[A pathological study of valvular dysplasia and attachment in Ebstein's anomaly]

Abstract

Forty hearts with Ebstein's disease of the tricuspid valve were studied in order to correlate dysplasia and attachment of tricuspid valve leaflets at the ventricular walls, atrialization of the right ventricle and fibrosis in this chamber. The correlation was positive. The right ventricular atrialization was directly proportional to leaflet attachment; the correlation in severity between leaflet attachment; the correlation in severity between leaflet attachment and dysplasia was positive in 45%. The right ventricular fibrosis was very frequent in the group with patent pulmonary valve (90%), and the contrary was in the group with pulmonary valve atresia (10%). The structural damage in the right ventricular wall was directly proportional to leaflet attachment in hearts with patent pulmonary valve (90%); in all of these hearts the anatomic annulus of the tricuspid valve was enlarged but in hearts with pulmonary valve atresia this feature only occurred in 10%. The specimens with atrioventricular discordant connexion, the anatomic annulus size, the degree of leaflet attachment, the dysplasia of the tricuspid valve as well as the fibrosis of the wall of the right ventricle were less severe compared with those hearts with concordant atrioventricular connexion. Ebstein's disease of the tricuspid valve is characterized by a disorder in the histodifferentiation of the right ventricle and the tricuspid valve which originates in great part from it.