Protein-losing Enteropathy after the Total Cavopulmonary Connection: Impact of Intravenous Immunoglobulin

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OBJECTIVE: Despite multiple treatment modalities, protein-losing enteropathy remains a serious complication to Fontan-type operations. Observations suggest inflammation to be involved in the pathogenesis of this condition, and immunomodulating treatment with high-dose intravenous immunoglobulin may modify the condition positively. PATIENTS: Four patients with protein-losing enteropathy occurring after the total cavopulmonary connection, presenting with edema, hypoalbuminemia, and hypogammaglobulinemia, received intravenous immunoglobulin replacement therapy. INTERVENTIONS: Standard replacement dose (1 g/kg) was used with intervals between infusions adjusted according to albumin and gamma globulin levels. Treatment periods ranged from 1 year to 5.3 years. RESULTS: Intravenous immunoglobulin treatment was associated with significant increase in plasma albumin and to some extent in immunoglobulin G levels, as well as resolution of edema and the children started to thrive normally. During treatment, no serious infections or serious side effects were seen. Additional follow-up intervals ranged from 2 years to 2.8 years, during which only one episode of clinical relapse was registered and treated. CONCLUSIONS: We find the increase in albumin level and the resolution of protein-losing enteropathy symptoms after treatment with intravenous immunoglobulin of particular interest considering this serious complication to Fontan-type operations.

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