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Welcome to Morning 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.

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  1. Welcome toMorning Report February 24, 2011

  2. 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

  3. 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

  4. Classification • Complete • Double aortic arch • Right aortic arch with aberrant L subclavian • Left ligamentumarteriosum

  5. Double Aortic Arch

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

  7. Most important clue?

  8. Pulmonary Sling • Concern for additional tracheal problems • Complete cartilaginous rings • Requires tracheoplasty

  9. Vascular Rings: Associations • Associated anomalies in 50% • Mostly cardiac lesions • TE fistula • Cleft lip-palate • Syndromes • DiGeorge • Down • CHARGE

  10. Management and Outcome • All complete rings require surgery • Outcome very favorable • Up to 10% may take 6months or more for resolution of symptoms

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