Transcatheter Thoracic Duct Decompression for Multicompartment Lymphatic Failure After Fontan Palliation

Abstract

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.

Transvenous retrograde thoracic duct embolization for effective treatment of recurrent plastic bronchitis after fontan palliation

“…Another viable treatment option is the disruption of cisterna chyli or the decompression of the TD into a lowerpressure chamber but both of these procedures are risky and can be technically challenging. 10,11,13,16 In our patient, abnormal lymphatic vessels were small and numerous and were originating at different levels from the TD. This anatomy was considered unfavorable for either selective embolization of all pathological lymphatic vessels or stenting of the TD as it would have required the implantation of several overlapping covered stents.…”

“…Maintaining TD patency can be important in patients with significant lymphatic congestion and targeted treatment of only the pathologic lymphatic vessels can potentially improve the outcome of such procedures. Another viable treatment option is the disruption of cisterna chyli or the decompression of the TD into a lower‐pressure chamber but both of these procedures are risky and can be technically challenging 10,11,13,16 . In our patient, abnormal lymphatic vessels were small and numerous and were originating at different levels from the TD.…”

“…Embolization of the caudal TD has not been always reported as successful in the setting of a transected TD. 16,18 Likewise, this technique was not clinically beneficial for the patient. The recurrence of PB was probably due to the incomplete closure of the TD caudal portion and the collateral pathways draining into the proximal portion beyond the area of caudal embolization with backflow into the leaks.…”

“…We chose to first embolize the caudal TD because we believed it would be sufficient to eliminate TD flow toward the lungs while mitigating the risk of the procedure. Embolization of the caudal TD has not been always reported as successful in the setting of a transected TD 16,18 . Likewise, this technique was not clinically beneficial for the patient.…”

Management of circulatory failure after Fontan surgery

“…Further, thoracic duct embolization can actually worsen abdominal symptoms in patients with multicompartment lymphatic disease. If selective lymphatic embolization is not successful in these patients with multicompartment involvement, there has been early promise in transcatheter thoracic duct decompression, although this procedure requires considerable technical expertise ( 78 ). Finally, in atriopulmonary Fontan patients with persistent atrial arrhythmias contributing to heart failure symptoms, catheter ablation has been demonstrated to be safe and effective at reducing arrhythmia burden, although approximately half of patients will experience a documented episode of recurrent atrial arrhythmia post procedure and close to one third will require repeat ablation ( 79 ).…”