Steroid pulse therapy for protein-losing enteropathy after the Fontan operation

Area of Science:

• Cardiology
• Gastroenterology
• Pediatric Medicine

Background:

• Fontan circulation is a complex surgical palliation for single-ventricle congenital heart disease.
• Protein-losing enteropathy (PLE) is a serious complication of Fontan circulation, leading to malnutrition and increased morbidity.
• Standard treatments for PLE, including low-dose steroids and anticoagulation, can be ineffective.

Observation:

• A 19-year-old male with Fontan circulation presented with PLE secondary to acute enteritis.
• Conventional therapies, including subcutaneous heparin and oral prednisolone, failed to resolve the condition.
• Central venous pressure was within the normal range, complicating the diagnostic and therapeutic approach.

Findings:

• Intravenous high-dose methylprednisolone pulse therapy (15 mg/kg/day for 3 days) followed by oral prednisolone (0.5 mg/kg/day for 4 days) was administered.
• This regimen was repeated after 2 weeks.
• Serum protein and albumin levels normalized within 2 months post-treatment.

Implications:

• High-dose intravenous methylprednisolone pulse therapy represents a potentially effective treatment strategy for refractory protein-losing enteropathy in Fontan patients.
• This approach achieved long-term remission (2 years) without the need for maintenance steroid therapy.
• Further research is warranted to elucidate the mechanisms and long-term outcomes of this intensive pulse therapy in similar patient populations.