Welcome toMorning Report

February 24, 2011

Vascular Ring

• Congenital anomalies of the aortic arch– Compression of tracheobronchial tree– Compression of the esophagus (+/-)– Respiratory and gastrointestinal symptoms

• Only 1 to 3% of congenital heart disease

Clinical Manifestations

• Variable:– May be life threatening in infants– Found incidentally in adults

• Complete ring most symptomatic– Stridor most frequent– Recurrent resp infections– Distress– Wheezing, cough– Esophageal: dysphagia, feeding difficulties, vomiting

Classification• Complete– Double aortic arch– Right aortic arch with aberrant L subclavian• Left ligamentum arteriosum

Double Aortic Arch

Classification• Incomplete rings– Pulmonary artery sling– Innominate artery compression– Aberrant right subclavian

Most important clue?

Pulmonary Sling

• Concern for additional tracheal problems– Complete cartilaginous rings– Requires tracheoplasty

Vascular Rings: Associations

• Associated anomalies in 50%– Mostly cardiac lesions– TE fistula– Cleft lip-palate– Syndromes• DiGeorge• Down• CHARGE

Management and Outcome

• All complete rings require surgery• Outcome very favorable• Up to 10% may take 6months or more for

resolution of symptoms