Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

Abstract

Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.

Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

“…This secondary disorder is characterized by profound loss of serum proteins into the gastrointestinal lumen caused by enteric mucosal injury, systemic inflammation, and venous or lymphatic obstruction [4]. The relative infrequency of PLE, combined with our rudimentary knowledge of the interplay between the cardiovascular and lymphatic systems, has hindered understanding of the pathophysiologic processes underlying this disease, as well as the development of effective therapy.…”

“…Transplantation is often the only reasonable option [5,6]. Historically, survival after diagnosis has been 50% at 5 years [4,7]. Nevertheless, a recent study reported 88% and 72% survival rates at 5 and 10 years, respectively, after diagnosis.…”

“…This causes increased lymph production, for which the lymphatic pump cannot compensate. In addition, chronic elevation of CVP retards lymphatic return to the central venous system, thus unbalancing lymphatic homeostasis [2][3][4][5]. The procedure of thoracic duct decompression was introduced to unburden the thoracic duct and increase the transport capacity of the lymphatic system.…”

“…This will unbalance lymphatic homeostasis, leading to fluid accumulation [2,3]. Lymphatic insufficiency has a central role in the disease process of 1 of the most devastating complications of Fontan procedures-protein-losing enteropathy (PLE) [4,5]. To improve lymphatic drainage, a new surgical concept based on decompression of the thoracic duct to the lower pressure levels of the systemic atrium was recently introduced [3].…”

Nutritional Approach of Pediatric Patients Diagnosed with Congenital Heart Disease

“…Th erapeutic measures in PLE are as follows: non-specifi c medical therapy, consisting in diets high in protein and low in fat, anti-congestive cardiovascular medical regimens, periodic albumin infusions and specifi c medical therapy, including the use of unfractionated heparin, corticosteroids, transcatheter therapy (dilation and/or stenting of obstructions in blood fl ow, coil embolisation of signifi cant aorto-pulmonary collaterals, fenestration of the circuit), heart transplant. Despite improvement in therapies used in Fontan patients with PLE, their prognosis has not signifi cantly improved and mortality related to PLE remains high [37].…”