Fontan F, Kirklin J W, Fernandez G, Costa F, Naftel D C, Tritto F, Blackstone E H
Department of Cardiovascular Surgery, Hopital Cardiologique du Haut-Leveque, Bordeaux, France.
Circulation. 1990 May;81(5):1520-36. doi: 10.1161/01.cir.81.5.1520.
A study was undertaken to determine the early and long-term outcomes dictated by the Fontan state per se (a state in which the force driving pulmonary blood flow is solely or largely a residue, in the systemic venous pressure, of the main ventricular chamber's contractile force) and the transition (by surgery) to it from the state of congenital heart disease under optimal conditions (after a "perfect" Fontan operation). The primary study design used a solution of a multivariate risk factor equation for death, by which survival rate under optimal conditions was predicted to be 92%, 89%, 88%, 86%, 81%, and 73% at 1 month, 6 months, and 1, 5, 10, and 15 years, respectively, after the Fontan operation. The hazard function (instantaneous risk of death at each moment in time after the operation) had an early rapidly declining phase of hazard that at about 6 months began to give way to a late hazard phase, which was rising by about 6 years after surgery. A secondary study design, using the theory of competing risks, yielded survival and hazard function information very similar to that of the primary study design. The functional capacity of the patients as expressed by New York Heart Association class was less, the longer the period of follow-up. No risk factors (other than older age at time of surgery) were found for the late decline in survival or the decline in functional status. The inference is that the premature decline in survival and functional status and the late rise in hazard function are from the Fontan state per se and that the Fontan operation is, therefore, palliative but not curative.
本研究旨在确定Fontan循环本身（一种状态，即驱动肺循环血流的力量完全或主要是主心室收缩力在体静脉压力中的残余部分）以及在最佳条件下（经过“完美”的Fontan手术）从先天性心脏病状态向该状态转变的早期和长期结果。主要研究设计采用了死亡的多变量风险因素方程解，据此预测在Fontan手术后1个月、6个月以及1年、5年、10年和15年时，最佳条件下的生存率分别为92%、89%、88%、86%、81%和73%。风险函数（手术后每个时刻的瞬时死亡风险）在早期有一个快速下降的风险阶段，大约在6个月时开始让位于后期风险阶段，该阶段在手术后约6年开始上升。使用竞争风险理论的次要研究设计得出的生存和风险函数信息与主要研究设计非常相似。纽约心脏协会分级所表示的患者功能能力随随访时间延长而降低。未发现（除手术时年龄较大外）生存后期下降或功能状态下降的风险因素。由此推断，生存和功能状态的过早下降以及风险函数的后期上升源于Fontan循环本身，因此Fontan手术是姑息性而非根治性的。
