Interrupted aortic arch (type A1) with Eisenmenger syndrome

Case contributed by Dr Abdallah Al Khateeb


Previously well patient, presenting with recent progressive shortness of breath following childbirth. Pulmonary embolism was considered.

Patient Data

Age: 25 years
Gender: Female

The aortic arch is interrupted at the level distal to the normal left subclavian artery.

The descending aorta is supplied by the left main pulmonary artery through the patent ductus arteriosus (PDA).

Large ventricular and atrial septal defects (VSD and ASD) are demonstrated.

There are changes reflective of pulmonary arterial hypertension which include right ventricular hypertrophy, bowing of the interventricular septum towards the left, dilated main pulmonary trunk and branches resulting to increased segmental artery-to-bronchial diameter ratio and increased main pulmonary artery to ascending aorta ratio.

Case 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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