日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

In the perioperative management of Fontan operation, it is important to preserve low pulmonary vascular resistance (PVR). In a normal heart, the pulmonary circuit and systemic circuit are connected in series and both circuits have respective ejection ventricles. On the other hand, in Fontan circulation, only systemic circuit has an ejection ventricle although the pulmonary circuit is maintained by a simple pressure gradient from the systemic vein to the left atrium without an ejection ventricle. Therefore, high systemic venous pressure is required to maintain the pulmonary circulation. Hence, low PVR is essential to obtain a stable Fontan circulation. It is necessary to understand the mechanics and dynamics of Fontan circulation in depth to achieve an ideal perioperative management of Fontan operation.