Almost Three Decades Conservative Follow-up of Pseudoaneurysm of the Mitral-Aortic Intervalvular Fibrosa Without Radical Surgery

A 76-year-old woman with Behçet’s disease was admitted because of heart failure. She underwent aortic valve replacement because of aortic valve regurgitation with a pseudoaneurysm arising from the mitral-aortic intervalvular fibrosa (p-MAIVF), which had been detected on magnetic resonance imaging (Figure A,B) at the age of 45 years. Radical surgery for the p-MAIVF was not performed at the same time because of vulnerable tissue, and it was confirmed on postoperative computed tomography (Figure C,D).

Figure.

Magnetic resonance imaging (A,B) and computed tomography (C,D) showing the pseudoaneurysm of the mitral-aortic intervalvular fibrosa (p-MAIVF) (white arrows). (E) Transthoracic echocardiography showing the shunt flows from the left ventricular outflow tract to the p-MAIVF (yellow arrow) and from the p-MAIVF to the left atrium (pink arrow). Newly suspected mitral valve regurgitation (white arrow). (F) Transesophageal echocardiography showing new shunt flow from the p-MAIVF (white arrow) to the left atrium (yellow arrow).

Severe mitral valve regurgitation was newly suspected on transthoracic echocardiography (Figure E), but was diagnosed as a new shunt flow from the p-MAIVF to the left atrium by transesophageal echocardiography (Figure F, Supplementary Movie).

Mitral-aortic intervalvular fibrosa is a rare, life-threatening abnormality, and a total of 161 cases of p-MAIVF were reported in 104 articles on PubMed between 1960 and March 2014.¹ It is mainly caused by infective endocarditis and complications after cardiac surgery, and there are few previous reports of a patient with both Behçet’s disease and p-MAIVF.¹ Fragile cardiovascular tissue due to Behçet’s disease might have induced p-MAIVF in this case.² As far as we know, this is the longest conservative follow-up of p-MAIVF without radical surgery.

Disclosure

Y.K. is a member of Circulation Journal’s Editorial Team.

Funding

This work was partially supported by the TSUCHIYA MEMORIAL MEDICAL FOUNDATION (grant no. J17KF00167).

Supplementary Files

Supplementary Movie. Transesophageal echocardiography showing new shunt flow from the pseudoaneurysm of the mitral-aortic intervalvular fibrosa (p-MAIVF) to the left atrium near the mitral valve.

Please find supplementary file(s);

http://dx.doi.org/10.1253/circj.CJ-21-0507

References

• 1.   Şahan E, Gül M, Şahan S, Sokmen E, Guray YA, Tufekçioglu O. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa: A new comprehensive review. Herz 2015; 40: 182–189.
• 2.   Ogino H. Surgical strategy for refractory aortitis. Gen Thorac Cardiovasc Surg 2019; 67: 25–31.