OBJECTIVES Complications after Fontan surgery have been associated with arise and classification of abnormal thoracic lymphatic perfusion pattern. This study compiles abnormal abdominal lymphatic perfusion patterns and investigates their impact on serum protein readings. METHODS We performed a retrospective analysis of patients who underwent magnetic resonance imaging with T2-weighted lymphatic imaging and serum protein measurements 6 months after having Fontan surgery. The abdominal lymphatic images were classified according to the anatomical lymphatic drainage patterns into 2 categories: (1) para-aortic (types 1–4); (2) portal-venous (types 1–3). Thoracic lymphatic images were classified (types 1–4) as described earlier. RESULTS A total of 71 patients were included in the study. Para-aortic lymphatic perfusion patterns were classified as type 1 in 4, type 2 in 13, type 3 in 37 and type 4 in16 out of 71 patients. Portal-venous lymphatic perfusion patterns were classified as type 1 in 20, type 2 in 10 and type 3 in 41 patients. Thoracic lymphatic perfusion patterns were classified as type 1 in 8, type 2 in 11, type 3 in 39 and type 4 in 13 patients. The serum protein level was 66 (interquartile range: 7.5) g/l (< standard value in 37%). Higher-grade para-aortic (p = 0.0062), portal-venous (p = 0.022) and thoracic (p = 0.011) lymphatic abnormalities were correlated with lower total serum protein levels. Higher ratings of para-aortic lymphatic abnormalities were significantly associated with higher ratings of portal-venous abnormalities (p < 0.0001). Ratings of para-aortic and portal-venous classifications were correlated with the thoracic classification (p < 0.001). CONCLUSIONS Abnormal abdominal lymphatic perfusion patterns can be classified according to anatomical structures with increasing severity. Higher grade abdominal and thoracic lymphatic perfusion patterns are associated with lower serum protein values.

Background: Reliable laboratory parameters identifying complications after Fontan surgery including the lymphatic abnormalities and the development of protein-losing enteropathy (PLE) are rare. Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocte ratio (PLR) are inflammatory markers and have been studied to predict outcome and prognosis in various diseases. The aim of this study was to investigate NLR and PLR from birth to follow-up after Fontan and evaluate their use as prognostic parameters for single ventricle patients regarding the development of lymphatic malformations during follow-up.Materials and Methods: Sixty-six univentricular patients who underwent Fontan surgery and had 6-month follow-up magnetic resonance imaging (MRI) with T2 weighted lymphatic imaging after total cavopulmonary connection (TCPC) surgery were included in the study. NLR and PLR were determined at specific time points, from neonatal age to follow-up after Fontan operation and correlated to data from the MRI 6 months after Fontan.Results: NLR and PLR increase significantly over time from the first surgery during infancy to the follow-up after Fontan (both p < 0.0001), with a significant increase after the Glenn surgery for both ratios (each p < 0.0001). Higher NLR (p = 0.002) and higher PLR (p = 0.004) correlated with higher-grade classification of lymphatic abnormalities in T2-weighted imaging 6 months after Fontan surgery and higher NLR correlated with higher transpulmonary gradient prior to Fontan surgery (p = 0.035) Both ratios showed a significant correlation to total protein at follow-up (NLR p = 0.0038; PLR<0.0001).Conclusion: Increased NLR and PLR correlate with higher degree lymphatic malformations after TCPC and therefore might contribute as valuable additional biomarker during follow-up after TCPC. NLR and PLR are simple, inexpensive and easily available parameters to complement diagnostics after TCPC.

Lymphatic congestion in single-ventricle patients has been associated with increased morbidity and poor outcomes. Little is known about the dynamics of lymphatic abnormalities over time, on their association with clinical presentation or response to catheter interventions. This retrospective, single-center study describes Fontan patients who underwent at least two magnetic resonance imaging (MRI) studies. T2-weighted lymphatic imaging was used to classify thoracic and abdominal (para-aortic and portal-venous) lymphatic abnormalities. The relationship between lymphatic congestion and hemodynamic changes after cardiac catheter interventions, clinical presentation and MRI data was analyzed. A total of 33 Fontan patients underwent at least two cardiac MRI studies. Twenty-two patients had two, eight had three and three had four lymphatic imaging studies (total of 80 MRIs studies). No significant changes in lymphatic classification between MRI 1 and 2 were observed for thoracic (p = 0.400), para-aortic (0.670) and portal-venous (p = 0.822) abnormalities. No significant correlation between lymphatic classification and hemodynamic changes after intervention or MRI parameters was found. This study illustrates thoracic and abdominal lymphatic abnormalities in serial T2-weighted imaging after Fontan. Fontan patients did not demonstrate significant changes in their lymphatic perfusion, despite clinical or hemodynamic changes. We assume that lymphatic congestion might develop after total cavopulmonary connection (TCPC) and remain relatively stable, despite further intervention targeting hemodynamic parameters.