Interrupted aortic arch (type A1) with Eisenmenger syndrome
A young medically and surgically free female with recent progressive shortness of breath following childbirth. Pulmonary embolism was considered.
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The aortic arch is interrupted at the level distal to the normal looking left subclavian artery.
There are changes reflective of pulmonary arterial hypertension which include right ventricular hypertrophy, bowing of the interventricular septum towards the left, dilated main pulmonary trunk and branches resulting to increased segmental artery-to-bronchial diameter ratio and increased main pulmonary artery to ascending aorta ratio.
Interrupted aortic arch is a congenital cardiovascular anomaly wherein there is discontinuation at the level to the aortic arch. It is usually associated with shunt anomalies like PDA, VSD and ASD, which are demonstrated in this case. Depending on the location of the interruption, the anomaly may be divided into three types, each of which can be further divided into subtypes depending on the subclavian artery anatomy.
The current case represents type A1, the second most common of the three types, wherein the interruption occurs distal to the origin of the left subclavian artery (type A) with normal subclavian artery anatomy (subtype 1). The long-standing uncorrected shunt anomalies in this case resulted to severe pulmonary arterial hypertension with shunt reversal (Eisenmenger syndrome).
- 1. Shirani S, Soleymanzadeh M. Diagnosis of aortic interruption by CT angiography. Pol J Radiol. 2013;78 (1): 72-4. doi:10.12659/PJR.883771 - Free text at pubmed - Pubmed citation
- 2. Kimura-Hayama ET, MeléNdez G, MendizáBal AL et-al. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 2010;30 (1): 79-98. Radiographics (full text) - doi:10.1148/rg.301095061 - Pubmed citation
- 3. Reardon MJ, Hallman GL, Cooley DA. Interrupted aortic arch: brief review and summary of an eighteen-year experience. Tex Heart Inst J. 2005;11 (3): 250-9. Free text at pubmed - Pubmed citation