Protein-losing enteropathy following Fontan completion: A 30-year national cohort study in Sweden

To characterize patients with protein-losing enteropathy (PLE) diagnosed before age 18 years within a nationwide Swedish Fontan cohort.

Surgical records and medical charts were reviewed for all patients born after January 1, 1993, who underwent Fontan completion before January 1, 2021.

Among 573 reviewed patients, 28 (4.8%) developed PLE. The median time to onset of PLE after Fontan was 1.5 years (interquartile range, 0.5-5.5 years). Right ventricular morphology was associated with PLE (odds ratio, 2.3; 95% confidence interval, 1.04-5.0). Twenty-three patients (82%) received PLE-directed therapies, including pulmonary vasodilators (n = 17; 61%), subcutaneous heparin (n = 14; 50%), and/or long-term corticosteroids (n = 13; 46%). Eleven patients (39%) underwent a total of 18 catheter interventions, including 1 fenestration and 1 lymphatic intervention. Three pacemaker procedures were performed after a PLE diagnosis. Eight patients underwent heart transplant (26%). Patients with an early onset of PLE (<2 years from Fontan completion) frequently had a history of pulmonary venous congestion. Five-year overall survival was 88%, and 5-year transplant-free survival was 79%.

PLE remains a serious complication following Fontan, with notable mortality. Patients with right ventricular morphology of the systemic ventricle had a higher risk of PLE. Previous pulmonary venous congestion was common when PLE was diagnosed early after Fontan. Catheter-based interventions were frequently used to optimize hemodynamics; lymphatic interventions are likely to become more common. In therapy-refractory patients, heart transplantation remains an option.