Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4–13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this “non-invasive” treatment should be considered as long term palliation or bridge to cardiac transplantation.
- adult congenital heart disease
- protein losing enteropathy
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.