. Congenital major heart anomalies were found in 261 (94.2%) patients, with the most common being right obstructive lesions related to tetralogy of fallot (TOF), seen in 115 (41.5%) patients. Complex congenital heart disease was present in 75 (27.1%) patients. Septal defects were observed in 44 (15.9%) patients, including ventricular septal defect in 39 (14.0%). Nine (3.2%) patients had left obstructive lesions, including coarctation of the aorta. Arterial anomalies, such as patent ductus arteriosus, were present in 14 (5.0%) patients. Sixteen patients had normal heart structures or minor cardiac anomalies. The most common non-cardiac abnormality was musculoskeletal. Finally, 189 (68.2%) patients underwent surgery, and 16 (5.8%) died. Conclusions :More detailed examinations during patient visits, attention to clinical symptoms, and modern imaging can hasten the identification of cardiac abnormalities in children, which plays an important role in the diagnosis and prognosis of patients.

A previously healthy 52-year-old man had a chest radiograph for medical check-up and found to have a right-sided aortic arch. Computed tomography of the thorax revealed a right-sided aortic arch with aberrant left subclavian artery originated from Kommerell’s diverticulum. Barium swallow examination showed compression of the posterior wall of the esophagus. He was asymptomatic and no surgical intervention was performed.

The standard approach for repair of type A aortic dissection includes repair or replacement of the aortic valve, the ascending aorta and the arch, alone or in combination with direct vision under hypothermic circulatory arrest. Although type A dissection usually extends into the distal thoracoabdominal aorta, the descending thoracic aorta is usually left alone. During the follow-up, surgeons will sequentially and periodically evaluate the patients looking for aneurysmal dilatation of the untreated aorta for an eventual late open surgical or endovascular repair. Surgery of the aorta continues to be a surgical challenge. Aortic disease and surgery are dynamic as new ideas are continuously brought up in terms of surgical approach, extension of repair and characterization of patients. Technology also plays an important role nowadays. Since the early days of high-porosity vascular grafts, until today where vascular grafts are being coated with albumin or collagen, results have ramatically changed in terms of control of hemorrhage and related intraoperative death. The inception of endovascular therapy seems to change the perception of patients suffering from descending thoracic aortic aneurysms and chronic dissections.1 Acute type B dissections are still a matter of concern regardless of the attempted therapy. Type A dissection routinely involves the aortic arch. Operation-related morbidity and mortality is still high. Some questions are still unanswered; like the need of performing complex combined resections of the arch and how to treat the descending aorta. To save the patient first has always been our major surgical standard and we believe this must always be priority in the critical decision-making process. Here we will have a quick look at evolving concepts, ideas and technologies, asking some questions for the near future.

Right-sided aortic arch with aberrant left subclavian artery is a rare congenital anomaly of the aorta that occurs in less than 0. 1% of the population. Patients are asymptomatic in most cases, and the anomaly is found incidentally; however, symptoms can occur due to the compression of other structures, mostly the trachea and esophagus. In this report, we present a case of esophageal compression by a right-sided aortic arch with aberrant left subclavian artery that mimicked gastro-esophageal reflux in a 3-month-old (87-day-old) infant with complaint of regurgitation, vomiting, and failure to gain weight who was diagnosed through a barium meal study.

Three major arteries normally originate from the aortic arch. Variations of the aortic arch branches are observed in some cases. Rare variation of the aortic arch branches is originated from the retroesophageal right subclavian (RRS) artery as the last branch of the aortic arch. Variation of artery is possibly associated with the variation of the recurrent laryngeal nerve. This research provides an in vivo report of retroesophageal right subclavian artery during examination of the cervical vessels using magnetic resonance angiography (MRA). It is very important to consider the variation of the right subclavian artery during head and neck surgery. Non-invasive techniques such as MRA of cervical arteries can be used in order to examine them.

A 28- year- old woman was admitted because of uncontrolled hypertension following delivery. Aortography showed severe coarctation of the aortic arch, and a large- sized aneurysm that was located in the aortic arch distal to the left common carotid artery. The patient was scheduled for surgery. The coarctation was removed and the aortic arch was replaced with a 28-mm Dacron tube, and the left subclavian artery was reimplanted to the Dacron tube. The patient tolerated the procedure well and was discharged from hospital 15 days later.