Protein-losing enteropathy after the total cavopulmonary connection: impact of intravenous immunoglobulin.

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.