Key result
An infant with corrected transposition of the great arteries and Ebstein's deformity of the left-sided tricuspid valve developed pulmonary hypertension and endocardial fibroelastosis.
Case Report (n=1)
Highlights the unusual association of pulmonary hypertension and endocardial fibroelastosis in a patient with L-TGA and Ebstein's anomaly.
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Suggests pulmonary hypertension monitoring in L-TGA with Ebstein anomaly; leaves open causal role of endocardial fibroelastosis.
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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Dahdah et al. (1998) conducted a case report in L-Transposition of the Great Arteries with Ebstein's Anomaly (n=1). An infant with corrected transposition of the great arteries and Ebstein's deformity of the left-sided tricuspid valve developed pulmonary hypertension and endocardial fibroelastosis.
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