Background: Ebstein's Disease (ED) is a rare congenital heart disease with the tricuspid valve
dysfunction. Little is known about the clinical course and the optimal strategy for
the management of adult ED patients. Method and Result: The clinical course of six consecutive adult patients of unrepaired ED was investigated.
Among them, four cases including three asymptomatic pregnant women (32, 36 and 37
y.o.) and one elderly patient (72 y.o.) with severe tricuspid regurgitation (TR) and
chronic atrial fibrillation (Af) had no cardiac event. On the other hand, two cases
required surgical intervention. One younger patient (32 y.o.) with atrial septal defect
and severe TR had surgical repair due to progressive polycythemia by hypoxia. Another
elder patient (64 y.o.) with severe TR had tricuspid valve repair due to exacerbated
heart failure induced by new onset of Af. Conclusion: The clinical course of unrepaired ED in adult patients is varied. Careful follow-up
is important not to miss the optimal timing of surgical intervention in adult ED patients
irrespective of their symptom.
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