The spectrum of Ebstein's anomaly of the tricuspid valve. 1979

J R Zuberbuhler, and S P Allwork, and R H Anderson

The structure of the tricuspid valve in 14 cases of Ebstein's malformation is described and compared with that in the normal heart. The anomalous hearts showed a spectrum of malformation, varying from minimal displacement of the adjacent parts of the septal and inferior leaflets of the tricuspid valve to presence of an imperforate membrane or muscle shelf between the inlet and trabecular zones of the right ventricle. In "typical" Ebstein's anomaly the anterosuperior leaflet was abnormally attached so as to partially obstruct the route from right atrium to distal right ventricle, the communication between these chambers being between the abnormally attached leaflet and its malformed neighbors. In two hearts, a muscular shelf produced stenosis between inlet and trabecular portions, but a competent valve was produced at this site, the atrialized part of the right ventricle being of normal thickness. The significance of these findings is considered with regard to possible methods of repair. Description of the anomaly is facilitated by considering the right ventricle as possessing inlet, trabecular, and outlet portions. The essence of the anomaly is displacement of the tricuspid orifice to the junction of the inlet and trabecular ventricular zones.

UI MeSH Term Description Entries
D009206 Myocardium The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow. Muscle, Cardiac,Muscle, Heart,Cardiac Muscle,Myocardia,Cardiac Muscles,Heart Muscle,Heart Muscles,Muscles, Cardiac,Muscles, Heart
D004437 Ebstein Anomaly A congenital heart defect characterized by downward or apical displacement of the TRICUSPID VALVE, usually with the septal and posterior leaflets being attached to the wall of the RIGHT VENTRICLE. It is characterized by a huge RIGHT ATRIUM and a small and less effective right ventricle. Ebstein's Anomaly,Ebstein's Malformation,Familial Ebstein's Anomaly,Anomaly, Ebstein,Anomaly, Ebstein's,Ebstein Malformation,Ebstein's Anomaly, Familial,Ebsteins Anomaly,Ebsteins Malformation,Familial Ebstein Anomaly,Familial Ebsteins Anomaly,Malformation, Ebstein's
D006321 Heart The hollow, muscular organ that maintains the circulation of the blood. Hearts
D006352 Heart Ventricles The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation. Cardiac Ventricle,Cardiac Ventricles,Heart Ventricle,Left Ventricle,Right Ventricle,Left Ventricles,Right Ventricles,Ventricle, Cardiac,Ventricle, Heart,Ventricle, Left,Ventricle, Right,Ventricles, Cardiac,Ventricles, Heart,Ventricles, Left,Ventricles, Right
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014261 Tricuspid Valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Tricuspid Valves,Valve, Tricuspid,Valves, Tricuspid

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