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Number 10-18: Aortic Coarctation Repair with Associated Bicuspid Aortic Valve
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Number 10-18: Aortic Coarctation Repair with Associated Bicuspid Aortic Valve

 

Case from:  Camastra GS, Cacciotti L, Sbarbati S, Danti M, Marconi F, Della Sala WS, Ansalone G
Institution: MG Vannini Hospital, Rome, Italy

Clinical history: A 30 year old male with native coarctation of the aorta was referred for breathlessness and uncontrolled hypertension.  He was noted to have elevated blood pressures in his bilateral arms and hypotension in his legs.  Echocardiography revealed a bicuspid aortic valve with dilated aortic root and a coarctation of the descending thoracic aorta with a pressure gradient of 60 mmHg.  The patient was referred for MRI to further define the anatomic location of the coarctation.  Following the scan the decision was made to proceed with percutaneuos intervention of the coarctation.

 

                               

Movie 1: Bicuspid aortic valve                          Movie 2: Dilated aortic root

 

CINE CMR:    Short axis and coronal SSFP were performed to demonstrate the bicuspid aortic valve (Movie 1) and proximal aorta (Movie 2).

 

 

                                            

Movie 3:  MRA Coarctation pre stent                            Movie 4:  MRA Coarctation post stent

 

3D Contrast enhanced MRA:  Coarctation of the descending thoracic aorta distal to the left subclavian artery is demonstrated (Movie 3).  Following stent placement there is signal loss within the lumen although minimal residual stenosis is present (Movie 4).  The enlarged still images below show the coarctation before (Figure 1A, 1C) and after stenting (Figure 1B, 1D).  Note minimal artifact created outside of the stent and reduction of signal enhancment within.

 

 

Figure 1:  Enlarged MRA Pre and post stenting of coarctation

 

Perspective:  Coarctation of the aorta is a relatively common defect that occurs in approximately 6-8 % of patients with congenital heart disease and is commonly associated with a bicuspid aortic valve.  Intravascular stents are finding increased applications in the treatment of patients with native coarctation of the aorta and percutaneous intervention is a reasonable alternative to surgical correction.  4D flow MR imaging has shown promise in the evaluation of both the clinical significance of the coarctation through the demonstration of collateral blood flow and the persistence of helical flow after coarctation repair.

An ascending aortopathy is associated with bicuspid aortic valves such that aortic aneurysmal disease may be present and continue to progress throughout the life of the individual.  The above mentioned patient will require lifelong surveillance of both his aortic root and coarctation.  Utilizing MRI for follow up will obviate the need for repetitive CT scans and lifelong radiation exposure.

Comment:  A nice example of 2D flow MR through an unrepaired coarctation can be seen in this 2006 SCMR Case of the Week.

References: 
1. Tan JL, Babu-Narayan SV, Henein MY, Mullen M, Li W. Doppler echocardiographic profile and indexes in the evaluation of aortic coarctation in patients before and after stenting. J Am Coll Cardiol. 2005 Sep 20;46(6):1045-53.
2. Lam YY, Kaya MG, Li W, Mahadevan VS, Khan AA, Henein MY, Mullen M. Effect of endovascular stenting of aortic coarctation on biventricular function in adults. Heart. 2007 Nov;93(11):1441-7.
3. Hope MD, Meadows AK, Hope TA, Ordovas KG, et al.  Clinical evaluation of aortic coarctation with 4D flow MR imaging.  J Magn Reson Imaging. 2010 Mar;31(3):711-8.
4. Puranik R, Tsang VT, Puranik S, Jones R, et al.  Late magnetic resonance surveillance of repaired coarctation of the aorta.  Eur J Cardiothorac Surg. 2009 Jul;36(1):91-5.

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COTW handling editor: Kevin Steel

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