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澳大利亚和新西兰的Fontan转换手术十年结果表明早期转换策略具有优越性。

Ten-year outcomes of Fontan conversion in Australia and New Zealand demonstrate the superiority of a strategy of early conversion.

作者信息

Poh Chin Leng, Cochrane Andrew, Galati John C, Bullock Andrew, Celermajer David S, Gentles Tom, du Plessis Karin, Winlaw David S, Hornung Tim, Finucane Kirsten, d'Udekem Yves

机构信息

Murdoch Children's Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, VIC, Australia.

Department of Cardiothoracic Surgery, Monash Medical Centre, Monash University Faculty of Medicine, Melbourne, VIC, Australia.

出版信息

Eur J Cardiothorac Surg. 2016 Feb;49(2):530-5; discussion 535. doi: 10.1093/ejcts/ezv112. Epub 2015 Apr 2.

Abstract

OBJECTIVE

To investigate the benefits of a strategy of early Fontan conversion.

METHODS

Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed.

RESULTS

Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.8 years (IQR: 19.3-28.2), 18.7 ± 5.0 years post-Fontan. One centre tended to perform conversion earlier: interval since first documented arrhythmia 2.9 ± 4.0 vs 4.0 ± 4.2 years, average NYHA Class 2 ± 0.4 vs 3 ± 0.9 (P = 0.008), mean number of preop anti-arrhythmics 1 ± 0.4 vs 2 ± 0.6 (P = 0.05). Two patients underwent conversion to an extracardiac conduit only, while 36 had concomitant right atrial cryoablation, of which 16 also had pacemaker implantation. Nine patients suffered major cardiac-related complications (7 low output syndrome, 3 ECMO, 3 acute renal failure, one stroke) (2/17 from the early conversion centre and 7/22 of the others; P = 0.14). Four patients died in hospital (10.3%) and 4 late after a median of 0.9 years [95% confidence interval (CI): 0.5-1] after conversion. An additional 2 patients needed transplantation at 1 and 8.8 years after conversion, respectively. The 10-year freedom from heart transplantation was 86% (95% CI: 51-97%). Outcomes from the centre with an early conversion strategy were significantly better: 8-year freedom from death or heart transplantation was 86% (95% CI: 53-96) vs 51% (95% CI: 22-74; log-rank P = 0.007). Eight additional patients required pacemaker implantation and 5 had arrhythmia recurrence.

CONCLUSIONS

Fontan conversion is associated with lasting survival outcomes up to 10 years after conversion. A strategy of surgical conversion at earlier stage of failure may be associated with better survival free from transplantation.

摘要

目的

探讨早期进行Fontan转换策略的益处。

方法

利用澳大利亚和新西兰Fontan注册中心的数据,对其长期随访资料进行回顾性分析。

结果

1990年至2014年间,6个中心共有39例患者接受了手术转换,中位年龄为23.8岁(四分位间距:19.3 - 28.2岁),Fontan手术后18.7±5.0年。有一个中心倾向于更早进行转换:自首次记录心律失常后的间隔时间为2.9±4.0年对比4.0±4.2年,平均纽约心脏协会(NYHA)心功能分级为2±0.4对比3±0.9(P = 0.008),术前抗心律失常药物平均使用数量为1±0.4对比2±0.6(P = 0.05)。2例患者仅转换为心外管道,36例同时进行了右心房冷冻消融,其中16例还植入了起搏器。9例患者出现严重的心脏相关并发症(7例低心排综合征、3例体外膜肺氧合(ECMO)、3例急性肾衰竭、1例中风)(早期转换中心2/17例,其他中心7/22例;P = 0.14)。4例患者在住院期间死亡(10.3%),4例在转换后中位时间0.9年[95%置信区间(CI):0.5 - 1]后晚期死亡。另外2例患者分别在转换后1年和8.8年需要进行心脏移植。10年免于心脏移植的比例为86%(95%CI:51 - 97%)。采用早期转换策略的中心结局明显更好:8年免于死亡或心脏移植的比例为86%(95%CI:53 - 96%)对比51%(95%CI:22 - 74%;对数秩检验P = 0.007)。另有8例患者需要植入起搏器,5例出现心律失常复发。

结论

Fontan转换与转换后长达10年的持久生存结局相关。在衰竭早期进行手术转换的策略可能与更好的免于移植的生存率相关。

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