Mechanical circulatory support for high-risk surgical aortic valve and ascending aortic replacement in severe bicuspid aortic valve stenosis: a case series

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect (reported incidence of 0.5%-2%) and is commonly associated with proximal aortic dilation. Patients with severe aortic stenosis (AS) of BAV have been shown to have worse pre-operative left ventricular (LV) function as well as a higher incidence of post-operative heart failure hospitalization when compared with analogous patients with tri-leaflet aortic valve disease. While surgical aortic valve replacement (SAVR) may be favoured over transcatheter aortic valve implantation (TAVI) due to anatomical factors or concomitant aortopathy and coronary artery disease, surgical candidacy is often limited by prohibitive operative risk.

Case summary: We report on three cases of severe AS in BAV with concomitant aortopathy and severe left ventricular dysfunction in whom we proceeded with SAVR with a priori planned venoarterial extracorporeal membrane oxygenation (VA-ECMO) support and inotrope-assisted wean. All patients had severe LV dysfunction (ejection fraction < 25%) at baseline with gradual substantial improvement or normalization after successful SAVR.

Discussion: These cases demonstrate the utility of planned VA-ECMO with SAVR and aortic root replacement as an integral component of the operative strategy for high surgical risk patients with severe BAV AS not amenable to TAVI. Appropriate pre-operative planning and consent for VA-ECMO as well as a multi-disciplinary approach involving anaesthesia, intensive care, and heart failure cardiology are the key to offering this option as an alternative to palliative medical therapy to a selected group of patients.