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Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. Methods and Results This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2-weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty-eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications ( P =0.001). Conclusions There is substantial morbidity in the early post-Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Recent advances in understanding lymphatic disorders have significantly improved noninvasive imaging of the lymphatic circulation, especially in pediatric patients. The ability to obtain high-quality MR imaging using 3-dimensional reconstruction has transformed the display of the lymph vessels and substantially enhanced the ability to diagnose various pediatric lymphatic disorders. Real-time dynamic imaging adds functional information to the assessment. MR imaging is increasingly used, not only to assess but also as a follow-up after lymphatic disorder treatment. This article discusses up-to-date imaging techniques and clinical applications of dynamic magnetic resonance lymphangiography for assessing central conducting lymphatic anomalies., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The lymphatic system has been poorly understood and its importance neglected for decades. Growing understanding of lymphatic flow pathophysiology through peripheral and central lymphatic flow imaging has improved diagnosis and treatment options in children with lymphatic diseases. Flow dynamics can now be visualized by different means including dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), the current standard technique to depict central lymphatics. Novel imaging modalities including intranodal, intrahepatic and intramesenteric DCMRL are quickly evolving and have shown important advances in the understanding and guidance of interventional procedures in children with intestinal lymphatic leaks. Lymphatic imaging is gaining importance in the radiologic and clinical fields and new techniques are emerging to overcome its limitations. [ABSTRACT FROM AUTHOR]