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PURPOSE: To assess effectiveness of selective lymphatic duct embolization (SLDE) for treatment of thoracic lymphatic flow disorders, performed to maintain patency of the thoracic duct (TD), compared to thoracic duct embolization (TDE). MATERIALS AND METHODS: Records of 39 patients (mean age 7.6±7.1 years) with thoracic lymphatic flow disorders who underwent 43 SLDE procedures were analyzed and compared to a cohort of 104 patients (7.8±7.6 y) who underwent TDE. Relevant clinical and imaging data were collated. All patients presented with chylous effusion and/or plastic bronchitis. For the SLDE cohort, etiology of disorders included single-ventricle heart disease palliation in 28 patients (72%) and vascular malformation/aneuploidy in 8 (21%). For the TDE cohort, 92/104(88%) had single-ventricle heart disease palliation. RESULTS: SLDE was performed with glue via microcatheter in 35 procedures, and dextrose flood technique was used in 26. SLDE via direct needle access was performed in 11. After SLDE, presenting symptoms resolved in 33/39 patients (85%), improved in 5 (13%), and were unchanged in 1 (3%) (mean follow-up 693±523 days). The TD remained patent in all cases. There was a significant difference in outcomes (P=0.001) with the TDE cohort-symptoms resolved or improved in 75/104 patients (72%), were unchanged in 15 (14%); and new leak (e.g., ascites, protein-losing enteropathy) developed in 14 TDE patients (13%) (follow-up 1041±879 d). CONCLUSION: SLDE was effective for treatment of thoracic lymphatic flow disorders, and outcomes were comparable to TDE. Selective techniques preserve the patency of the TD and may have potential benefits that warrant further study.
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Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible. Lymphatic abnormalities were classified into four types based on severity and location of lymphatic vessels. Classifications were compared between images and related to clinical outcomes such as postoperative drainage and hospitalization, lymphatic complications, heart transplant, and death. Results Forty-three patients (median age, 10 years [IQR, 8-11]; 20 [47%] boys, 23 [53%] girls) were included in the study. Lymphatic abnormalities progressed in 19 individuals after Fontan completion (distribution of lymphatic classifications: type 1, 23; type 2, 11; type 3, 6; type 4, 3 vs type 1, 10; type 2, 18; type 3, 10; type 4, 5; P = .04). Compared with individuals showing no progression of lymphatic abnormalities, those progressing to a high-grade lymphatic classification had longer postoperative drainage (median time, 9 days [IQR, 6-14] vs 17 days [IQR, 10-23]; P = .04) and hospitalization (median time, 13 days [IQR, 9-25] vs 26 days [IQR, 18-30]; P = .03) after Fontan completion and were more likely to develop chylothorax (12% [three of 24] vs 75% [six of eight]; P < .01) and/or protein-losing enteropathy (0% [0 of 24] vs 38% [three of eight]; P < .01) during a median follow-up of 8 years (IQR, 5-9). Progression to any type was not associated with an increased risk of adverse events. Conclusion The study demonstrated that lymphatic structural abnormalities may progress in select individuals with single ventricle physiology after Fontan completion, and progression of abnormalities to a high-grade classification was associated with worse postoperative outcomes. Keywords: Congenital Heart Disease, Glenn, Fontan, Lymphatic Imaging, Cardiovascular MRI Supplemental material is available for this article. Published under a CC BY 4.0 license.