Secondary Combined Immunodeficiency on Pediatric Patients after the Fontan Operation: Three Case Reports

作者：Eva Avackova; Liska Martin; Jicinska Hana; Navratil Jiri; Litzman Jiri^*

来源：International Archives of Allergy and Immunology, 2016, 170(4): 251-256.

DOI：10.1159/000449163

• Fontan operation
• Protein-losing enteropathy
• Secondary combined immunodeficiency
• Hypogammaglobulinennia
• CD4(+) lymphocytopenia

The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by uni-ventricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4(+) lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation. The severity of hypogammaglobulinemia correlated with the nature of the infectious complications; however, clinical manifestations of T cell deficiency such as severe viral or opportunistic infections were not observed. The clinical consequences of the secondary combined immunodeficiency were modified by immunoglobulin replacement treatment and antibiotic prophylaxis. Heart transplantation led to the resolution of PLE signs and the restitution of IgG levels in 1 transplanted patient. Our experience shows that the immunological follow-up was delayed in all 3 patients. We suggest that all patients should be followed regularly by a clinical immunologist after the Fontan surgery.

• 出版日期2016