OBJECTIVE: It was thought that congenital heart disease (CHD) may be part of DiGeorge syndrome (DGS) and that the susceptibility to infection in children with CHD was related to immonodeficiency. This study explores whether immunodeficiency is present in children with CHD and aims to clarify the relationship between CHD and DGS by reviewing published papers. METHODS: The sizes of thymus shadow on chest X ray film were measured in 72 neonates with simple CHD, 34 neonates with complex CHD (n= 34) and 50 neonates with pneumonia. The partial immunologic laboratory data, including lymphocyte subsets counts, the peripheral blood monoeuclear cell (PBMC) IL-4 and IFN-7 mRNA expressions and production in culture supernatant, the PBMC proliferative response to phytahematoagglutinin (PHA) or lipopolysaccharide (LPS), and plasma IgG, IgA, IgM and complement 3 (C3) levels' were measured in 28 pre-school children with CHD and 20 age-matched healthy children. RESULTS: The thymus shadows on chest X-ray films were found in both neonates with CHD and neonates with pneumonia. There was no difference in the sizes of the thymus shadow between them. The peripheral lymphocyte subsets counts in children with CHD did not differ from those in the healthy children. There were also no differences in the plasma IgG, IgA, IgM and C3 levels between them. The counts of per minute impulse (cpm) of PBMC induced by PHA and LPS and the PBMC IL-4 and IFN-γ mRNA expressions in children with CHD did not differ from those in the healthy children. CONCLUSIONS: Not all children with CHD have congenital thymus aplasia or immunodeficiency. It is not certain that primary immunodeficiency is the reason why children with CHD may be prone to infection.
Abstract:OBJECTIVE: It was thought that congenital heart disease (CHD) may be part of DiGeorge syndrome (DGS) and that the susceptibility to infection in children with CHD was related to immonodeficiency. This study explores whether immunodeficiency is present in children with CHD and aims to clarify the relationship between CHD and DGS by reviewing published papers. METHODS: The sizes of thymus shadow on chest X ray film were measured in 72 neonates with simple CHD, 34 neonates with complex CHD (n= 34) and 50 neonates with pneumonia. The partial immunologic laboratory data, including lymphocyte subsets counts, the peripheral blood monoeuclear cell (PBMC) IL-4 and IFN-7 mRNA expressions and production in culture supernatant, the PBMC proliferative response to phytahematoagglutinin (PHA) or lipopolysaccharide (LPS), and plasma IgG, IgA, IgM and complement 3 (C3) levels' were measured in 28 pre-school children with CHD and 20 age-matched healthy children. RESULTS: The thymus shadows on chest X-ray films were found in both neonates with CHD and neonates with pneumonia. There was no difference in the sizes of the thymus shadow between them. The peripheral lymphocyte subsets counts in children with CHD did not differ from those in the healthy children. There were also no differences in the plasma IgG, IgA, IgM and C3 levels between them. The counts of per minute impulse (cpm) of PBMC induced by PHA and LPS and the PBMC IL-4 and IFN-γ mRNA expressions in children with CHD did not differ from those in the healthy children. CONCLUSIONS: Not all children with CHD have congenital thymus aplasia or immunodeficiency. It is not certain that primary immunodeficiency is the reason why children with CHD may be prone to infection.