Lymphatic Embolization of Protein-losing Enteropathy in the Fontan Kreutzer Circulation. Case series

pp. 137-143

Abstract

Background: Between 3% and 20% of Fontan-Kreutzer surgery (FS) patients will develop protein-losing enteropathy (PLE), with high impact on survival. Selective hepatoduodenal lymphatic duct embolization (SHDLE) technique has been proposed as a novel therapeutic target.

Objective: The aim of this study was to evaluate the feasibility, complications and analytical clinical outcome of SHDLE in patients with FS and PLE.

Methods: A retrospective, single-center, case series of patients with FS was carried out, including 5 patients with PLE, who underwent percutaneous SHDLE.

Results: Five patients who underwent FS at a median age of 4 years (range: 3.5-4.3), developed PLE at a median postoperative time of 7 years (range 2.3-15.3). Seven SHDLE were performed, with catheter lymphography, under echocardiographic control. In all 5 patients dilated and tortuous hepatoduodenal lymphatic ducts draining into the small intestine were found, and their embolization was effectively achieved in all cases. Immediately after the procedure all these patients increased their serum albumin level. During a median follow-up of 20 months (range 12-43) 4 patients maintained their albumin levels, while one patient required two new embolizations due to persistent hypoalbuminemia. One patient required surgical intervention due to residual anatomical defect. All patients showed clinical improvement. There were no major complications related to the procedure; 3 patients presented a self-limited episode of melena.

Conclusions: SHDLE was a safe and beneficial tool. The procedure allowed improving serum albumin levels and symptoms in all our patients.