Redefining Expectations of Long-Term Survival After the Fontan Procedure Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand
Redefining Expectations of Long-Term Survival After the Fontan Procedure Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand
复制标题
DOI：
10.1161/circulationaha.113.007764
复制
发表时间：
2014-09-09
期刊：
影响因子：
37.8
通讯作者：
Winlaw, David S.
中科院分区：
文献类型：
--
作者：
d'Udekem, Yves;Iyengar, Ajay J.;Winlaw, David S.
背景-接受Fontan程序的患者的预期寿命是unknown.Methods and Results-随访的所有1006幸存者的1089例患者谁接受了Fontan程序在澳大利亚和新西兰是从一个两国人口为基础的注册表，包括所有的儿科和成人心脏中心。有203个心房连接（AP; 1975-1995），271个侧隧道（1988-2006）和532个心外管道（1997-2010）。左心发育不良综合征的比例从1990年前的1/173（1%）增加到2000年后的80/500（16%）。AP的10年生存率为89%（84%-93%），外侧隧道和心外管道的10年生存率为97%（95%置信区间[CI]，94%-99%）。AP患者25年时的最长生存率估计值为76%（95% CI，67%-82%）。与心外管道相比，AP独立预测生存率更差（风险比，6.2; P
Background-The life expectancy of patients undergoing a Fontan procedure is unknown.Methods and Results-Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975-1995), 271 lateral tunnels (1988-2006), and 532 extracardiac conduits (1997-2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%-93%) for AP and 97% (95% confidence interval [CI], 94%-99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%-82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P