Impaired elastic matrix development in the great arteries after ablation of the cardiac neural crest

The cells that form the aorticopulmonary septum in the avian embryo have been shown to be similar to the cells that form the walls of the great vessels in two ways: both are derived from the cardiac neural crest and both are able to synthesize an elastogenic matrix in the early embryo. Because of these similarities, and because ablation of the cardiac neural crest causes congenital defects of the outflow tract that are related to failure of proper septation, it was hypothesized that such an ablation also would cause the walls of the great vessels to be defective. The purpose of this study was to compare the elastic matrix in the mediae of the great vessels of normal embryos with those from which the cardiac neural crest had been ablated. The results show that the elastic matrix in the great vessels of the experimental embryos was impaired 1) in the rate of downstream propagation of the initiation of elastogenesis among younger embryos, incubation days 4-8 and 2) in the spatial configuration of the elastic matrix among the older embryos, incubation days 16-20. These results may provide a biological explanation for the elastin defect that affects the pulmonary artery of many patients with cyanotic congenital heart defects.