Guérin F, Hazan E, Herreman F, Toussaint M
Arch Mal Coeur Vaiss. 1977 Jun;70(6):653-61.
A case is reported of hypoplasie of the right ventricle with a wide atrial septal defect in a child of 10. The presence of cyanosis with right atrial and left ventricular preponderance led us to suspect tricuspid atresia, but this was excluded by catheterisation and angiocardiography. There was definitely and atrial septal defect with a two-directional shunt, but the tricuspid valve could be negotiated, and the right-sided opacity provided the essential diagnostic criterion, namely hypoplasia of the sinus portion of the right ventricle with a normal infundibulum. 17 other published cases of hypoplasia of the right ventricle with atrial septal defect are reviewed. The current indications for surgery are discussed in the light of the nature of the disordered physiology. Simple closure of the atrial septal defect, which was carried out successfully in 3 cases, appears to be of great value in the larger child, when there is a wide defect.
报道了一例10岁儿童右心室发育不全合并巨大房间隔缺损的病例。患儿出现紫绀,右心房和左心室优势,这使我们怀疑三尖瓣闭锁,但心导管检查和心血管造影排除了这种情况。确实存在房间隔缺损并有双向分流,但三尖瓣可通过导管,右侧不透明提供了关键的诊断标准,即右心室窦部发育不全而漏斗部正常。本文回顾了其他17例已发表的右心室发育不全合并房间隔缺损的病例。根据紊乱的生理状况讨论了当前的手术指征。在3例患儿中成功进行的单纯房间隔缺损闭合术,对于缺损较大的大龄儿童似乎具有重要价值。