Abstract
In recent decades, the number of living patients with a functionally single ventricle has increased in the population. Complex congenital heart defects are associated under this diagnosis, in which the heart cannot be surgically divided into two separate functional ventricles chambers.
The prognosis of children born with a functionally single ventricle has significantly improved with the introduction of the Fontan circulation principle into clinical practice in the 1980s. It consists in the direct connection of the systemic venous return to the pulmo- nary circulation.
The target operation is currently a Total Cavopulmonary Connection (TCPC), in which the upper vena cava is connected to the pulmonary branch directly and the inferior vena cava via a valve less intracardiac or extracardiac conduit. The heart is excluded from the pulmonary circulation and the ventricle is connected only to the systemic circulation.
From a pathophysiological point of view, blood flow through the lungs is dependent on the transpulmonary gradient between the central venous pressure and the pressure in the atrium of the pulmonary veins. Strict indication criteria must be met for this system to function, including low pulmonary pressure, low arteriolar pulmonary resistance, sufficient lung capacity, and good myocardial and heart valve function.
In the end, TCPC ensures almost complete oxygenation of the blood during optimal cardiac work at the cost of increased venous pressure.