Abstract
It has been more than 4 decades since the development of Fontan operation. With the contemporary surgical strategies to treat the patients with a single ventricle physiology, the medium-term survival of the patients following Fontan operation is excellent. Nonetheless, the Fontan circulation with a pumpless pulmonary circulation has fundamental physiologic limitations, which are associated with late Fontan failure and deaths. There has been an exponential growth of adolescence and adults living with a Fontan circulation, which poses significant challenges in future. In this review, we discuss challenges and potential opportunities to treat the failing Fontan circulation. The specific topics include medical therapy, imaging, and therapeutic interventions for lymphatic abnormalities, transplantation, and mechanical support. The article also summarizes quality of life among the patients with the Fontan circulation. Surgical techniques, developing novel diagnostic and therapeutic tools, and increasing our understanding of the failing Fontan physiology is essential to improve the overall long-term outcome of this entity.
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