Abstract
The Fontan circulation is surgical solution of complicated heart defects in cases when it is not possible to maintain distinct systemic and pulmonary circulations. It involves several consecutive surgical operations, the final step being the attachment of the superior vena cava to a pulmonary artery branch and connection of the vena cava to the pulmonary artery through an intra-atrial or extracardiac tunnel.
This gives rise to neoportal circulation comprising two capillary circulations in tandem, dependent on good systolic and diastolic function of the common ventricle, central venous pressure, and low pulmonary vascular resistance. Long-term survival of patients with the Fontan circulation is very good; however, morbidity is high.
Frequent complications include heart failure, arrhythmias, and increased pulmonary vascular resistance that, alone or in combination, may lead to collapse of this delicate circulation. Moreover, damage to other systems often occurs, most commonly involving the liver function and haemostasis.
Residual findings following primary surgery can be managed with surgery or catheterization; no evidence base exists for medical therapy.