Ichikawa Hajime
Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Japan.
Kyobu Geka. 2013 Jul;66(8 Suppl):674-9.
In the recent years, the Fontan completion rate for hypoplastic left heart syndrome (HLHS) is improved because of the outcome of stage-1 palliation improved. However, there are still problems to be solved to achieve better hemodynamics after Fontan operation in patients with HLHS. The 2 major commandments other than classic 10 commandments are A:"good ventricular function" and B:"low pulmonary vascular resistance". To obtain these 2, the following problems should be rectified. They include residual arch obstruction, impaired right ventricule (RV) function, tricuspid regurgitation, elevated pulmonary vascular resistance, development of aorto-pulmonary collaterals and etc. The modern strategy for the treatment to achieve "good" Fontan circulation in HLHS patients, are 1)Norwood procedure with RV-pulmonary artery( PA) conduit, 2)staged palliation strategy, 3)repair co-existent and residual abnormality before stage-3(preferably at stage-2 palliation), 4)coil embolization of aorto-pulmonary collateral arteries before Fontan procedure, 5)the type of Fontan operation is extracardiac total cavopulmonary connection (TCPC) type, 6)fenestration if necessary, 7)careful and continuous follow-up is mandatory for early detection of arrhythmia, protein losing enteropathy (PLE), liver dysfunction and other fatal complications of long term after Fontan type procedure.
近年来，由于一期姑息治疗效果的改善，左心发育不全综合征（HLHS）的Fontan手术完成率有所提高。然而，在HLHS患者中，为了在Fontan手术后实现更好的血流动力学，仍有一些问题有待解决。除了经典的十诫之外，另外两条主要戒律是A：“良好的心室功能”和B：“低肺血管阻力”。为了实现这两点，需要纠正以下问题。这些问题包括残余主动脉弓梗阻、右心室（RV）功能受损、三尖瓣反流、肺血管阻力升高、主肺动脉侧支形成等。在HLHS患者中实现“良好”Fontan循环的现代治疗策略包括：1）采用右心室-肺动脉（PA）导管的Norwood手术；2）分期姑息治疗策略；3）在三期之前（最好在二期姑息治疗时）修复并存和残余的异常；4）在Fontan手术前对主肺动脉侧支动脉进行弹簧圈栓塞；5）Fontan手术类型为心外全腔静脉-肺动脉连接（TCPC）型；6）必要时开窗；7）必须进行仔细且持续的随访，以便早期发现心律失常、蛋白丢失性肠病（PLE）、肝功能障碍以及Fontan手术长期后的其他致命并发症。
