Endocardial fibroelastosis in L-transposition of the great arteries with Ebstein's anomaly: revisited.
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.
Department of Pediatric Cardiology, Sainte-Justine Hospital, 3175 Côte Sainte-Catherine, Montréal, Québec, Canada H3T 1C5.
N S
NS
Dahdah
N H
NH
van Doesburg
P
P
Russo
D004437:Ebstein Anomaly / Q000150:complications / Q000473:pathology*; D004695:Endocardial Fibroelastosis / Q000150:complications / Q000473:pathology*; D005260:Female; D005355:Fibrosis / Q000473:pathology; D006325:Heart Atria / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007223:Infant; D011651:Pulmonary Artery / Q000473:pathology; D014188:Transposition of Great Vessels / Q000150:complications / Q000473:pathology*