Interrupted aortic arch

Last revised by Yahya Baba on 7 Nov 2020

Interrupted aortic arch (IAA) is an uncommon congenital cardiovascular anomaly where there is a separation between the ascending and descending aorta. It can either be complete or connected by a remnant fibrous band. An accompanying large ventricular septal defect (VSD) and/or patent ductus arteriosus (PDA) is frequently present.

It may account for ~1.5% of congenital cardiac anomalies. 

Faulty embryological development of the aortic arch (thought to occur during the 5th to 7th week of intrauterine life).

According to the Celoria-Patton classification, IAA can be classified into three types according to the location of the anomaly:

  • type A: second most common, the interruption occurs distal to the left subclavian arterial origin 
  • type B: most common (>50%), the break occurs between the left common carotid and left subclavian arterial origins
  • type C: rare, interruption occurs proximal to the left common carotid arterial origin

Each type is divided into three subtypes 7:

  • subtype 1: normal subclavian artery
  • subtype 2: aberrant subclavian artery
  • subtype 3: isolated subclavian artery that arises from the ductus arteriosus

Plain film features are often non-specific 3:

The right ventricle may appear a lot larger than the left, although this is a non-specific finding. The ascending aorta may also appear more vertical than usual. These modalities may not allow differentiation of IAA from severe aortic coarctation with a hypoplastic arch 9.

Allows visualization of the interrupted aortic arch and associated anomalies. 

  • non-visualization of the portion of interruption
  • great vessels may show a "V" configuration on coronal imaging 2

If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days. Prostaglandin E1 may be given to initial management to keep the ductus open. Surgical correction (either single- or multistage) is the definitive treatment.

General differential considerations include:

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Cases and figures

  • Figure 1: classification
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  • Case 1: type A1
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  • Case 2: type A1
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  • Case 3: type B1
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  • Case 3: 3D
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  • Case 4: type B2 with truncus arteriosus
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  • Case 5: type A1 with aortopulmonary window
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  • Case 6: type B (antenatal ultrasound)
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  • Case 7: type B
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  • Case 8
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