Interrupted aortic arch (type B1) with hiatus hernia

Case contributed by Dr Timothy Reynold Lim

Presentation

Difficulty of breathing with desaturations and murmur. Facial dysmorphisms were also noted.

Patient Data

Age: Newborn
Gender: Male

Minimal ground glass haziness are seen in both lower lungs. Cardiomegaly is also noted. There is an air-filled structure in the retrocardiac region.

Upon administration of oral contrast, radiographs show a displaced gastroesophageal junction located above the diaphragm. The cardia, fundus, and proximal body of the stomach also protrude superiorly into the thorax through the esophageal hiatus, with the middle body constricted at the diaphragmatic opening. The distal body, antrum, and pylorus are intra-abdominal in location.

  • Interrupted aortic arch, type B1
    • interruption between the left common carotid and left subclavian artery
    • normal right subclavian artery
    • left subclavian artery emanates from the proximal descending aorta which arises from the main pulmonary artery via a patent ductus arteriosus
  • Patent ductus arteriosus
  • Atrial septal and membranous ventricular septal defects
  • Slight mesocardia with mild right atrial and right ventricular dilatation, and larger main pulmonary trunk than the aorta
  • Normal atrio-ventricular and ventriculo-arterial concordance
  • Normal left atrium and ventricle
  • Sliding type hiatal hernia
  • Diffuse ground-glass densities in both lungs

Case Discussion

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
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Case information

rID: 40059
Case created: 5th Oct 2015
Last edited: 7th Oct 2015
System: Paediatrics
Inclusion in quiz mode: Excluded

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