David P, Piot J D, Leriche H, Bouchard F, Belhaj M, Issad M S, Faivre G, Binet J P
Arch Mal Coeur Vaiss. 1985 Jun;78(6):925-7.
2 cases of severe pulmonary valvular stenosis in two young adults aged 21 and 29 years respectively are presented. Despite the degree of stenosis (4 mm jet), the right ventricular pressures did not exceed systemic pressures because of severe tricuspid regurgitation. This dominated the clinical and anatomical findings, causing aneurysmal dilatation of the right atrium and displacement of the tricuspid valve to the left. The diagnosis of Ebstein's anomaly was raised in one case but excluded by echocardiography and angiocardiography. In spite of the severity of the symptoms and peripheral clinical signs, surgery of the right ventricular outflow tract, tricuspid valvuloplasty and resection of part of the right atrium led to a complete and rapid cure, confirmed by catheterisation one month after operation.
本文报告了2例分别为21岁和29岁的年轻成人重度肺动脉瓣狭窄病例。尽管狭窄程度（射流4毫米）严重，但由于严重的三尖瓣反流，右心室压力并未超过体循环压力。这主导了临床和解剖学表现，导致右心房瘤样扩张和三尖瓣向左移位。其中1例曾提出埃布斯坦畸形的诊断，但经超声心动图和心血管造影排除。尽管症状和外周临床体征严重，但右心室流出道手术、三尖瓣成形术和部分右心房切除术导致了完全且迅速的治愈，术后1个月通过心导管检查得以证实。
