A review of vascular rings 1980-1992

Over a period of 13 years 21 patients between the ages of three weeks and 13 years presenting to this centre had a vascular ring. A double aortic arch was present in 11 cases, a right aortic arch with a retroesophageal left subclavian artery in five cases, a right aortic arch with a normal left innominate artery, a Kommerell's diverticulum and a left ligamentum arteriosum encircling the trachea and oesophagus occurred in two cases and an aberrant left pulmonary artery arising from the right pulmonary artery occurred in three cases. The majority (19/21) presented with a history of chronic stridor, although other significant associated symptoms included: recurrent respiratory infection, wheeze, cyanotic episodes and difficulty or pain on swallowing. The barium swallow was the most useful method of investigation and was diagnostic in all of the 95% of cases in which it was performed. Nineteen patients underwent surgery uneventfully although stridor often remained a problem for some time following repair. Six patients (28%) had associated congenital heart disease. Three (14%) patients who did not have surgery died.