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Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation
Gianluca Brancaccio1, Adriano Carotti, Patrizia D'Argenio
1Cardiac Pediatric Surgery Department of Hospital Bambino Gesù, Rome, Italy. gbrancaccio70@hotmail.com
Insights
Protein-losing enteropathy (PLE) after Fontan procedure can be reversed with heart transplantation and bowel rest via total parenteral nutrition. This case study shows long-term recovery and normal albumin levels post-transplant.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatric Surgery
Background:
- Protein-losing enteropathy (PLE) is a severe complication following the Fontan procedure, affecting 4-13% of patients.
- Medical therapies for post-Fontan PLE have shown limited success.
Observation:
- A 14-year-old boy with Fontan circulation presented with persistent PLE despite medical treatment.
- Following heart transplantation, protein loss continued, necessitating intervention.
Findings:
- Total parenteral nutrition was initiated to provide bowel rest.
- Gradual reduction in protein loss was observed over 16 months.
- The patient achieved normal serum albumin levels and remains well 5 years post-heart transplantation.
Implications:
- Heart transplantation may offer a viable treatment option for refractory post-Fontan PLE.
- Bowel rest through total parenteral nutrition can aid in the reversal of protein loss.
- Successful management of PLE post-transplant leads to improved long-term outcomes in Fontan patients.
Abstract:
Protein-losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4% to 13% of patients after the Fontan procedure. We report a case of PLE reversal after heart transplantation in a 14-year-old boy with Fontan circulation who previously was treated unsuccessfully with medical therapy. The protein loss continued after heart transplantation. We administered total parenteral nutrition to rest the bowel. After 16 months, we observed a gradual decrease in protein loss. The patient is doing well 5 years after heart transplantation and had has a normal serum albumin level.