Dahdah N S, van Doesburg N H, Russo P
Department of Pediatric Cardiology, Sainte-Justine Hospital, 3175 Côte Sainte-Catherine, Montréal, Québec, Canada H3T 1C5.
Pediatr Dev Pathol. 1998 Sep-Oct;1(5):427-32. doi: 10.1007/s100249900058.
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.
埃布斯坦畸形是一种主要由瓣叶附着异常引起的三尖瓣先天性畸形。临床表现从无症状患者到因严重瓣膜反流和右心室输出量低继发充血性心力衰竭的患者不等。我们在此报告一例诊断为大动脉转位矫正型和左侧三尖瓣埃布斯坦畸形的婴儿病例，该婴儿出现了肺动脉高压和心内膜弹力纤维增生症，这两种情况与该病变的关联并不常见。我们还讨论了这种关联的病理生理学及相关文献。
