Hypoplastic left heart syndrome.
Hypoplastic left heart syndrome.
复制标题
低塑性左心综合征。
DOI：
10.1186/1750-1172-2-23
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发表时间：
2007-05-11
影响因子：
3.7
通讯作者：
中科院分区：
文献类型：
--
作者：
左心发育不全综合征(HLHS)是指左侧心脏结构发育异常，导致左室流出道血流受阻。此外，该综合征还包括左心室、主动脉和主动脉弓发育不良，以及二尖瓣闭锁或狭窄。据报道，在所有活产儿中，约有0.016%至0.036%发生HLHS。患有这种疾病的新生儿通常在足月出生，最初看起来很健康。当动脉管闭合时，全身血流减少，导致低氧血症、酸中毒和休克。通常，可能检测不到心脏杂音或非特异性心脏杂音。由于主动脉闭锁，第二心音是响亮的，而且是单一的。充血性心力衰竭继发于肝脏肿大。这种疾病的胚胎学原因，就像大多数先天性心脏缺陷一样，并不完全清楚。最有用的诊断方法是超声心动图。胎儿超声心动图可在妊娠18至22周时诊断出该综合征。鉴别诊断包括其他左侧梗阻性病变，这些病变的体循环依赖于导管血流(严重的主动脉狭窄、主动脉缩窄、主动脉弓中断)。患有该综合征的儿童在新生儿时期就需要手术，因为他们的全身循环依赖于导管。目前，主要有两种方式，一次心脏移植或一系列阶段性功能性单室姑息治疗。所选择的治疗取决于机构的偏好、其经验以及偏好。尽管在过去的20年里，初次手术治疗后的存活率有了显著的提高，但两种手术方法都存在显著的死亡率和发病率。因此，儿科心脏病医生继续面临与家庭讨论关于治疗的初步决定和长期预后的挑战，因为关于出生时患有这种综合征的人的长期生存和生活质量的信息有限。
Hypoplastic left heart syndrome(HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch). Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision relative to treatment, and long-term prognosis as information on long-term survival and quality of life for those born with the syndrome is limited.