Interrupted aortic arch (type A1)

Case contributed by Dr Jacqueline Austine Uy


This patient from the Marshall Islands was diagnosed as a child to have a congenital heart disease, although no intervention was supposedly done. She presented with dyspnea on extertion. 2-D echogardiography showed a ventricular septal defect, severe mitral regurgitation and mild to moderate pulmonary regurgitation. She was referred to a tertiary medical center for further evaluation.

Patient Data

Age: 11
Gender: Female

There is discontinuity of the aortic arch distal to the take-off of the left subclavian artery.  The descending aorta emanates from the main pulmonary artery via a patent ductus arteriosus.  The innominate including the right subclavian and common carotid arteries, left common carotid and left subclavian arteries branch normally without evidence of stenosis or aberrant configuration.  The included intercostal and internal thoracic vessels are prominent with multiple collateral.

The main, left and right pulmonary arteries are dilated without thrombus formation.  The pulmonary arteries are engorged relative to their accompanying bronchi, which likely relate to some degree of arterial hypertension.

The heart is enlarged, with multichamber dilatation.  A small membranous ventricular septal defect can be identified, although the interatrial septum is intact.

Case Discussion

CT scanning in the evaluation of interrupted aortic arch is essential during pre-surgical planning, detection of associated cardiac anomalies, determination of the size of the patent ductus arteriosus, and presence of stenosis.  CT scan also detects the presence of vascular collaterals such as in this patient, which allows for some individuals to survive to adulthood without surgical intervention.  90% of infants with Interrupted aortic aches usually die within the first year of life from circulatory failure.

An Interrupted aortic arch type A  (Celoria-Patton classification) is the second most common type (approximately 42% of all IAA cases) next to type B.   This type A arch occurs when the interruption occurs beyond the left subclavian artery.  The subclavian arteries of this patient are normal in configuration, without aberrant origin hence this may be further subdivided to sub-type 1 (normal subclavian artery).  The specific types and sub-types are further discussed in the main article regarding Interrupted aortic arches.

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Case information

rID: 40375
Published: 19th Oct 2015
Last edited: 14th Aug 2019
System: Vascular, Cardiac
Inclusion in quiz mode: Included

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