The aortic uncrossing procedure.

OBJECTIVE: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure. METHODS: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy. RESULTS: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia. CONCLUSIONS: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.

A left circumflex aorta with a displaced thoracic duct in a 94-year-old male cadaver: a case report with discussion on embryology.

A left circumflex aorta (LCA) is an extremely rare variation of the thoracic aorta. It is distinguished by a retroesophageal descending aorta that subsequently travels down the right side of the thoracic vertebrae towards the aortic hiatus. Nonetheless, its embryological origin ought not to be overly generalised, but each case should be considered individually due to its unique vascular patterns. This study presents a description of a LCA in a 94-year-old male cadaver. The dissection revealed the descending aorta posteriorly from the trachea and oesophagus and then laterally on the right from the thoracic vertebral bodies. The branching pattern of the aortic arch was typical, so was the course of the left and right recurrent laryngeal nerves. However, the thoracic duct was placed on the right, and drained into the right internal carotid vein. Due to the normal appearance of the ascending part and the arch of the aorta, it is safe to presume that the variation originated from the persistent right dorsal aorta, with the retroesophageal part from the persistent left dorsal aorta. Detailed understanding of the variations of the thoracic aorta, and the anomalies associated with the LCA, can help to improve management of these conditions, and with that, improve patients' overall outcomes. Patients with a LCA, or another vascular ring, can either be asymptomatic or present with oesophageal and/or tracheal compression symptoms. Management of this anomaly consists namely of ligation of the patent ductus arteriosus/ligamentum arteriosum and aortic uncrossing.

Concurrent double aortic arch and circumflex aorta repair in a symptomatic child: a case report.

Double aortic arch with circumflex aorta is a rare type of vascular ring. Symptoms result from external compression of the trachea and esophagus. The best surgical approach for patients with double arch and circumflex aorta is debated, and options include the highly complex aortic uncrossing procedure. Herein we describe the surgical treatment of a patient with concurrent double aortic arch and circumflex aorta by division of the non-dominant arch and ligamentum arteriosum, plication and posterior tacking of the diverticulum of Kommerell. This left thoracotomy approach provided complete symptom resolution.

Left cervical and circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum: A rare vascular ring.

We report a rare case of vascular ring due to a left cervical circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum. This is a very rare case of vascular ring in a newborn with stridor and stenosis of the left pulmonary artery. A cardiac catheterization and an angio-computed tomography scan were helpful to clarify the diagnosis. The surgical correction was performed with division of the arterial duct, reimplantation of the right subclavian artery, aortic and tracheal suspension, and pulmonary arterial plasty. The patient was asymptomatic at discharge and 1-year follow-up.

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.

Aortic uncrossing with concomitant arch augmentation in a rare case of left circumflex aorta with arch hypoplasia.

Retro-esophageal circumflex aortic arch is a rare form of vascular ring. Majority of cases occur in isolation without any intracardiac lesions. Only a few cases of left circumflex aorta have been reported in the literature. We present a case of left circumflex aorta with associated arch hypoplasia and coarctation, ventricular septal defect, who underwent single-stage repair.

Aortic uncrossing and tracheobronchopexy corrects tracheal compression and tracheobronchomalacia associated with circumflex aortic arch.

OBJECTIVE: Aortic uncrossing is an effective procedure for relieving the external airway compression from a circumflex aortic arch by transferring the aortic arch to the same side as the descending aorta. However, patients frequently have residual tracheobronchomalacia (TBM), which may result in persistent postoperative symptoms. We review a series of patients who underwent an aortic uncrossing and concomitant tracheobronchopexy to correct the airway compression and residual TBM. METHODS: Retrospective review of all patients who underwent aortic uncrossing and concomitant tracheobronchopexy at a single institution between September 2016 and March 2019. Preoperative evaluation included computed tomography angiography and rigid 3-phase dynamic bronchoscopy. RESULTS: Eight patients who ranged in age from 4 months to 15 years with significant respiratory symptoms underwent an aortic uncrossing procedure with concomitant tracheobronchial procedures. Mild hypothermic cardiopulmonary bypass (mean time, 105.6 ± 39.4 min) and regional perfusion (mean time, 44 ± 10 min) were used without circulatory arrest. Intraoperative bronchoscopy demonstrated no patients had residual TBM. There were no postoperative mortalities, neurologic complications, chylothoraces, coarctations, or obstructed aortic arches. Two patients required tracheostomy and gastrostomy for bilateral recurrent laryngeal nerve paresis (patients 2 and 3). One patient with bronchial stenosis after concomitant slide bronchoplasty required stenting. At a median follow-up of 22 months (range, 5-34 months), all patients were alive without evidence of significant respiratory symptoms. CONCLUSIONS: The aortic uncrossing procedure can be performed safely in pediatric patients of all ages without circulatory arrest. Concomitant procedures addressing associated TBM can significantly improve respiratory symptoms.

Left aortic arch with right descending aorta and severe coarctation: an unusual "vascular clamp" with airway compression.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

Circumflex aorta with double aortic arch in an infant.

Circumflex aorta and double aortic arch are two forms of rare vascular rings. We present a case of an infant who was diagnosed with circumflex aorta and double aortic arch, and describe the surgical management of this rare anomaly.