Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation

Study Questions:

What are the contemporary outcomes of patients with protein-losing enteropathy (PLE) following the Fontan operation?


A retrospective review was performed at a single center. From 1992-2010, 42 patients with PLE were identified. Patient characteristics including underlying cardiac anatomy, symptoms, medical therapy, echocardiogram findings, and hemodynamics were recorded.


The mean age at initial Fontan procedure was 10.1 ± 10.8 years, with mean age at PLE diagnosis of 18.9 ± 11.0 years. The mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years from diagnosis of PLE. Risk factors for decreased survival included high Fontan pressure (mean >15 mm Hg, p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis. Patients who died had less favorable hemodynamics as compared with survivors, with higher pulmonary vascular resistance (3.8 ± 1.6 Wood units vs. 2.1 ± 1.1 Wood units), lower cardiac index (1.6 ± 0.4 L/min/m2 vs. 2.7 ± 0.7 L/min/m2), and lower mixed venous saturation (53% vs. 66%; p = 0.01). Treatments used more frequently in survivors with PLE included spironolactone, octreotide, sildenafil, fenestration creation, and relief of Fontan obstruction.


The authors concluded that PLE remains difficult to treat, although survival has improved with advances in treatment.


Historical studies have demonstrated mortality rates approaching 50% in the first 5 years after diagnosis of PLE. This study shows significantly improved outcomes in the current era at a single center. Given different study methodologies, it is difficult to compare this study with those from the mid 1990s demonstrating higher mortality rates. In the current study, patients were older at diagnosis of PLE. It is possible that there was early attrition of the sicker patients, leading to improved survival of this older group. Additionally, the patient group is not representative of patients currently undergoing the Fontan procedure. More recently, the Fontan has been done in younger patients, including those with a differing array of underlying anatomy, potentially limiting the generalizability of this study to future patients with PLE.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement

Keywords: Survivors, Octreotide, Fontan Procedure, Purines, Ventricular Function, Risk Factors, Vascular Resistance, Piperazines, Spironolactone, Protein-Losing Enteropathies, Sulfones, Hemodynamics

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