Abstract
Since the advent of the Fontan palliation, survival of patients with univentricular congenital heart disease has increased significantly. These patients will, however, ultimately develop heart failure requiring advanced therapies such as heart transplantation. As wait times are long, mechanical circulatory support (MCS) is an attractive therapy, both for bridge to transplantation and destination therapy in patients not suitable for transplantation. This review aims to summarize current thinking about how to determine which patients would benefit from a ventricular assist device (VAD), the optimal time for implantation and which device should be considered. VAD implantation in end-stage Fontan is still in its infancy; however, case reports and research interest have increased extensively in the past few years. Mortality is significantly higher than in noncongenital heart disease patients. Implantation in patients with primarily systolic dysfunction is indicated, whereas patients with increased transpulmonary gradient may not benefit from a single-VAD solution. When possible, implantation should occur prior to clinical decompensation with evidence of end-organ damage, as outcomes at this point are worse. Fontan patients demonstrating signs of heart failure should be evaluated early and often for feasibility and optimal timing of VAD implantation. The frequency of this procedure will likely increase significantly in the future.
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