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Ebstein anomaly

Sunday 15 January 2006

Ebstein’s anomaly is a congenital deformity of the tricuspid valve consisting mainly of leaflet malinsertion.

Clinical presentation varies from asymptomatic patients to those with congestive heart failure secondary to significant valvular regurgitation and low right ventricular output.

Synopsis

- Ebstein’s deformity of the left-sided tricuspid valve
- pulmonary hypertension
- endocardial fibroelastosis

Associations

- transposition of the great arteries
- non-syndromal bilateral non-obstructive renal dysplasia (non-syndromal BNORD) (8725780)

References

- Dahdah NS, van Doesburg NH, Russo P. Endocardial fibroelastosis in L-transposition of the great arteries with Ebstein’s anomaly: revisited. Pediatr Dev Pathol. 1998 Sep-Oct;1(5):427-32. PMID: 9688767