[Aortic insufficiency caused by valvular dysplasia in childhood. Apropos of 3 cases].
Three patients whose aortic incompetence (A.I.) was recognized in childhood, have had an angiographic study 10, 9 and 4 years, respectively, following the first clinical diagnosis of their valvular disease. A.I. was related, in all three cases, to a dysplastic valve ("floppy valve syndrome"); in none of them was there an abnormal dilatation of ascending aorta. In our opinion, the absence of an abnormal aortic root dilatation separates our cases from those in whom aortic regurgitation is part of Marfan's syndrome, either in its full form or in the "forme fruste". From the clinical viewpoint, the presence of a normal ascending aorta could explain the mild course of these cases, as compared to the rapid progression observed in Marfan's syndrome. In the attempt to differentiate the two types of dysplastic valvular disease from an etiologic viewpoint, we suggest that the basic problem in our patients could be an alteration of the valvular connective tissue development, rather than a genetic disorder, like the one possibly responsible for Marfan's syndrome.
D000293:Adolescent; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D001022:Aortic Valve Insufficiency / Q000209:etiology*; D001027:Aortography; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis*
Insufficienza aortica da displasia valvolare nella fanciullezza. A proposito di tre casi osservati.