Anderson Robert H, Spicer Diane
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America.
Cardiol Young. 2010 Dec;20 Suppl 3:86-91. doi: 10.1017/S1047951110001125.
Neonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.
与诺伍德手术序列发展之前的情况相比，患有左心发育不全综合征的新生儿现在的预后有了显著改善。然而，一些在室间隔完整的肺动脉闭锁情况下出生的右心室发育不全的新生儿，预后仍然相对较差。部分原因是发育不全的右心室腔与冠状动脉树之间存在瘘管交通。现在越来越认识到这种瘘管交通在左心发育不全综合征中很重要。在这篇简短的综述中，我们描述了这些交通的解剖结构。当心室腔实际上仅代表入口时，在发育不全的右心室中发现的情况最为常见，这反过来又反映了胎儿发育过程中的心尖小梁和流出道成分的壁过度生长。这几乎肯定反映了促进腔室发育不全的肺动脉瓣病变的更早出现。在左心室发育不全的患者中，区分有瘘管交通的患者的关键特征是二尖瓣开放，因为在二尖瓣闭锁或左房室连接缺失的情况下，左心室通常只不过是后下室壁中的一个虚拟缝隙。
