Norwood W I, Jacobs M L
Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Pennsylvania 19104.
Am J Surg. 1993 Nov;166(5):548-51. doi: 10.1016/s0002-9610(05)81151-1.
Since 1985, 381 patients with various cardiac malformations that share the central common feature of only one effective ventricle have been treated with some modification of Fontan's operation. Since 1989, the Fontan operation has been staged by associating the superior vena cava with the branch pulmonary arteries (hemi-Fontan) initially followed, some months later, by associating the inferior vena cava to the branch pulmonary arteries (completion Fontan). The difference in early mortality for a primary-Fontan operation (16%) compared with a completion-Fontan operation (7%) is substantial (p < 0.05). Since January 1991, mortality associated with the hemi-Fontan operation has been 5.5% (7 deaths in 127 patients). A systematic staged approach to Fontan's operation has been undertaken with a hemi-Fontan operation in patients who are 6 months of age and a completion Fontan operation when those patients are 12 to 18 months of age in an effort to reduce the volume load of the ventricle as early as possible, to minimize intermediate mortality from the palliated state, and to reduce the impact of rapid changes in ventricular geometry and diastolic function that can accompany either the hemi-Fontan or primary Fontan operation, but that are lethal only after a primary Fontan operation.
自1985年以来，381例具有仅一个有效心室这一共同核心特征的各种心脏畸形患者接受了Fontan手术的某种改良治疗。自1989年起，Fontan手术分阶段进行，最初将上腔静脉与肺动脉分支相连（半Fontan手术），数月后再将下腔静脉与肺动脉分支相连（完成Fontan手术）。一期Fontan手术的早期死亡率（16%）与完成Fontan手术的早期死亡率（7%）相比差异显著（p<0.05）。自1991年1月以来，半Fontan手术的死亡率为5.5%（127例患者中有7例死亡）。已对Fontan手术采用系统的分阶段方法，对6个月大的患者进行半Fontan手术，对12至18个月大的患者进行完成Fontan手术，以尽早减轻心室的容量负荷，将姑息状态下的中期死亡率降至最低，并减少半Fontan手术或一期Fontan手术可能伴随的心室几何形状和舒张功能快速变化的影响，而这种变化仅在一期Fontan手术后才是致命的。
