interrupted aortic arch (IAA)

Newborn with
an abnormal fetal echo. Axial CT with contrast of the heart (above), obtained in a venous phase, shows discontinuity of the ascending and descending aorta while the sagittal image (below right) shows a dilated patent ductus arteriosus reconstituting the descending aorta and the coronal image (below left) shows dextrocardia.The diagnosis was interrupted aortic arch supplied by a patent ductus arteriosus in a patient with dextrocardia.
pediatricimaging.org • CC-by-nc-sa 4.0
Primary
repair of transposition of the great arteries with an interrupted aortic arch: a case report and literature review. Preoperative three dimensional reconstruction images which showed significant mismatch of the great arteries
cardiothoracicsurgery.biomedcentral.com • CC-by-4.0
Interrupted
aortic arch • Aortopulmonary window, interrupted aortic arch and large PDA giving the descending aorta - Ganzer Fall bei Radiopaedia
ElBeialy, M. Aortopulmonary window, interrupted aortic arch and large PDA giving the descending aorta. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-35573 • CC by-nc-sa 3.0 (modified)
Interrupted
aortic arch • Interrupted aortic arch (type B1) with hiatus hernia - Ganzer Fall bei Radiopaedia
Lim, T. Interrupted aortic arch (type B1) with hiatus hernia. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-40059 • CC by-nc-sa 3.0 (modified)
Interrupted
aortic arch • Celoria-Patton classification of interrupted aortic arch (illustration) - Ganzer Fall bei Radiopaedia
Tatco, V. Celoria-Patton classification of interrupted aortic arch (illustration). Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-51881 • CC by-nc-sa 3.0 (modified)
Interrupted
aortic arch type A subtype 1. 3D volume rendering CT aortic angiography image showing a stent inserted at the level of the interrupted aortic arch.
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Trans-femoral cardiac catheter reveals non-passage of the catheter proximal to the level of descending thoracic aorta which is opacified with non-opacification of the aorta proximally.
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. 3D VR images. White arrow refers to the IAA seen distal to the left SCA (blue arrow) (type A). Yellow arrows refer to the anterior anastomotic channels with dilated internal mammary arteries (red arrow)
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. 3D VR images. White arrow refers to the IAA seen distal to the left SCA (blue arrow) (type A). Yellow arrows refer to the anterior anastomotic channels with dilated internal mammary arteries (red arrow).
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Sagittal CT aortography reformatted images revealed posterior anastomotic channels along the posterior intercostal arteries.
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Sagittal CT aortography reformatted images revealed anterior anastomotic channels between the superior and inferior epigastric artery connecting the IMA proximally to the CFA distally. Notice the abnormal dilatation of the IMA (arrowed).
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Coronal CT aortography reformatted images revealed anterior anastomotic channels between the superior and inferior epigastric artery connecting the IMA proximally to the CFA distally. Notice the abnormal dilatation of the IMA (arrowed).
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Sagittal CT aortography reformatted images revealed interrupted aortic arch at the level between the arch of the aorta and the descending aorta distal to the left SCA with a complete fibrous band (arrowed).
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Coronal CT aortography reformatted images revealed interrupted aortic arch at the level between the arch of the aorta and the descending aorta distal to the left SCA with a complete fibrous band (arrowed).
www.eurorad.org • CC by-nc-sa-4.0
Interrupted
aortic arch type A subtype 1. Trans-axillary cardiac catheterization reveals non-passage of the catheter distal to the level of the junction between the arch and descending aorta with non-opacified descending thoracic aorta.
www.eurorad.org • CC by-nc-sa-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

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.

Epidemiology

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

Pathology

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

Classification

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 :

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

Radiographic features

Plain radiograph

Plain film features are often non-specific:

Antenatal ultrasound/Echocardiography

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 .

CT

Allows visualization of the interrupted aortic arch and associated anomalies.

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

Treatment and prognosis

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.

Differential diagnosis

General differential considerations include:

Siehe auch:
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Assoziationen und Differentialdiagnosen zu interrupted aortic arch: