MRI of lymphatic abnormalities after functional single-ventricle palliation surgery.
OBJECTIVE
Protein-losing enteropathy (PLE) and plastic bronchitis are serious complications that occur after single-ventricle surgery. A lymphatic cause for these conditions has been proposed, but imaging correlation has not been reported. The objective of this study was to evaluate lymphatic abnormalities in patients after functional single-ventricle palliation compared with patients with non-single-ventricle congenital heart conditions using T2-weighted MR lymphangiography.


METHODS
We retrospectively reviewed imaging data from 48 patients who underwent T2-weighted MR lymphangiography in our institution between May 1, 2012, and October 24, 2012. The patients were divided into four groups: patients who underwent superior cavopulmonary connection, patients who underwent total cavopulmonary connection, patients with total cavopulmonary connection and lymphatic complications, and patients with non-single-ventricle cardiac anomalies.


RESULTS
There were 38 patients with single ventricles in this study. The lymphatic abnormalities observed in these patients included thoracic duct dilation greater than 3 mm (31%), lymphangiectasia and lymphatic collateralization (78%), and tissue edema (86%). There were five patients with PLE, one patient with plastic bronchitis, and one patient with chronic chylous effusions and superior cavopulmonary connection. The patients with PLE and plastic bronchitis had statistically significant larger thoracic duct maximal diameters (median, 3.9 mm; range, 3-7.2 mm) than did the other patients with total cavopulmonary connection (p < 0.01). In the two-ventricle patient group, there were no abnormal lymphatic findings.


CONCLUSIONS
Lymphatic abnormalities are found in many patients after functional single-ventricle palliation. T2-weighted unenhanced MRI is capable of anatomic assessment of the lymphatic system in this patient population and has promise for guiding treatment in the future.
1 Division of Cardiology and Department of Radiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104.
Yoav
Y
Dori
Marc S
MS
Keller
Mark A
MA
Fogel
Jonathan J
JJ
Rome
Kevin K
KK
Whitehead
Matthew A
MA
Harris
Maxim
M
Itkin
D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D021621:Imaging, Three-Dimensional; D007223:Infant; D044148:Lymphatic Abnormalities / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D010166:Palliative Care; D012189:Retrospective Studies
MR lymphangiography; chylous effusions; plastic bronchitis; protein-losing enteropathy; single ventricle