Right Aortic Arch in Pediatric: Experience of a Center

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.