INTRODUCTION

Cardiac surgery in the patient with sickle cell disease (HbSS) presents several challenges, as many of the techniques used to safely perform cardiopulmonary bypass and to ensure myocardial protection may precipitate sickling and the complications thereof. Several strategies exist to prevent sickling during cardiac surgery requiring cardiopulmonary bypass. A case of a patient with HbSS requiring mitral valve repair is presented, along with a discussion of the approach to this case.