Protein-Losing Enteropathy in Patients With Congenital Heart Disease

Abstract 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 ly...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 2017-06, Vol.69 (24), p.2929-2937
Main Authors: Itkin, Maxim, MD, Piccoli, David A., MD, Nadolski, Gregory, MD, Rychik, Jack, MD, DeWitt, Aaron, MD, Pinto, Erin, MSN, RN, Rome, Jonathan, MD, Dori, Yoav, MD, PhD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Albumin
Bleeding
Bronchitis
Cardiology
Cardiovascular
Cardiovascular disease
central venous pressure
Child
Child, Preschool
Congenital diseases
Coronary artery disease
Duodenum
Edema
Embolization
Embolization, Therapeutic - methods
Endoscopy
Female
Females
Follow-Up Studies
Heart
Heart Defects, Congenital - complications
Heart diseases
Heart failure
Hepatology
Humans
Internal Medicine
Intestine
Leakage
Liver
liver lymphangiogram
Lymph
Lymphangiography
lymphatic embolization
Lymphatic system
Lymphography
Male
Males
Middle Aged
n-BCA
Patients
Pediatrics
Pressure
Protein-Losing Enteropathies - diagnosis
Protein-Losing Enteropathies - etiology
Protein-Losing Enteropathies - surgery
Proteins
Retrospective Studies
Serum albumin
Treatment Outcome
Young Adult
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Summary:Abstract 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.
ISSN:0735-1097
1558-3597
DOI:10.1016/j.jacc.2017.04.023