Interrupted aortic arch (type B1) with hiatus hernia


Interrupted aortic arch (IAA) is defined as a complete discontinuity of the aortic lumen between the ascending and descending portions of the aorta. It is a rare congenital malformation accounting for only 1% of congenital heart diseases. It usually presents with severe congestive heart failure in infants.

IAA usually occurs in association with other cardiac malformations, more commonly ventricular septal defect (VSD) and patent ductus arteriosus, both of which are seen in this case. Other associated anomalies include subaortic stenosis, truncus arteriosus, aortopulmonary window, double outlet right ventricle, and DiGeorge syndrome.

IAA is classified according to the site of interruption:

  • Type A: distal to the subclavian artery (2nd most common)
  • Type B: between the second carotid and the ipsilateral subclavian (most common)
  • Type C: between the two carotid arteries

Further subdivision according to the origin of the right subclavian artery:

  • Subtype 1: normal subclavian artery
  • Subtype 2: aberrant subclavian artery (distal to the left subclavian artery)
  • Subtype 3: isolated subclavian artery that arises from the ductus arteriosus