Late Total Cavopulmonary Connection in Adolescents and Adults: Narrative Review of Patient Selection and Outcomes

Background: Although most patients with univentricular physiology undergo staged palliation during childhood, a small subset reaches adolescence or adulthood without complete cavopulmonary connection. The role of late total cavopulmonary connection (TCPC) in these patients remains poorly defined. This review summarizes the available evidence regarding TCPC surgery in adolescents and adults and discusses contemporary patient selection. Methods: A structured narrative review of PubMed/MEDLINE was conducted from database inception to December 2024. Studies reporting primary Fontan or TCPC completion in adolescents and adults were included, whereas studies exclusively addressing Fontan conversion were excluded. The review was conducted according to SANRA recommendations. Results: Sixteen studies reporting outcomes after late TCPC completion were identified. Most adult candidates presented with cyanosis, reduced functional capacity, previous palliation, and dominant left ventricular morphology. Surgical techniques evolved from atriopulmonary connections to lateral tunnel and extracardiac conduit TCPC. Early postoperative mortality ranged from 0% to 20%, with low cardiac output syndrome representing the leading cause of death. Reported survival at 5, 10, and 15 years ranged from 76 to 95%, 71-81%, and 66-79%, respectively. Preserved ventricular function, low pulmonary vascular resistance, and absence of significant atrioventricular valve regurgitation were consistently associated with better outcomes. Conclusions: Late TCPC surgery in carefully selected adults is feasible and may improve oxygenation and functional status. Contemporary decision-making should integrate anatomical, hemodynamic, functional, rhythm, and end-organ assessment while considering alternative strategies such as transplantation or conservative management.