Assessment of site and severity in congenital aortic stenosis.

It has frequently been stated that congenital aortic valve stenosis cannot be distinguished with any confidence from discrete subvalvar aortic stenosis on clinical grounds, and that their separation rests on the results of hmmodynamic investigation and angiocardiography (Nadas, 1957; Ongley et al., 1958; Dotter et al., 1961; Braunwald et al., 1963; Engle and Ehlers, 1964). Our experience has led us to the opposite conclusion. We believe that the distinction may nearly always be made clinically with considerable assurance and is based on reliable differences in the auscultatory characteristics to which we have previously called attention (Oakley, Hallidie-Smith, and Bentall, 1964). Hancock (1961), Engle and Ehlers (1964), Gamboa, Hugenholtz, and Nadas (1964), Epstein et al. (1965), Raftery (1965), and Vogel and Blount (1965) have all emphasized the importance of the early systolic click in aortic valve stenosis. Vogel and Blount also confirmed our finding that the closure sound of the aortic valve is soft in discrete subvalvar aortic stenosis and normal or of increased intensity in aortic valve stenosis. The purpose of this report is to relate these auscultatory differences to the hemodynamic differences between the two major types of congenital aortic stenosis and to left ventricular obstruction associated with hypertrophic cardiomyopathy. Clinical judgement of the severity of congenital aortic stenosis also presents a problem: the arterial pulse, the length of the obstructive murmur, and the assessment of left ventricular hypertrophy, all being misleading on occasions. Some auscultatory characteristics which might be more consistently related to the amount of obstruction were therefore also studied.