Background:Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented.Methods:Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. A modified approach was developed where covered stents redirected innominate vein directly to the left atrium via an extravascular course. Baseline and follow-up data on all patients undergoing TDD were reviewed.Results:Twelve patients underwent TDD between March 2018 and February 2021 at a median age of 12 (range: 2–22) years. Lymphatic failure occurred in median of 3 compartments per patient (protein-losing enteropathy, ascites, pleural effusions, plastic bronchitis); 10 patients had lymphatic embolizations before TDD. TDD method was intra-Fontan tunnel in 4, direct approach in 7, and other in 1. There were no major procedural complications; 6 patients underwent subsequent procedures, most commonly to treat endoleaks. Lymphatic failure resolved in 6 patients, improved in 2, and was unchanged in 4 at 6 (range: 1–20) months follow-up. One patient died after TDD from Fontan failure.Conclusions:TDD is a promising new treatment for the failing Fontan physiology from multicompartment lymphatic failure. Additional work is needed to refine the technique and define optimal candidates.

中文翻译：

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Fontan 姑息术后多室淋巴衰竭的经导管胸导管减压术

背景：淋巴栓塞治疗已被证明对塑料性支气管炎或蛋白丢失性肠病引起的 Fontan 衰竭有效，但在涉及多个淋巴隔室时无效；此外，栓塞不会改变淋巴功能障碍的潜在病理生理学。描述了一种经导管胸导管减压 (TDD) 技术，将胸导管重新引导至肺静脉心房以治疗多室淋巴衰竭并提出了早期结果。方法：最初覆盖的支架用于引导腔肺通路内的无名静脉流动进入肺静脉心房。开发了一种改进的方法，其中覆盖支架通过血管外路径将无名静脉直接重定向到左心房。回顾了所有接受 TDD 患者的基线和随访数据。结果：12 名患者在 2018 年 3 月至 2021 年 2 月期间接受了 TDD，中位年龄为 12 岁（范围：2-22）岁。每名患者发生淋巴衰竭的中位数为 3 个（蛋白质丢失性肠病、腹水、胸腔积液、塑料性支气管炎）；10 名患者在 TDD 前有淋巴栓塞。TDD法4例为Fontan隧道内，7例为直接入路，1例为其他。无重大手术并发症；6 名患者接受了后续手术，最常见的是治疗内漏。在 6 个月（范围：1-20）的随访中，6 名患者的淋巴衰竭得到解决，2 名患者改善，4 名患者没有变化。一名患者在 TDD 后因 Fontan 失败而死亡。结论：TDD 是一种有前途的新疗法，可用于治疗多室淋巴衰竭导致的 Fontan 生理功能障碍。需要额外的工作来完善该技术并定义最佳候选者。