Aortopulmonary window, interrupted aortic arch and large PDA giving the descending aorta

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Breathlessness.

Patient Data

Age: 8 years
  • A relatively large aortopulmonary window is seen connecting the ascending aorta (about 1.5 cm distal to the aortic annulus) & the main pulmonary artery.  The aorta is seen arising from the left ventricle and the ascending aorta ends by giving three orderly branches (the left subclavian, the left common carotid and brachiocephalic arteries).
  • Interrupted aortic arch is seen with a large PDA arising from the anterosuperior aspect of the distal main pulmonary artery and continues as the descending aorta in the left paravetebral gutter.  No evident abdominal coarctation.
  • Antegrade continuity between the right ventricle and the main pulmonary artery. Confluent dilated main pulmonary artery and its branches.

  • Situs solitus, with the cardiac apex to the left.
  • Mild cardiomegaly is noted with mild left ventricular dilatation. 
  • Atrio-ventricular concordance and ventriculo-atrial concordance.
  • Intact interatrial septum (IAS) & interventricular septum (IVS).

  • Normal origin and distribution of the coronaries.
  • Increased pulmonary vascularity. No evidence of anomalous pulmonary venous drainage.

Aortopulmonary window, interrupted aortic arch & large PDA giving the descending aorta..

Case Discussion

Aortopulmonary window, (partial truncus arteriosus or aortopulmonary septal defect) is a rare congenital conotruncal cardiac anomaly with communication between the ascending aorta & the main or right pulmonary artery. It is caused by incomplete division of the embryonic common trunk. The two semilunar valves are usually separately identified and the absence of VSD differentiates it from truncus arteriosus. Association with PDA in 15%. Aortopulmonary window is associated with VSD or aortic coarctation.

In this case, interrupted aortic arch is seen with a large PDA is giving the descending aorta.

The Case is courtesy of Dr. Mohammad A. ElBeialy, MD, FRCR & Dr. Heba Kamal.

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Case Information

rID: 35573
Diagnostic certainty: Almost Certain
Case created: 12th Apr 2015
Last edited: 16th Dec 2015
System: Cardiac
Inclusion in quiz mode: Included

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