Ikai Akio
Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
Kyobu Geka. 2021 Sep;74(10):793-798.
Recently, the number of patients with hypoplastic left heart syndrome reaching the final stage of Fontan operation is increasing due to the systematization of staged therapies including bilateral pulmonary artery banding. However, in case, the systemic and pulmonary circulatory pathways initially formed by the Norwood procedure are not always sufficient to obtain a good Fontan circulation. In particular, depending on the method of reconstruction of the aortic arch, aortic re-coarctation may result in increased ventricular afterload. Furthermore, inadequate aortopulmonary space causes pulmonary artery stenosis, which increases the resistance of the pulmonary artery. In addition, tricuspid regurgitation and increased collateral circulation due to the synergistic effects of multiple reoperations and cyanosis can worsen cardiac function due to increased volume load and further increase pulmonary vascular resistance, preventing the establishment of a good Fontan circulation. In order to resolve these factors before Fontan operation and establish a better Fontan circulation, it is important to develop a comprehensive treatment strategy as well as a step-by-step surgical treatment strategy.
近年来，由于包括双侧肺动脉环扎术在内的分期治疗的系统化，处于Fontan手术最后阶段的左心发育不全综合征患者数量正在增加。然而，在这种情况下，最初由Norwood手术形成的体循环和肺循环途径并不总是足以获得良好的Fontan循环。特别是，根据主动脉弓的重建方法，主动脉再缩窄可能导致心室后负荷增加。此外，主肺动脉间隙不足会导致肺动脉狭窄，从而增加肺动脉阻力。此外，由于多次再次手术和紫绀的协同作用导致的三尖瓣反流和侧支循环增加，会因容量负荷增加而使心功能恶化，并进一步增加肺血管阻力，从而阻碍良好的Fontan循环的建立。为了在Fontan手术前解决这些因素并建立更好的Fontan循环，制定全面的治疗策略以及逐步的外科治疗策略非常重要。
