Protein-losing enteropathy after the Fontan operation

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Gastroenterology

Background:

• The Fontan operation is a palliative procedure for complex single-ventricle congenital heart defects.
• Protein-losing enteropathy (PLE) is a known but serious complication following the Fontan procedure.
• Understanding the incidence and risk factors for PLE is critical for patient management.

Purpose of the Study:

• To determine the frequency and severity of protein-losing enteropathy (PLE) in patients after the Fontan operation.
• To identify risk factors associated with the development of PLE.
• To evaluate survival rates after PLE diagnosis.

Main Methods:

• Retrospective analysis of 427 patients who survived at least 30 days post-Fontan operation (1973-1987).
• Assessment of PLE development, cumulative risk, and survival post-diagnosis.
• Correlation of hemodynamic parameters and perioperative factors with PLE incidence.

Main Results:

• Protein-losing enteropathy developed in 47 of 427 patients (11%).
• The 10-year cumulative risk of PLE was 13.4%; 5-year survival after diagnosis was 46%.
• Associated factors included increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, ventricular anatomy, preoperative ventricular end-diastolic pressure, longer bypass time, longer hospital stay, and postoperative renal failure.

Conclusions:

• Protein-losing enteropathy is a significant long-term complication of the Fontan procedure with poor survival.
• Careful patient selection for the Fontan operation is essential.
• Certain perioperative factors may predispose patients to developing PLE, necessitating vigilant management.