Abstract 16061: Lymphatic Abnormalities in Patients With Congenital Heart Disease: New Insights From Non-Contrast Magnetic Resonance Lymphangiography
Background: Lymphatic abnormalities are thought to play a key role in 2 often fatal complications of Fontan physiology: protein losing enteropathy (PLE) and plastic bronchitis (PB). However, the prevalence of lymphatic abnormalities in this population is unknown and the lymphatic systems in these patients have never been directly visualized.
Methods: We reviewed imaging data for the presence of thoracic duct (TD) dilation, TD tortuosity, lymphangiectasia, lymphatic collateralization, and tissue edema from 91 patients who underwent T2 weighted (T2W) MRI lymphangiograms in our institution over the past year.
Results: There were 79 patients after single ventricle palliation in this study including 5 patients with PLE, 1 with PB, and 2 with chronic chylous effusions, all after superior cavopulmonary connection or total cavopulmonary connection palliation. Twelve patients had other forms of congenital heart disease. In the single ventricle group all patients had lymphatic abnormalities including significant TD dilation (30%), TD tortuosity (40%), lymphangiectasia (80%), lymphatic collateralization (80%), and tissue edema (90%). The patients with PLE and PB had more severe lymphatic abnormalities with larger TD maximal diameter (median 3.9 mm (range 3-7.2 mm) versus 2.3 mm (range 1.3-5.3 mm)) (p<0.01) and more TD tortuosity (p=0.016). In the non-single ventricle group there were 2 patients (17%), one with TOF and one with pulmonary hypertension, with abnormal lymphatic findings including lymphatic collateralization and mild thoracic duct (TD) dilation. Both patients had elevated RA pressure.
Conclusions: Direct visualization of the lymphatic system in patients with congenital heart disease is feasible. Lymphatic abnormalities appear common in patients after single ventricle palliation and are significantly more prominent in patients with PLE and PB. Our findings shed new light on the lymphatic system in these patients and warrant additional investigation.
- © 2013 by American Heart Association, Inc.