Elastin and
collagen concentrations were determined in intimal-medial samples of ascending aortas from healthy controls of different ages and from 20 patients with
annuloaortic ectasia (AAE). Five patients had the
Marfan syndrome. In controls the highest
elastin concentrations (estimated from
desmosine concentrations or insoluble residues after hot-
alkali extraction) were found in children. During aging until 60 years,
elastin concentration decreased when determined by the hot-
alkali extraction method while
desmosine concentration changed less. Aorta samples from the
Marfan-syndrome patients showed a great variation of
elastin concentration from total lack to normal values. Samples from the other AAE patients could be divided into two groups. One contained clearly less
elastin and more
collagen than the controls whereas in the other group this difference was less marked. Histological examination of the aortic wall of the first group also showed marked
fibrosis accompanied by severe
elastin fragmentation and acellularity. From the 15 non-Marfan patients 14 were men. By means of clinical examination these patients could also be divided into "familial" and "nonfamilial" groups, because increased diameter of the aortic root was found in relatives of almost half of the patients. However, there were no differences in
elastin and
collagen concentrations between the familial and nonfamilial cases. As well, no correlation was found between biochemical findings and diameters of the aortic roots. These results point to altered
elastin and/or
collagen metabolism in the aortic wall of AAE patients.