Patients with single-ventricle heart disease, a congenital defect in which the heart has only one functional chamber, must often undergo corrective surgery to repair the heart. However, a rare complication of the heart surgery is the leakage from the liver’s lymphatic system, which can be life-threatening.
Protein-losing enteropathy (PLE) is a condition in which a patient suffers a dramatic loss of proteins into the intestine. Researchers at Children’s Hospital of Philadelphia (CHOP) and Penn Medicine have now published a paper in the Journal of the American College of Cardiology which provides a better understanding of the condition, and offers a possible treatment.
“We are learning that more and more conditions are caused by leaks in the lymphatic system,” said Dr. Maxim Itkin, co-author of the study and co-director of the Center for Lymphatic Imaging and Interventions at CHOP and Penn Medicine. “After identifying the leakage site in a lymphatic vessel, the team intervenes, using a technique called lymphatic embolization. Through a small needle, we can block the abnormal flow with a variety of tools – endovascular glue, coils, iodized oil – based on an individual patient’s needs.”
While doctors aren’t sure exactly what causes PLE, the new research supports the conclusion that the abnormal lymphatic circulation between the small intestine and the liver likely plays an important role. Using lymphatic imaging and a procedure to repair the technique, the researchers have developed a new way to help improve symptoms in these patients.
“Patients with devastating side effects of the single-ventricle palliation process, such as protein-losing enteropathy, endure frequent trips to the bathroom, and their bodies lose essential protein,” said Dr. Yoav Dori, co-author of the study and co-director of the Center for Lymphatic Imaging and Interventions at CHOP and Penn Medicine. “We have long suspected the lymphatic system was involved and can now begin to provide hope for these patients.”
The researchers used a retrospective review of medical records for eight patients – including six pediatric patients – with congenital heart disease. The patients all suffered from PLE and were treated using interventions following lymphatic imaging.
The researchers found that the primary cause of PLE in these cases – and likely in most patients with congenital heart disease – was leakage of the liver lymph into the first portion of the small intestine, known as the duodenum. Lymphatic congestion within the liver was also identified as a potential causative agent for PLE.
Since the retrospective study focused on a small patient population, these initial findings will need to be confirmed through larger trials. “Further research and experience with the technique is needed to improve long-term outcomes of this procedure,” said the researchers.