Surgical thoracic duct decompression: The right choice for Fontan-associated protein-losing enteropathy?

Objective

Protein-losing enteropathy is among the most debilitating complications of Fontan circulation. Central venous hypertension increases lymphatic pressure in the thoracic duct, potentially leading to significant intestinal protein loss. Current treatment options for recurrent or refractory protein-losing enteropathy are limited to complex lymphatic interventions, fenestration creation, and heart transplantation. We have implemented an alternative surgical approach—thoracic duct decompression—and report our early experience in pediatric Fontan patients.

Methods

We studied 5 Fontan patients, analyzing their preoperative history, treatment, and surgical approach tailored to their unique anatomy, postoperative course, and symptoms at their most recent Fontan clinic follow-up.

Results

Since February 2024, 5 Fontan patients aged 5 to 16 years diagnosed with recurrent protein-losing enteropathy underwent surgical thoracic duct decompression. The first 2 patients experienced mild anastomotic narrowing, which was successfully treated with transcatheter angioplasty. The remaining 3 patients had uneventful postoperative course and hospital stays of approximately 1 week. All patients were closely monitored with echocardiograms and laboratory testing. At 6 months postoperatively, all remained symptom-free and transplant-free (follow-up range, 7-12 months), and reported improved quality of life. No cases of turndown stenosis were observed. Additionally, all patients were successfully weaned off enteral steroids and aggressive diuretic therapy. A mild decrease in oxygen saturations (1%-3%) was noted with no clinical significance.

Conclusions

Thoracic duct decompression appears to be a feasible intervention for recurrent protein-losing enteropathy in Fontan patients, demonstrating a low rate of short-term complications. This procedure may serve as a viable alternative to heart transplantation in select cases; however, its long-term efficacy warrants further investigation.