Tracheal compression with "hairpin" right aortic arch: management by aortic division and aortopexy by right...

Four patients with severe tracheal obstruction due to right aortic arch, aberrant left subclavian artery, diverticulum of Kommerell, ligamentum or ductus arteriosum, and, additionally, right descending aorta, mild pectus excavatum, and high aortic arch apex, with narrow space between the ascending and descending aortic limbs, underwent division of ligamentum, excision of diverticulum and division (and reimplantation) of aberrant subclavian, either in multiple or single operations, but failed to achieve relief of obstruction.

In addition to the procedures noted, fabric sling aortopexy of ascending and descending aortic limbs around adjacent ribs, with or without aortic division after prosthetic graft between ascending and descending aortic limbs was required, all performed through a right thoracotomy and adjunctive cervical incision, and with flexible bronchoscopic monitoring of each step.

Three patients obtained full relief of airway obstruction, which has persisted in follow-up from eight to over 12 years. One who had persistent severe tracheal malacia after prior tracheal resection and resultant chronic pulmonary sepsis died from these complications.

In this unusual subset of a rare vascular ring anomaly, radical methods were necessary for correction of airway obstruction after failure of prior conventional procedures.