Right aortic arch analysis – Anatomical variant or serious vascular defect?

Abstract

BackgroundThe right-sided aortic arch (RAA) is a rare congenital defect of the aorta. The aim of the study was to assess the occurrence of RAA in diagnoses performed by the University Radiology Department and analyze the frequency of concomitant vascular abnormalities.MethodsThe database of the Radiology Department was retrospectively analyzed between January 2008 and May 2016 with the keyword “right aortic arch”. Twenty patients with this diagnosis were identified from a total of 11,690 CT examinations of the chest area, 19,623 CT examinations of brain-supplying vessels, and 1863 MRI examinations of the heart and aortic arch or brain-supplying arteries. The type of aortic arch, the occurrence of Kommerell’s diverticulum and possible other vascular abnormalities, such as stenosis, kinking or occlusion, were then investigated.ResultsThe analysis identified nine patients with type I and 11 patients with type II RAA. Eight of the 11 type II patients presented Kommerell’s diverticulum. Concomitant vascular abnormalities were detected in four patients with type II RAA. In two cases, the right common carotid artery (RCCA) was narrowed by up to 80%, with steal phenomenon confirmed in one of them. In the second coincident right subclavian artery (RSA) stenosis was depicted. In two other cases, the aberrant left subclavian arteries (ALSA) were found to be narrowed at the level of origin by up to 70%. One patient was found with type B aortic dissection including ALSA and Kommerell’s diverticulum.ConclusionsOur observations indicate that concomitant vascular abnormalities may occur more often than reported in literature. Patients diagnosed with type II RAA should be examined with Doppler ultrasonography to identify coincident vascular disorders, especially stenosis of the common carotid arteries or subclavian arteries.Electronic supplementary materialThe online version of this article (doi:10.1186/s12872-017-0536-z) contains supplementary material, which is available to authorized users.

Strategies for Accurate Diagnosis of Fetal Aortic Arch Anomalies: Benefits of Three-Dimensional Sonography With Spatiotemporal Image Correlation and a Novel Algorithm for Volume Analysis

“…16 Symptoms of compression and cystic medial necrosis of the KD wall may develop in the elderly, a time when aneurysmal diseases develop. 17 ARSA is another common abnormal aortic branching pattern. Rembouskos et al 7 indicated that ARSA was frequently related to trisomy 21 and associated with other CHDs.…”

Bicuspid aortic valve with right‐sided aortic arch aneurysm and Kommerell's diverticulum

“…In infancy, the symptoms are related to other congenital heart anomalies or to compression of the trachea or the esophagus. In adulthood, the symptoms are more often the result of dissection or aneurysmal dilatation of anomalous vessels accompanied by compression of surrounding structures …”

Kommerell Diverticulum: Right Aortic Arch with Anomalous Origin of Left Subclavian Artery and Duplicity of Right Vertebral Artery in a 16-Year-Old Girl

“…A possible etiology of the subclavian artery’s anomalous origin is an abnormality in regression of the fourth primitive aortic arch during embryonic development [3]. This condition was named Kommerell diverticulum in honor of Dr. Kommerell, the radiologist who first made the diagnosis [4].…”

“…This condition was named Kommerell diverticulum in honor of Dr. Kommerell, the radiologist who first made the diagnosis [4]. Anatomical changes are usually not associated with clinical symptoms, and the diagnosis of Kommerell diverticulum typically occurs incidentally during adulthood [3]. In some situations, especially when there is a right aortic arch and left subclavian artery, the diverticulum may be associated with dysphagia and dyspnea due to tracheal and/or esophageal compression, which directly influence the clinical and surgical treatment of this pathology.…”