重新定义法乐四联症根治术后长期生存的预期：来自澳大利亚和新西兰全部人群的 25 年随访结果。
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.
机构信息
From the Department of Cardiac Surgery (Y.d., A.J.I.) and Department of Cardiology (R.G.W.), Royal Children's Hospital, Melbourne, Victoria, Australia; Murdoch Childrens Research Institute, Melbourne, Victoria, Australia (Y.d., A.J.I, J.C.G.); Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia (A.J.I., R.G.W.); Department of Mathematics & Statistics, La Trobe University, Melbourne, Victoria, Australia (J.C.G.); Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia (R.G.W., G.F.S., D.S.W.); Department of Cardiology, Women's and Children's Hospital, Adelaide, South Australia, Australia (G.R.W.); Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia (A.B); Paediatric Cardiology, Queensland Paediatric Cardiac Service, Mater Children's Hospital, Brisbane, Queensland, Australia (R.N.J.); Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia (L.E.G.); Paediatric Cardiology Services, Monash Heart, Monash Health, Melbourne, Victoria, Australia (S.H.); Department of Medicine, Monash University, Melbourne, Australia (S.H.); Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia (D.J.R.); Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand (T.L.G.); Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia (D.S.C.); Department of Medicine, The University of Sydney, New South Wales, Australia (D.S.C.); The University of Notre Dame, Sydney, New South Wales, Australia (V.F.); on behalf of the Australia and New Zealand Fontan Registry.
出版信息
Circulation. 2014 Sep 9;130(11 Suppl 1):S32-8. doi: 10.1161/CIRCULATIONAHA.113.007764.
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<0.001; 95% CI, 2.4-16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%-76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0-7.1). Ten-year freedom from failure was 79% (95% CI, 61%-89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%-95%) for other morphologies.
CONCLUSIONS
The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.
背景
行 Fontan 手术患者的预期寿命尚不清楚。
方法和结果
从包括所有儿科和成人心脏中心的一个澳新两国基于人群的注册中心获取了在澳大利亚和新西兰接受 Fontan 手术的 1089 例患者中的 1006 例幸存者的随访资料。有 203 例房室连接（AP；1975-1995 年），271 例侧隧道（1988-2006 年）和 532 例心外管道（1997-2010 年）。左心发育不全综合征患者的比例从 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%），侧隧道和心外管道的生存率为 97%（95%置信区间[CI]，94%-99%）。AP 的最长生存估计值为 25 年时的 76%（95%CI，67%-82%）。AP 独立预测与心外管道相比生存状况较差（风险比，6.2；P<0.001；95%CI，2.4-16.0）。Fontan 术后 20 年无失败（死亡、移植、拆除、转换为心外管道、纽约心脏协会 III/IV 级或蛋白丢失性肠病/塑料性支气管炎）的比例为 70%（95%CI，63%-76%）。左心发育不全综合征是 Fontan 失败的主要预测因素（风险比，3.8；P<0.001；95%CI，2.0-7.1）。左心发育不全综合征患者的 10 年无失败生存率为 79%（95%CI，61%-89%），而其他形态患者为 92%（95%CI，87%-95%）。
结论
澳大利亚和新西兰 Fontan 人群的长期生存率非常好。AP Fontan 患者的 25 年生存率为 76%。技术改良进一步提高了生存率。左心发育不全综合征患者失败风险较高。此类大型综合性注册中心将进一步提高我们对 Fontan 手术后晚期结果的了解。
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