Protein Losing Enteropathy

Protein losing enteropathy is a condition where there is a loss of the valuable proteins and nutrients in the intestinal lumen. This condition is often occurring in patients with congenital heart disease, especially in patients who were born with single ventricle and underwent Fontan surgery.

The clinical presentation in these patients are hypoalbunemia, diarrhea, tissue edema, ascites and pleural effusions. The level alpha-1-antitrypsin in stool is often elevated. The lymphatic cause of these disease has been suspected due to presence of the dilation of the intestinal submucosal lymphatic ducts, condition that is called intestinal lymphangiectasia.

Recently, we discovered one of the mechanisms of the lymphatic leaks in patients with heart disease (reference). In these patients, the elevated central venous pressure results in liver congestion, that in turn results in increase of the lymphatic production. Our group performed liver lymphangiography in these patients and discovered that there is a leakage of the lymphatic lymph into the intestinal lumen (Figure). Embolization of the liver and retroperitoneal lymphatic ducts with n-BCA glue can resulted in resolution of the patient’s symptoms (reference).

In patients with PLE we initially perform liver lymphangiography and in selected patients DCMRL, following by liver lymphatic embolization. The procedure is often performed in conjunction with upper endoscopy in order to confirm the location of the leakage point in the duodenum, by injecting of the blue dye in the liver lymphatic system (Figure).