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Protein-losing enteropathy after the Fontan operation
R H Feldt1, D J Driscoll, K P Offord
1Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA.
Insights
Protein-losing enteropathy is a serious complication after the Fontan operation. Careful patient selection and management of perioperative factors are crucial to reduce its incidence and improve outcomes.
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.
Abstract:
Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hemodynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure. Medical management of protein-losing enteropathy was only partially successful. Statistical analysis has shown that factors related to protein-losing enteropathy were ventricular anatomy, increased preoperative ventricular end-diastolic pressure, longer operative bypass time, increased length of hospital stay, and postoperative renal failure. This study suggests that scrupulous selection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.