Introduction: The lymphatic system is crucial for fluid balance, fat transport, and immune response. Patients with congenital heart disease are at increased risk of complications due to lymphatic system abnormalities, such as protein-losing enteropathy, chylothorax, and chylous ascites. Identifying and managing lymph leakage sources remains challenging, with percutaneous interventions emerging as viable treatment options.

Objective: This report details our initial experiences conducting lymphangiography on patients with congenital heart disease and lymphatic disorders at a quaternary hospital between 2021 and 2023.

Method: Patients with congenital heart disease displaying signs of lymphatic disorders underwent lymphangiography. We punctured inguinal lymph nodes and hepatic lymphatics, administering Lipiodol® with or without Histoacryl®. Upon identifying the main thoracic duct, we attempted either direct puncture or retrograde access through its outlet in select cases. For patients with a fenestrated Fontan, we occluded the fenestrations during the procedure to prevent Lipiodol® embolization into the systemic circulation.

Results: We performed ten lymphangiographies on nine patients from 2021 to 2023. The patients' average age was 12.3 years (range: 5 months to 28.3 years), and their average weight was 29.48 kg (range: 5.5 to 71 kg). Eight of these patients had undergone univentricular palliation (including five Fontans, two Glenn procedures, and one Fontan with Hraska modification), and one was post-heart transplant. Clinically, five patients presented with chylothorax, three with protein-losing enteropathy, and one with ascites. We identified the thoracic duct in seven patients and successfully performed transabdominal punctures in four (two successfully). Two patients underwent successful retrograde thoracic duct catheterization. There was a resolution of chylothorax in four patients (80%), with output improving within an average of 4.7 days (range: 1-8 days) and drain removal by 11 days (range: 6-17 days). In cases of protein-losing enteropathy, two patients experienced improved diarrhea and edema/ascites (one lacked clinical data). One case showed transient improvement, leading to a follow-up study ten months later where a thoracic duct outlet stenosis was treated, resulting in clinical improvement. The patient with ascites, related to multiple abdominal surgeries, showed no improvement. There were no procedural complications. Patients undergoing transabdominal punctures reported post-procedural abdominal discomfort; one experienced gastrointestinal bleeding.

Conclusion: Our initial experience indicates that lymphangiography with Lipiodol® is a safe and effective percutaneous intervention for diagnosing and treating lymphatic disorders.