Pulmonary Hypertension Associated With Congenital Heart Disease Pulmonary Vascular Disease: The Global Perspective

被引:71
作者
Adatia, Ian [1 ]
Kothari, Shyam S. [2 ]
Feinstein, Jeffrey A. [3 ]
机构
[1] Univ Alberta, Stollery Childrens Hosp, Edmonton, AB, Canada
[2] All India Inst Med Sci, New Delhi, India
[3] Stanford Univ, Med Ctr, Lucile Packard Childrens Hosp, Palo Alto, CA 94304 USA
关键词
VENTRICULAR SEPTAL-DEFECT; INHALED NITRIC-OXIDE; ARTERIAL-HYPERTENSION; BLOOD-FLOW; EISENMENGER-SYNDROME; SURGICAL CLOSURE; AORTOPULMONARY TRANSPOSITION; DEVELOPING-COUNTRY; ENDOTHELIAL-CELLS; BOSENTAN THERAPY;
D O I
10.1378/chest.09-2861
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
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
引用
收藏
页码:52S / 61S
页数:10
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