Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania.
Circ Cardiovasc Interv. 2022 Jul;15(7):e011733. doi: 10.1161/CIRCINTERVENTIONS.121.011733. Epub 2022 Jun 16.
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.
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.
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.
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.
淋巴栓塞疗法已被证明对因塑性支气管炎或蛋白丢失性肠病导致的 Fontan 衰竭有效，但对于涉及多个淋巴间隙的情况则无效；此外，栓塞并不能改变淋巴功能障碍的潜在病理生理学。本文介绍了一种经导管胸导管减压（TDD）技术，该技术可将胸导管重新引导至肺静脉心房，以治疗多间隙淋巴衰竭，并报告了早期结果。
最初使用覆盖支架将无名静脉血流引导至腔静脉肺循环内。开发了一种改良方法，其中覆盖支架通过血管外途径将无名静脉直接引导至左心房。回顾了所有接受 TDD 的患者的基线和随访数据。
2018 年 3 月至 2021 年 2 月期间，12 例患者接受了 TDD，中位年龄为 12 岁（范围：2-22 岁）。中位每个患者有 3 个淋巴间隙发生衰竭（蛋白丢失性肠病、腹水、胸腔积液、塑性支气管炎）；10 例患者在 TDD 前接受了淋巴栓塞。TDD 方法为经 Fontan 隧道内 4 例，直接途径 7 例，其他途径 1 例。无重大手术并发症；6 例患者随后进行了手术，最常见的是治疗内漏。6 例患者在 6 个月（范围：1-20 个月）的随访中淋巴衰竭得到缓解，2 例改善，4 例无变化。1 例患者在 TDD 后死于 Fontan 衰竭。
TDD 是治疗多间隙淋巴衰竭导致的 Fontan 衰竭生理功能的一种有前途的新治疗方法。需要进一步的工作来完善该技术并确定最佳的候选人群。
