Graham Eric M, Bradley Scott M, Atz Andrew M
Medical University of South Carolina, 165 Ashley Avenue, PO Box 250915, Charleston, SC 29425, USA.
Expert Opin Pharmacother. 2005 May;6(5):687-93. doi: 10.1517/14656566.6.5.687.
Hypoplastic left heart syndrome (HLHS) is the most common functional single ventricle congenital cardiac defect. This syndrome is characterised by a functional single right ventricle and systemic outflow obstruction. The systemic and pulmonary circulations compete for cardiac output with a resultant precarious balance among systemic, pulmonary and coronary blood flows. A once fatal diagnosis, advances in operative and perioperative care have resulted in a dramatic improvement in survival. The preoperative management of neonates with HLHS is based predominately on clinical experience and extrapolated data from the postoperative literature. Management focuses on maintaining patency of the systemic outflow, balancing the pulmonary and systemic blood flows, and preserving the function of a single right ventricle to maximise oxygen delivery to the tissues. This paper reviews the available therapies for the preoperative management of HLHS.
左心发育不全综合征（HLHS）是最常见的功能性单心室先天性心脏缺陷。该综合征的特征是功能性单右心室和体循环流出道梗阻。体循环和肺循环争夺心输出量，导致体循环、肺循环和冠状动脉血流之间的平衡不稳定。曾经是致命的诊断，手术和围手术期护理的进展已使生存率显著提高。HLHS新生儿的术前管理主要基于临床经验和术后文献的推断数据。管理重点是维持体循环流出道的通畅，平衡肺循环和体循环血流，并保留单右心室的功能，以最大限度地向组织输送氧气。本文综述了HLHS术前管理的可用治疗方法。
