Outcomes After Lymphatic Embolization for Protein-Losing Enteropathy in Congenital Heart Disease

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BACKGROUND: Protein-losing enteropathy (PLE) in patients with congenital heart disease (CHD) is traditionally managed with symptom-focused therapies, with few targeted medical therapies and limited intervention options. Although abnormal hepatic and extrahepatic lymphatic connections are increasingly implicated in PLE, prior embolization reports have largely focused on intrahepatic targets, with variable outcomes and limited long-term follow-up. OBJECTIVES: The goal of this study was to compare the short- and long-term outcomes of isolated intrahepatic (IH) vs combined intrahepatic and periduodenal (IH + PD) embolization strategies in CHD patients. METHODS: We conducted a single-center retrospective review of 71 patients with CHD who underwent percutaneous lymphatic embolization for medically refractory PLE. Multicompartment magnetic resonance lymphangiography was performed, followed by IH or combined IH + PD embolization. The primary endpoint was duration of remission (serum albumin ≥3.0 g/dL without IV albumin supplementation or repeat percutaneous PLE intervention). Secondary analyses included initial response (albumin ≥3.0 g/dL within 30 days without intravenous albumin supplementation), impact of thoracic duct obstruction, and transplant-free survival. RESULTS: A total of 71 patients underwent embolization procedures (17 IH, 54 IH + PD). Duodenal leaks were identified in all patients undergoing multicompartment magnetic resonance lymphangiography. IH + PD embolization was associated with higher initial response rates (87% vs 41%; P < 0.0004). Median serum albumin levels increased from 2.3 g/dL preprocedure to 4.2 g/dL postprocedurally in the IH + PD group (P < 0.0001). PLE recurrence occurred in fewer IH + PD patients than IH patients (45% vs 100%; P = 0.005) during the follow-up period. Thoracic duct obstruction was associated with shorter remission duration than those without obstruction (P = 0.0005). Sustained remission (albumin ≥3 g/dL for ≥1 year) was achieved in 40% (22 of 54) of IH + PD patients and only 11% (2 of 17) of IH patients (P = 0.04). In an exploratory secondary analysis, sustained remission was associated with a higher likelihood of transplant-free survival compared with patients with recurrence or no response (at 3 years: 85% vs 50%; P = 0.003). Adverse events were common in this high-risk population, including transient pancreatitis in 75% and hyperbilirubinemia in 40%, most of which were self-limited or managed conservatively. The likelihood of serious and catastrophic adverse events after IH and IH + PD procedures was not significantly different (level 3c/4/5: 43% vs 44% [P > 0.99]; level 4/5: 8.7% vs 3.5% [P = 0.57]). CONCLUSIONS: A combined IH + PD embolization strategy was associated with higher initial response rates and longer duration of remission compared with IH embolization alone. These findings support a more comprehensive embolization approach as a promising interventional therapy for this high-risk population.

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