Aortic Valve Perforation Inducing Severe Aortic Valve Regurgitation in a Patient With Takayasu Arteritis

A 23-year-old male attended hospital because of weight loss, fever and chronic cough. Computed tomography (CT) revealed wall thickening of the ostium of the innominate and left common carotid and subclavian arteries (Figure A). He was diagnosed with Takayasu arteritis (TA), and steroid therapy was started. CT performed 6 years later revealed improved artery wall thickening (Figure B), and blood tests revealed well-controlled inflammation (C-reactive protein (CRP) 0.00 mg/dL) under steroid and tocilizumab therapy. However, cardiac dilatation was newly detected on chest X-ray. Transthoracic echocardiography revealed left ventricular dilatation and new findings of severe aortic valve (AV) regurgitation with Valsalva dilatation (Figure C). Transesophageal echocardiography revealed perforation of the left AV leaflet (Figure D), with an eccentric regurgitant jet hitting the interventricular septum on longitudinal view (Figure E); this was visible as a counterclockwise jet on the short-axial view (Figure F,G).

Figure.

Computed tomography revealed wall thickening of the ostium of the innominate and left common carotid and subclavian arteries (A) and improved artery wall thickening under steroid therapy. (B) Transthoracic echocardiography revealed severe aortic valve regurgitation. (C) Transesophageal echocardiography revealed perforation of the left aortic valve (AV) leaflet (white arrows) (D,F) and an eccentric regurgitant jet (yellow arrow). (E,G) Valve-sparing aortic root replacement with AV plasty using an autologous pericardial patch was performed to close the perforation (H,I). LCC, left coronary cusp; NCC, noncoronary cusp; RCC, right coronary cusp.

Valve-sparing aortic root replacement with AV plasty using an autologous pericardial patch was performed to close the perforation (Figure H,I). After surgery, inflammation remained well-controlled (CRP 0.01 mg/dL) under medical therapy.

A literature review located a case report of a 15-year-old female with TA complicated by aortic dilatation and acute fatal aortic regurgitation due to AV perforation, which was confirmed by autopsy.¹ Therefore, the present case is the only report of a TA patient with successful, surgically treated AV perforation.

Acknowledgement

We thank Libby Cone, MD, MA, from DMC Corp. (www.dm ed.co.jp) for editing a draft of this manuscript.

Disclosure

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

Reference

• 1.   Satoh T, Chino M, Takahashi M, Suzuki K. Aortitis syndrome with fatal acute aortic regurgitation due to aortic dilatation and aortic valve perforation: A case report. Angiology 1992; 43: 869–872.