An Early Glenn Operation May be Associated with the Later Occurrence of Protein-Losing Enteropathy in Fontan Patients: Association of Early Glenn and Failing Fontan

Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5–8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group ( n = 14) and a non-PLE/PB group ( n = 92). Prevalence of PLE was 9.4% ( n = 10) and of PB 3.8% ( n = 4). At follow-up, five patients (4.7%) died of PLE or PB. Median age at death was 6.2 years (IQR 10.5, 95% CI 5.3–23.4). We observed no significant group differences in gender distribution ( p = 0.73), ventricular morphology ( p = 0.87), surgical technique ( p = 0.64), conduit fenestration ( p = 0.34), age at Fontan operation ( p = 0.54), and need for diuretics ( p = 0.56). Hypoplastic left heart syndrome was more frequent in the PLE/PB group 50 vs. 22.8% ( p = 0.03) OR 3.4 (95% CI 1.1–10.8). The modified Glenn procedure was performed at a median age of 4 months (IQR 4.0) in the PLE/PB group versus 8 months (IQR 8.0) in the non-PLE/PB group ( p = 0.01). The early Glenn procedure and hypoplastic left heart syndrome may be associated with the development of PLE and PB.