Jaquiss Robert D B, Imamura Michiaki
Arkansas Children's Hospital & University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA.
Curr Opin Cardiol. 2009 Mar;24(2):113-8. doi: 10.1097/HCO.0b013e328323d85a.
Among the most frequently encountered congenital heart malformations are those with so-called single ventricle physiology, in which there is only one ventricle to pump blood to the pulmonary and systemic vascular beds, respectively. Long-term survival is possible, based on the principle of right heart bypass, whereby the ventricle pumps blood only to the systemic circuit, whereas pulmonary blood flow occurs passively. Such a circulatory system is achieved in a series of staged reconstructive operations, each of which was formerly accompanied by very high rates of major morbidity and mortality. Current approaches to single ventricle physiology as well as areas of controversy will be reviewed.
The development of a number of inventive operations, combined with a greater understanding of the physiologic requirements for success after single ventricle reconstruction has resulted in dramatic improvements in outcomes. The identification and modification of risk factors as well as the recent development of catheter-based intervention offer the real prospect of significant continued improvement.
Advances in the care of children with single ventricle hearts have resulted in remarkably improved prognosis, with the expectation of continued improvement in not only survival but also quality of life.
最常见的先天性心脏畸形包括那些具有所谓单心室生理特征的畸形，即只有一个心室分别向肺循环和体循环血管床泵血。基于右心旁路的原理，长期生存是可能的，即心室仅向体循环泵血，而肺血流被动发生。这种循环系统是通过一系列分期重建手术实现的，以前每一次手术都伴随着很高的严重发病率和死亡率。本文将综述目前针对单心室生理的治疗方法以及存在争议的领域。
一些创新手术的发展，加上对单心室重建术后成功的生理需求有了更深入的了解，使得治疗结果有了显著改善。危险因素的识别与修正以及基于导管介入的最新发展为持续显著改善提供了切实的前景。
单心室患儿护理方面的进展已使预后得到显著改善，不仅预期生存率会持续提高，生活质量也会提高。
