Ishigami Shuta, King Gregory, Buratto Edward, Fricke Tyson A, Weintraub Robert G, Brizard Christian P, Konstantinov Igor E
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia.
Department of Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
J Thorac Cardiovasc Surg. 2025 Feb;169(2):400-410.e7. doi: 10.1016/j.jtcvs.2024.05.030. Epub 2024 Jun 17.
The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution.
We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up.
The median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7).
Rates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures.
本研究旨在评估单中心体外循环Fontan手术的结果。
我们对一家机构的398例患者进行了回顾性研究，这些患者在1997年至2020年间首次接受了体外循环Fontan手术。我们确定了Fontan循环衰竭的发生率和危险因素，其中包括死亡、Fontan手术拆除、心脏移植、蛋白丢失性肠病、塑料支气管炎以及最后一次随访时的功能状态。
中位随访时间为10.3年（四分位间距，6.4 - 14.6年）。体外循环Fontan手术后10年和20年的总生存率分别为96%和86%。有6例早期死亡（6/398，1.5%）和15例晚期死亡（15/398，3.8%）。49例患者（12.5%）发生了Fontan循环衰竭。Fontan循环无衰竭生存率在10年时为88%，在20年时为76%。Fontan循环衰竭的危险因素包括右心室优势（风险比，4.7；P <.001；95%可信区间，2.1 - 10.5）、主动脉闭锁（风险比，5.5；P <.001；95%可信区间，2.3 - 12.8）以及平均肺动脉压升高（风险比，2.3；P =.002；95%可信区间，1.2 - 6.7）。
现代体外循环Fontan手术后Fontan循环衰竭的发生率较低。体外循环Fontan循环衰竭的危险因素包括右心室优势、主动脉闭锁和肺动脉压升高。
