Intrahepatic Dynamic Contrast‐Enhanced Magnetic Resonance Lymphangiography: Potential Imaging Signature for Protein‐Losing Enteropathy in Congenital Heart Disease

Abstract

Background Protein‐losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast‐enhanced magnetic resonance lymphangiography (IH‐DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH‐DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single‐center retrospective study of IH‐DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH‐DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH‐DCMRL than patients without PLE ( P <0.001). Conclusions IH‐DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH‐DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk‐assessment tool for PLE in at‐risk patients.

Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome

“…Protein losing enteropathy is the most common lymphatic flow disorder in the abdomen and affects 5%-12% (40, 41) of Fontan patients. Intrahepathic and intramesenteric dynamic contrast magnetic resonance lymphangiography recently demonstrated abnormal increased lymphatic flow from the liver lymphatics (Figures 4A,B) or to a lesser extend from the mesenterial lymphatics (Figures 4C,D) to the duodenum in these patients (42,43). Duodenal wall edema, inflammation and lymphangiectasia is commonly seen on endoscopy and even holes formed in the duodenal wall have been demonstrated in later stages in these patients (25).…”

“…The three main access points are intranodal for the central lymphatics, intrahepatic for hepato-systemic lymphatics and intramesenteric whose role is not well-defined yet. It can even be done in very small infants that weight approximately 3 kg (Figures 4A-E) (42,49,50). In patients where multiple compartments are involved all three access points can be investigated at the same time.…”

Serial T2-Weighted Thoracic and Abdominal Lymphatic Imaging in Fontan Patients—New Insights into Dynamics of Lymphatic Abnormalities after Total Cavopulmonary Connection

“…However, not all patients with a Fontan circulation develop severe lymphatic abnormalities and it is hypothesized that the physiologic changes in venous and lymphatic congestion, plus an anatomical variant of the lymphatic vasculature, might lead to lymphatic failure in some of those patients [ 14 ]. This would explain why some patients with high CVP do not develop lymphatic failure and some who seem to have favorable hemodynamics might show severe lymphatic dysfunction [ 2 , 3 , 15 , 16 ]. In our cohort, we did not identify any correlation between ventricular dysfunction or hemodynamic parameters with lymphatic classification.…”

“…T2-weighted imaging has become increasingly important for single-ventricle patients and represents a ‘game changer’ to screen patients with Fontan failure, including PLE and plastic bronchitis. However, new modalities have been described to illustrate the lymphatic perfusion, including nodal, hepatic and mesenteric dynamic contrast lymphangiography (DCMRL) [ 11 , 16 , 18 ]. DCMRL allows for a full characterization of lymphatic perfusion, including physiology, anatomy, and flow of the lymphatic system.…”

Lymphatic Disorders in Patients With Single Ventricle Heart Disease

“…In a recent manuscript, Smith et al showed the correlation between the different hepatic lymphatic flow patterns and systemic lymphatic diseases (16). In addition, Lemley et al demonstrated that all patients with PLE have duodenal involvement with IH-DCMRL (17). In contrast, most patients without PLE do not have duodenal involvement with hepatic injection.…”