Circumflex aorta: An uncharted territory

Background Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons. Methods A comprehensive search of peer reviewed journals was completed based on the key words, “Circumflex aorta,” “Circumflex retro-esophageal aorta” and “circumflex arch” using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies. Results Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported. Conclusion Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.

Use of 3D printing for parent and patient education in repair of vascular ring and circumflex aorta

A clear understanding by the patient and family of airway pathology caused by vascular rings can be difficult to achieve. The pathology is three-dimensional in nature, and is a compilation of differing anatomic structures. Patient confusion can lead to misunderstandings regarding the nature of the operation, attendant risks, and the expected post-operative course. In this review we describe our use of 3D printing in the setting of circumflex aorta and double aortic arch to help guide a child and their family through the decision for treatment of what can be a difficult problem in both diagnosis and management. Our patient underwent two operations. First, a thoracoscopic division of a left ligamentum and atretic distal left aortic arch. While symptoms improved somewhat post-operatively, moderate dysphagia and dyspnea persisted. Based on further evaluation we concluded that the circumflex aorta was the etiology of the ongoing symptoms, so an aortic uncrossing procedure under circulatory arrest was recommended and performed. We used a 3D model in the education of the patient and family prior to the second surgery and greatly enhanced their understanding of why a repeat operation was required to address an additional component of this complex vascular ring. The use of 3D printing in preoperative discussions facilitated better understanding of complex three dimensional anatomy for the patient and patient’s family. In addition, this communication strategy helped frame expectations regarding the post-operative course and convalescence.

Modified Aortic Uncrossing Procedure: A Novel Approach for Norwood Palliation of Complex Univentricular Congenital Heart Disease With a Circumflex Aorta

The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The “aortic uncrossing procedure” described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual. We report such a case and describe our approach to its surgical management.