Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Children's Research Institute, Melbourne, Australia.
Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiology, The Prince Charles Hospital, Brisbane, Australia.
J Thorac Cardiovasc Surg. 2021 Jun;161(6):2158-2165.e4. doi: 10.1016/j.jtcvs.2020.07.107. Epub 2020 Aug 12.
Protein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors and outcomes of protein losing enteropathy and plastic bronchitis in patients undergoing the Fontan.
We performed a retrospective analysis of 1561 patients from the Australia New Zealand Fontan Registry. Two end points were death and cardiac transplantation examined with Cox regression (if no competing risks) or cumulative incidence curves and cause-specific Cs regression.
A total of 55 patients with protein losing enteropathy/plastic bronchitis were included. Their median age at the Fontan was 5.7 years, and time to onset after the Fontan for protein losing enteropathy was 5.0 years and plastic bronchitis was 1.7 years. Independent predictors for developing protein losing enteropathy/plastic bronchitis were right-ventricular morphology with hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older age at Fontan (hazard ratio, 1.13; confidence interval, 1.03-1.23), and pleural effusions after Fontan (hazard ratio, 2.43; confidence interval, 1.09-5.41); left-ventricular morphology was protective (hazard ratio, 0.36; confidence interval, 0.18-0.70). In the protein losing enteropathy/plastic bronchitis population, freedom from death or transplantation after protein losing enteropathy/plastic bronchitis diagnosis at 5, 10, and 15 years was 70% (confidence interval, 58-85), 65% (confidence interval, 51-83), and 43% (confidence interval, 26-73), respectively; only older age (hazard ratio, 1.23; confidence interval, 1.01-1.52) was an independent predictor. Twenty-six surgical interventions were performed in 20 patients, comprising Fontan revisions (n = 5), fenestrations (n = 11), Fontan conversions (n = 5), atrioventricular valve repairs (n = 3), and hepatic vein diversion (n = 2).
Protein losing enteropathy and plastic bronchitis remain severe complications, preferably affecting patients with dominant right single ventricle, with older age at Fontan being a predictor of developing protein losing enteropathy/plastic bronchitis and poorer prognosis. Heart transplantation remains the ultimate treatment, with 30% dying or requiring transplantation within 5 years, and the remaining being stable for long periods.
蛋白丢失性肠病和塑型性支气管炎是 Fontan 循环中的严重并发症，5 年生存率为 46%至 88%。我们报告了在 Fontan 手术后发生蛋白丢失性肠病和塑型性支气管炎患者的风险因素和结局。
我们对澳大利亚新西兰 Fontan 登记处的 1561 例患者进行了回顾性分析。使用 Cox 回归（如果没有竞争风险）或累积发生率曲线和特定因果 Cs 回归分析死亡和心脏移植两个终点。
共纳入 55 例蛋白丢失性肠病/塑型性支气管炎患者。Fontan 时的中位年龄为 5.7 岁，蛋白丢失性肠病的 Fontan 后发病时间为 5.0 年，塑型性支气管炎为 1.7 年。发生蛋白丢失性肠病/塑型性支气管炎的独立预测因素包括右心室形态伴左心发育不全综合征（风险比，2.30；置信区间，1.12-4.74）、Fontan 时年龄较大（风险比，1.13；置信区间，1.03-1.23）和 Fontan 后胸腔积液（风险比，2.43；置信区间，1.09-5.41）；左心室形态为保护因素（风险比，0.36；置信区间，0.18-0.70）。在蛋白丢失性肠病/塑型性支气管炎人群中，蛋白丢失性肠病/塑型性支气管炎诊断后 5、10 和 15 年的无死亡或移植生存率分别为 70%（置信区间，58-85）、65%（置信区间，51-83）和 43%（置信区间，26-73），仅年龄较大（风险比，1.23；置信区间，1.01-1.52）是独立预测因素。20 例患者中有 26 例接受了手术干预，包括 Fontan 修正术（n=5）、开窗术（n=11）、Fontan 转换术（n=5）、房室瓣修复术（n=3）和肝静脉分流术（n=2）。
蛋白丢失性肠病和塑型性支气管炎仍是严重的并发症，主要影响右优势单心室患者，Fontan 时年龄较大是发生蛋白丢失性肠病/塑型性支气管炎和预后较差的预测因素。心脏移植仍是最终治疗方法，5 年内 30%的患者死亡或需要移植，其余患者可长期稳定。
