Abstract

Sinus of Valsalva aneurysms (SVAs) are rare congenital entities arising from eccentric aortic root dilatation that can protrude and rupture into adjacent cardiac chambers. Treatment entails aneurysmal sac excision and aortic defect closure. We present a young patient with a ruptured SVA fistulizing into the right ventricle and acute decompensated heart failure. He also had moderate aortic root enlargement and a dysmorphic aortic valve with 3 highly asymmetrical leaflets. This pathologic condition was successfully repaired with a novel combination of valve-sparing root replacement, aortic valve leaflet neochordal repair, right ventricular reconstruction, and tricuspid valve annuloplasty.

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