Pulmonary Hypertension Associated With Congenital Heart Disease Pulmonary Vascular Disease: The Global Perspective
Pulmonary Hypertension Associated With Congenital Heart Disease Pulmonary Vascular Disease: The Global Perspective
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DOI：
10.1378/chest.09-2861
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发表时间：
2010-06-01
期刊：
影响因子：
9.6
通讯作者：
Feinstein, Jeffrey A.
中科院分区：
文献类型：
--
作者：
Adatia, Ian;Kothari, Shyam S.;Feinstein, Jeffrey A.
先天性心脏病的发病率约为8/1,000活产，在世界各地似乎是恒定的。目前公认的与先天性心脏病相关的肺血管疾病的发展模式认为，肺血流量和压力增加会引发不利的血管重塑。内皮细胞功能障碍、异常剪切应力、周壁拉伸和血管活性介质失衡共同促进血管收缩、炎症、血栓形成、细胞增殖、细胞凋亡受损和纤维化。我们估计，全世界有300万儿童由于先天性心脏病而面临发展为肺血管疾病的风险。全球大多数面临风险的儿童都有可修复的心脏缺陷，如孤立的房间隔或室间隔缺损或动脉导管未闭。2岁前的心脏修复可以预防艾森曼格综合征的发展，艾森曼格综合征是继发于先天性心脏病的肺血管疾病的最晚期形式。在世界范围内，只有一小部分有风险的人接受手术修复。因此，获得及时的医疗护理将消除艾森门格综合症造成的绝大多数痛苦、残疾和死亡。在全球范围内，与先天性心脏病相关的肺血管疾病可能是肺动脉高压及其相关死亡率和发病率的最可预防的原因。胸部2010;137(6)(增刊):52 - 61年代
The incidence of congenital heart disease is approximately 8/1,000 live births and appears to be constant around the world. The currently accepted paradigm for the development of pulmonary vascular disease associated with congenital heart disease maintains that increased pulmonary blood flow and pressure trigger unfavorable vascular remodeling. Endothelial cell dysfunction, abnormal shear stress, circumferential wall stretch, and an imbalance in vasoactive mediators conspire to promote vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apoptosis, and fibrosis. We estimate that worldwide 3 million children are at risk for the development of pulmonary vascular disease due to congenital heart disease. The majority of children at risk globally will have a reparable heart defect, such as an isolated atrial septal or ventricular septal defect or patent duct-us arteriosus. Cardiac repair in the first 2 years of life would prevent the development of Eisenmenger syndrome, the most advanced form of pulmonary vascular disease secondary to congenital heart disease. Worldwide, only a small fraction of those at risk are offered surgical repair. Thus, access to timely medical care would eliminate the vast majority of suffering, disability, and death from Eisenmenger syndrome. Globally, pulmonary vascular disease associated with congenital heart disease may be the most preventable cause of pulmonary artery hypertension and related mortality and morbidity. CHEST 2010; 137(6)(Suppl):52S-61S