Heart transplant in Ebstein's anomaly with endocardial fibroelastosis

Cabañero, J; Buruaga, J. Sáenz de; Gomez, Jose Arturo; Álava, Enrique de; Llorens, R.; Caro, D. Martínez

doi:10.1016/0002-8703(92)90629-a

Cited by 5 publications

(2 citation statements)

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“…Our collection includes 20 cases of Ebstein's deformity of the TV, 6 associated with C-TGA. In 1992, Cabanero et al [4] reported the ''first case'' of Ebstein's anomaly in association with fibroelastosis. This patient, whose 5S,D,S6 heart demonstrated Ebstein's anomaly, required heart transplantation because of severe left ventricular failure where EFE was described in the explanted heart.…”

Section: Discussionmentioning

confidence: 99%

Endocardial Fibroelastosis in L-Transposition of the Great Arteries with Ebstein's Anomaly: Revisited

1998

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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. We report here the case of an infant with a diagnosis of corrected transposition of the great arteries and Ebstein's deformity of the left-sided tricuspid valve who developed pulmonary hypertension and endocardial fibroelastosis, two unusual associations with this lesion. We also discuss the pathophysiology of this association and related literature.

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