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Ebstein's anomaly
Also known as:
Ebstein’s syndrome
Ebstein’s malformation
Associated persons:
Wilhelm Ebstein
Description:
A very rare congenital condition of the heart with facultative cyanosis and downward displacement of the tricuspid valve from the annulus fibrosus. Symptoms are cardiac arrythmias, especially extracystoles and paroxysmal tachyardia, fatigability, dyspnea, cyanosis, and sudden death. Both sexes equally affected. Asymptomatic for years. Some patients die in infancy, others may survive to advanced age. Average lifespan is 22 years. Probably recessive inheritance.
Bibliography:
- 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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