Cardiology in the Young

Review Article

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

Jeffery Meadowsa1 c1 and Kathy Jenkinsa2a3

a1 Division of Pediatric Cardiology, University of California, San Francisco, California, United States of America

a2 Department of Cardiology, Children's Hospital, Boston, Massachusetts, United States of America

a3 Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, United States of America


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.

(Received September 16 2010)

(Accepted December 23 2010)

(Online publication February 25 2011)


c1 Correspondence to: J. Meadows, MD, Division of Pediatric Cardiology, 505 Parnassus Avenue, Box 0632, San Francisco, California 94143-4144, United States of America. Tel: 415 476 4904; Fax: 415 476 3112; E-mail: