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Ebstein's anomaly

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A rare congenital malformation of the heart with downward and rotational displacement of the right atrioventricular valve to the apex of the right ventricle. Symptoms are various and essentially determined by the degree of valve dysplasia and the reduction of size of the right ventricle and its outflow. They range from none to cyanosis, cardiac arrythmias, especially paroxysmal tachyardias, fatigability, and dyspnea. Both sexes are equally affected. Some patients die in infancy, others may survive to advanced age.

Description submitted by Martin Lehn, Dortmund, Germany.


  • W. Ebstein:
    Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben.
    Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238-254.
    Translation in American Journal of Cardiology, New York, 1968, 22: 867-872.

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