Protein-losing enteropathy due to abnormal lymph flow, treatable with embolization

Last Updated: 2017-06-19

By Anne Harding

NEW YORK (Reuters Health) - Protein-losing enteropathy (PLE) in patients with congenital heart disease is often due to liver lymph leakage, and can be treated with lymphatic embolization, new findings show.

Dr. Maxim Itkin of Children�€™s Hospital of Philadephia and colleagues report on eight PLE patients treated with lymphoangiographic imaging and liver lymphatic embolization in the June 20 issue of the Journal of the American College of Cardiology.

PLE occurs in up to 15% of patients with Fontan circulatory pattern, on average about eight years after Fontan surgery. Patients lose protein into the gastrointestinal tract, and develop hypoalbuminuria, peripheral edema and ascites. Current treatment aims to reduce inflammation, venous pressure and lymph formation, and 10-year mortality is about 30%.

Dr. Itkin and his team previously demonstrated that plastic bronchitis is due to abnormal lymphatic flow and can be repaired with lymphatic embolization. The new report confirms that a similar mechanism is involved in PLE.

Seven of the eight patients had leakage of liver lymph into the duodenum on lymphangiography. Two patients underwent liver lymphatic embolization with ethiodized oil. These patients had a temporary rise in blood albumen, and one had symptom improvement, but duodenal bleeding developed in both patients.

The remaining six patients received liver lymphatic embolization with n-butyl cyanoacrylate glue. Three showed sustained improvement in serum albumin and symptoms, two had temporary improvement, and one patient had no change.

�€œSpecifically in these children with PLE or plastic bronchitis, these children have abnormal lymphatics that point in the wrong direction in their lungs or the wrong direction from liver to duodenum, and then when they have a heart condition where the flow of the liver lymph is enormous . . . it overflows these abnormal lymphatics,�€ Dr. Itkin explained.

With a procedure to block off lymphatic flow, which can be repeated, �€œthere�€™s much more hope for these children,�€ he said. �€œWe can temporize the condition for a longer period of time.�€

Dr. Larry Latson, who wrote an accompanying editorial, told Reuters Health by email, �€œSome, and perhaps many, patients with protein-losing enteropathy may have a localized lymphatic leak rather than diffuse leakage of protein throughout the gastrointestinal tract.�€

�€œThis mechanism is best identified by advanced imaging techniques that require a coordinated study with an interventional radiologist and gastroenterologist,�€ said Dr. Latson, of Joe DiMaggio Children�€™s Hospital and Memorial Healthcare System in Hollywood, Florida.

�€œA localized leak may be amenable to blockage of only the leaking vessel instead of using multiple drugs aimed at reducing inflammation throughout the gastrointestinal tract,�€ he said, adding that such medications also have �€œsignificant serious side effects.�€

�€œSome patients with PLE are candidates for heart transplantation,�€ Dr. Latson said. �€œThe PLE improves in many of the patients after heart transplantation, but not all. Localized leakage of lymphatic fluid may explain why heart transplantation alone does not cure all of the patients.�€

SOURCE: http://bit.ly/2sKRbAO and http://bit.ly/2rIH6EI

J Am Coll Cardiol 2017.