Few reports have described the implantation and management of an implantable ventricular assist device in patients with functional univentricular anatomy. We herein report a case of a patient who underwent Heartware ventricular assist device (Medtronic, Minneapolis, MN) implantation due to severe cardiac dysfunction and Fontan failure in the remote period after total cavopulmonary connection. Double valve replacement was subsequently required 1 year later due to repeated Fontan failure caused by the progression of aortic and atrioventricular regurgitation. The low-profile and saddle-shaped sewing cuff design of the MITRIS mitral valve (Edwards Lifesciences, Irvine, CA) were beneficial for avoiding the Heartware ventricular assist device inflow and atrioventricular valve interference in the less-dilated ventricle.
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