Bailout transcatheter aortic valve implantation for a bicuspid aortic valve with severe mixed aortic valve disease presenting in cardiogenic shock

A 32-year-old male with severe aortic stenosis (AS) and severe aortic regurgitation (AR) due to a partial-fusion bicuspid aortic valve with an aortic annulus area of 917mm2 was admitted with cardiogenic shock. Transthoracic echocardiography (TTE) demonstrated aortic valve mean gradient (MG) of 73mmHg, peak gradient (PG) 108mmHg, aortic valve area (AVA) 0.4cm2 and severe eccentric AR. Left ventricular ejection fraction (LVEF) was 16%. Following Heart Team review, the patient was deemed unsuitable for surgical aortic valve replacement (SAVR), upfront left-ventricular assist device (LVAD) or extracorporeal membrane oxygenation (ECMO). He therefore underwent transcatheter aortic valve implantation (TAVI). A 29mm Edward SAPIEN-3 valve was deployed with balloon dilatation at nominal volume plus 6mL. Anticipated inferior valve shortening did not occur and the implantation was relatively deep as the valve was gripped within the leaflets, resulting in moderate AR through the struts. There was restriction of the superior aspect of valve; thus, post-dilatation was performed at nominal volume plus 8mL, resulting in balloon rupture. Valve migration was noted due to the lemon seeding effect from the restricted superior aspect of the stent frame; thus, a second 29mm S3 valve was implanted and dilated to nominal volume plus 7mL. This resulted in a stable valve position with improvement in AR. The final LVEDP was 16mmHg with an aortic diastolic pressure of 95mmHg. ECMO was not required. TTE at two-months demonstrated mild paravalvular leak (PVL) with LVEF of 56%. We demonstrate a successful off-label TAVI in a critically unwell patient with aortic annulus area >900mm2.