Key result
Fontan operation increases single ventricle survival but carries inevitable long-term morbidities requiring specific management.
This review highlights the long-term morbidities associated with the Fontan circulation and proposes contemporary management strategies for patients with single ventricle physiology.
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Supports specialized Fontan follow-up; leaves open optimal strategies pending prospective trials.
Since its original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology and has resulted in an increasing number of these patients surviving to adulthood. The Fontan operation is a unique approach to create a circulation in series without two distinct pumping chambers. Although the Fontan operation increased the survival rates of patients with single ventricle physiology, it carries an inevitable risk of long-term morbidities that impacts the outcomes and quality of life in these patients. In this review, we discuss the challenges resulting from the unique pathophysiology of Fontan circulation and propose management strategies.
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Kheiwa et al. (2018) conducted a review in Single ventricle physiology. Fontan operation was evaluated. The Fontan operation for single ventricle physiology increases survival but carries an inevitable risk of long-term morbidities, necessitating specific management strategies.
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