Surgical replacement of a small, calcified intra-atrial Fontan conduit with an adult-sized extracardiac conduit in an adult with hypoplastic left heart syndrome safely normalized liver enzymes and delayed the need for heart/liver transplantation at 1-year follow-up.
May support Fontan conversion for conduit-related liver issues in select patients; leaves open need for prospective validation.
A 38-year-old man with a functional single ventricle secondary to hypoplastic left heart syndrome presented with exertional fatigue. His last palliation was an intra-atrial conduit Fontan procedure. Comprehensive evaluation showed elevated liver enzyme values and a small, calcified conduit. Successful conversion to a nonfenestrated extracardiac conduit Fontan was performed with normalization of his liver enzyme values.
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Said et al. (2024) studied this question.
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