Protein-losing enteropathy (PLE) is a rare and complicated disorder regarding the loss of proteins in the intestines. The signs and symptoms of PLE include bloating, diarrhea, edema, pleural effusions, ascites, and failure to thrive.
In some situations, PLE can occur after the Fontan procedure. The Fontan procedure is typically the third surgery for patients with hypoplastic left heart syndrome (HLHS) that reduces the amount of work of the right ventricle. During this procedure, the surgeon creates a path for the oxygen-poor blood in one of the blood vessels that returns blood to the heart (the inferior vena cava) to flow directly into the pulmonary arteries. The pulmonary arteries then transport the blood into the lungs. This procedure allows the rest of the oxygen-poor blood returning from the body to flow to the lungs. After this procedure, there's no mixing of oxygen-rich and oxygen-poor blood in the heart.
Currently, there is no single treatment for PLE but rather multiple treatments used together to decrease the loss of proteins. These treatments can include a variety of options including dietary changes, medications, and sometimes surgical or catheter-based interventions. Talk to your doctor or care team for more information.
PLE does not exclude participation in research studies here at the Todd and Karen Wanek Family Program for HLHS. Email the program at HLHS@mayo.edu to find out more.
The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. To learn more or to participate in the research, email HLHS@mayo.edu and follow the HLHS program on Facebook, Twitter and Instagram.