The adult population with congenital heart defects of varying complexity is growing rapidly. This includes a significant number of patients over the age of 18 who underwent surgery in early childhood to correct conotruncal anomalies and septal defects. While patients with a functional single ventricle palliated with the Fontan procedure are still relatively rare in developing countries, the expertise gained from managing these patients in expert centers in Europe and the United States of America, as well as the success of pediatric cardiac surgery, has drawn increasing attention to this subject. In this review article, we aim to discuss the unique pathophysiology of Fontan circulation and clinical issues in the management of adult patients, with a particular focus on identifying specific predictors of heart failure decompensation and potential complications in this population.
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