The Fontan operation is the final stage of palliative surgery for children born with single ventricle heart defects. The most common configuration is called total cavopulmonary connection (TCPC), wherein the inferior vena cava and superior vena cava are anastomosed directly to the pulmonary arteries; therefore the pulmonary circulation is driven by venous pressure only. The Fontan procedure, although successful in the early postoperative period, with time can decrease in efficiency or even fail within several years after the operation. The reasons of different clinical outcomes for some of the Fontan patients are not clear enough, even though it is commonly accepted that certain factors such as low pulmonary vascular resistance and proper shape and size of the TCPC construction are crucial for the succesful long term outcomes. Accordingly, one of the major problems is the increase in pulmonary vascular resistance due to altered hemodynamics after the surgery, causing venous hypertension and respiratory-dependent pulmonary regurgitation [1]. The main pulmonary arteries may also see increased resistance due to congenital malformations, surgical scarring, or deliberate surgical banding. Thus, the consequence of the increased pulmonary vascular resistance at both proximal and distal locations with respect to the TCPC junction, and its effect on the systemic pressures and flow rates, is the main objective of this study.

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