Interrupted aortic arch (type A1) with Eisenmenger syndrome

Discussion:

Interrupted aortic arch is a congenital cardiovascular anomaly wherein there is discontinuation at the level to the aortic arch. It is usually associated with shunt anomalies like PDA, VSD and ASD, which are demonstrated in this case. Depending on the location of the interruption, the anomaly may be divided into three types, each of which can be further divided into subtypes depending on the subclavian artery anatomy.  

The current case represents type A1, the second most common of the three types, wherein the interruption occurs distal to the origin of the left subclavian artery (type A) with normal subclavian artery anatomy (subtype 1). The long-standing uncorrected shunt anomalies in this case resulted to severe pulmonary arterial hypertension with shunt reversal (Eisenmenger syndrome).

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