Circulation, Vol 75, 1124-1129, Copyright © 1987 by American Heart Association
P Zielinsky, M Rossi, JC Haertel, D Vitola, FA Lucchese and R Rodrigues
The purpose of this study was to test the hypothesis that the presence of a
subaortic ridge associated with a ventricular septal defect (VSD) is
related to a malaligned ventricular septum caused by anterior or posterior
deviation of the infundibular septum with or without obstructive lesions of
the aortic arch. Thirty-two of 295 patients in whom a diagnosis of VSD was
made by two-dimensional echocardiography and who were studied from June
1983 to April 1985 presented with a subaortic shelf. Every patient (p less
than .00001) had a malalignment type of defect; the defect was produced by
anterior deviation of the outlet septum (without compromise of the right
ventricular outflow tract) in 28 and by posterior deviation of the
infundibular septum in four. The prevalence of a subaortic shelf in the
malalignment VSD group was 82% (32/39). Among the 28 patients with a
subaortic ridge and anterior deviation of the outlet septum only three had
aortic coarctation, but all four patients with subaortic stenosis and
posterior infundibular malalignment had obstructive lesions of the aortic
arch--coarctation in three and interruption of the aortic arch in one (p
less than .001). We conclude that a malalignment type of VSD may be a
consistent feature in patients with VSD and associated discrete subaortic
stenosis. We also noted a high prevalence of subaortic ridge in the
presence of a malalignment VSD and therefore speculate that there may be a
common morphogenesis for malalignment VSD, subaortic shelf, and obstructive
lesions of the aortic arch.
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Subaortic fibrous ridge and ventricular septal defect: role of septal malalignment
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