Abstract
DiGeorge Syndrome (DGS) consists of the association of CHD, immunodeficiency, and hypoparathyroidism. Since recent data suggest that DGS is more common than usually appreciated, we prospectively tested immune function during the first 3 months of life in 22 infants referred for evaluation of CHD and 7 age-matched controls. CHD patients as a group had T-cell rosettes (TCR) lower (mean ± SE = 48% ± 5) than age-matched controls (64% ± 4) or older normals (71% ± 2). Eight of the 22 CHD infants had TCR below 2 standard deviations from the mean for age-matched controls (<40%). Six of 21 CHD infants had low peak phytohema-glutinin (PHA) responses (<40,000 cpms); however the mean peak PHA response for CHD patients (78,000 cpms) was not different from age-matched controls (86, 100 cpms) or older normals (98,000 cpms). All CHD patients had B-cell numbers comparable to controls (8-20%). When grouped by cardiac abnormality, 8 of 12 infants who had lesions previously described in DGS (interrupted aortic arch, aortic atresia/hypoplasia, truncus arteriosus, tetralogy of Fallot, VSD), had low TCR or PHA, with 4 of the 8 having both low TCR and PHA. In contrast, 4 of 10 infants with CHD not associated with DGS had some evidence of immunodeficiency, with boarderline low TCR only in 3 of the 4.
These findings suggest that about 75% of patients with congenital heart disease, especially those with aortic arch abnormalities or truncus arteriosus, may have laboratory evidence indicative of depressed immune function.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Barrett, D., Drummond, W., Keil, E. et al. 899 IMMUNODEFICIENCY IN CONGENITAL HEART DISEASE (CHD). Pediatr Res 15 (Suppl 4), 592 (1981). https://doi.org/10.1203/00006450-198104001-00924
Issue Date:
DOI: https://doi.org/10.1203/00006450-198104001-00924