Abstract
BACKGROUND: Transcatheter aortic valve replacement (TAVR) has become a standard therapy for severe aortic stenosis, yet bicuspid aortic valve (BAV) patients remain challenging, especially with complex aortic root. CASE SUMMARY: A 72-year-old man with type 0 BAV and sinus of Valsalva aneurysm underwent transapical J-Valve implantation for severe stenosis. Post-procedural incremental paravalvular leak (PVL) due to aneurysm-related malapposition was successfully treated with a self-expanding valve-in-valve (ViV) prosthesis (TaurusElite, 29 mm). Multimodal imaging, including echocardiography, computed tomography angiography, and 3-dimensional-printed modeling, ensured precise sealing with excellent hemodynamic results. DISCUSSION: We report the first case of self-expanding ViV for PVL after J-Valve TAVR in type 0 BAV with aortic root aneurysm. This case underscores the feasibility of self-expanding ViV in complex BAV anatomy with concomitant aortic root pathology sighting into optimal device selection and imaging integration. TAKE-HOME MESSAGES: Self-expanding prostheses are preferred for BAV with concomitant aortic root pathology due to adaptive sealing capabilities. Early ViV TAVR mitigates PVL-driven ventricular remodeling.