This study is phase 2 open-label, single-arm, multi-center clinical trial to evaluate the efficacy and safety of Camostat mesylate in patients with Protein-losing enteropathy after Fontan operation.

Inclusion Criteria:

• history of protein-losing enteropathy after Fontan operation

  • more than 6 months after Fontan operation
  • more than 3 months of protein-losing enteropathy history (ascites, edema, diarrhea, etc) and less than 3.0 mg/dL of serum albumin without evidence of renal or liver disease

Exclusion Criteria:

• inability to take oral camostate mesylate
• hypersensitivity to camostate mesylate
• patients with taking similar medication or prohibited combination drug
• patients participating in other clinical trials
• patients with diet limitation
• patients with genetic disease such as galactose intolerance, Lapp lactase deficiency, glucose-galactose malabsorption