Abstract
In an attempt to clarify the pathogenetic morphology of aortic regurgitation (AR) due to prolapse of the aortic valve (prolapsing AR) associated with ventricular septal defect (VSD), 201 specimens from Japanese autopsy series with isolated VSD were examined. Among these hearts, there were 128 cases (64%) of infundibular VSD (IVSD); 29 of them (14%) showed AR due to prolapsed cusp, of which nine cases developed a large aneurysm of the sinus of Valsalva. Another 32 cases (16%) had varying degrees of prolapse but without AR and were considered to show the prodrome of prolapsing AR. These 61 cases (30%) were examined with special reference to the type of septal alignment, location of the defect, relation of the defect to the aortic valve, and anomalies of the aortic valve and sinus of Valsalva. There were two principal forms in this syndrome: The common form, i.e., simple punched-hole IVSD with normal septal alignment in 82% (50/61) of cases, and a rare form, i.e., malalignment IVSD in 18% (11/61) of cases. The latter included Eisenmenger-type IVSD due to anteriorly deviated outlet septum (10/11 cases) and coarctation-type IVSD due to posteriorly deviated septum (1/11). Both forms had several subtypes according to the location of t...Continue Reading
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