Protein-losing enteropathy after the Fontan operation: associations and predictors of clinical outcome.

Objective: We sought to define what clinical parameters were related to the ultimate outcome in Fontan patients who had developed protein-losing enteropathy (PLE).
Background: PLE is a serious complication of the Fontan operation. Several preoperative and perioperative findings are associated with later PLE. However, there is limited information regarding postoperative abnormalities contributing to or influencing outcome in PLE.
Methods: We evaluated 44 consecutive Fontan patients with PLE. A matched control group of Fontan patients without PLE was used for comparison (matched-pair analyses). Kaplan-Meier and Cox proportional hazard methods were used for survival analyses.
Results: Median age was 18 years (range 4-48 years). Short-axis and apical fractional area change (Delta FA) were less in PLE patients than in controls (50 vs. 57% [P < .0001] and 49 vs. 54% [P = .01]). Five and 10-year actuarial survival rates for the PLE group were 49 +/- 9% and 30 +/- 11%. Deceleration time <120 milliseconds (hazard ratio [HR] = 9.2, P = .04), New York Heart Association classification III or IV (HR = 4.0, P = .01), and lower serum albumin (HR = 0.30, P = .04) were independent predictors of mortality in those with PLE.
Conclusion: Mild reduction in Delta FA was the only late echocardiographic finding associated with development of PLE. However, short deceleration time, poor New York Heart Association (NYHA) class, and low serum albumin identify a group of patients at the greatest risk for death. Presence of these findings in a PLE patient should lead to aggressive management strategies and may warrant early consideration of transplantation.