Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy

BackgroundProtein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation.MethodsTwo hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5 years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records.ResultsWithin the median follow-up duration of 5 years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2 years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2 years (range 0.21-7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p = 0.0019), central venous pressure at postoperative 12 hours (p = 0.0026), days of ICU stay (P = 0.0449), days of hospitalization (p = 0.0135), and days of chest tube indwelling (p = 0.0493). Multivariate analysis, however, showed that only Cpv (p = 0.0367) remained significant. The range of Cpv was 8.8-26.1 mm2/m2/mmHg (median 17.9) in patients with PLE, and 6.6-122.3 mm2/m2/mmHg (median 26.8) in patients without PLE.ConclusionsLow pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.