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