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The interrupted aortic arch is very rare and occurs in about 1% of the infants with critical congenital heart disease. Perfusion distal to the interruption is dependent on the patency of the ductus arteriosus. Unrecognized and untreated cases have a median age of death at 4–10 days of life. Management of the interruption requires timely diagnosis, use of prostaglandin E infusion immediately after birth to ensure end-organ perfusion, and eventual surgical repair of the interruption. Very rarely there have been cases of patients surviving longer with significant collaterals supplying the lower half of the body. High-quality echocardiography can reveal types of interrupted aortic arch and associated cardiac lesions. Cardiac CT angiogram has played a very important role in delineating details of the interrupted aortic arch, absence/presence of collaterals, intersegmental distance to plan appropriate surgical approaches, and follow-up study to evaluate recurrent coarctation postoperatively.

The coarctation of the aorta occurs in 7% of congenital heart defect cases with male predominance. High-quality echocardiography can reveal much information in most patients; however, it can be difficult in older/larger children. Cardiac CT angiogram is very important in delineating the extent of the aortic arch obstruction and any presence of collaterals. Prospective ECG-gated cardiac CT angiography using dual-source multidetector CT scanner has high spatial resolution and short acquisition time. Management of the obstruction depends on the severity and associated defects, but surgical repair is the most time-tested management particularly for the pediatric population.