Author + information
- Received January 7, 2017
- Revision received March 31, 2017
- Accepted April 4, 2017
- Published online June 12, 2017.
- Maxim Itkin, MDa,∗ (, )
- David A. Piccoli, MDb,
- Gregory Nadolski, MDa,
- Jack Rychik, MDc,
- Aaron DeWitt, MDc,
- Erin Pinto, MSN, RNa,
- Jonathan Rome, MDc and
- Yoav Dori, MD, PhDa
- aCenter for Lymphatic Imaging and Interventions, Children’s Hospital of Philadelphia/Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
- bDivision of Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
- cDivision of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
- ↵∗Address for correspondence:
Dr. Maxim Itkin, Center for Lymphatic Imaging and Interventions, Hospital of University of Pennsylvania, 3400 Spruce Street, Philadelphia, Pennsylvania 19104.
Background Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected.
Objectives The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE.
Methods This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE.
Results Liver lymphangiography was performed in 8 patients (5 males, 3 females; median age, 21 years), 7 of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in 6 of 7 patients. Liver lymphatic embolization with ethiodized oil in 2 patients resulted in a temporary increase in albumin blood level and symptom improvement in 1 patient, but was complicated by duodenal bleeding in both patients. Of the remaining 6 patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in 3 patients, temporary improvement in 2 patients, and no change in 1 patient with median follow-up of 135 days (range, 84 to 1,005 days).
Conclusions The authors demonstrated liver lymph leakage as a cause of PLE in patients with congenital heart disease and elevated central venous pressure. Lymphatic embolization led to improved albumin levels and relief of symptoms. Further experience with the technique is needed to determine long-term outcome of this procedure.
Dr. Itkin has received grant funding from Guerbet, LLC. Dr. Nadolski has received grant funding from Teleflex Medical and Guerbet, LLC. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received January 7, 2017.
- Revision received March 31, 2017.
- Accepted April 4, 2017.
- 2017 American College of Cardiology Foundation