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先天性心脏病(简称先心病)合并肺动脉高压是严重影响我国儿童身心健康的主要疾病之一,其复杂的发病机制仍未完全明了。但是,近年来对于先心病病理生理条件的动物模型的建立、肺组织的病理学资料与血液分子水平的生化指标的研究日益增多,并已取得较大进展。本文综述了近几年来国内外有关先心病、PH发病机制的研究进展,以便能更深入理解其机制,指导临床治疗。
Congenital heart disease (referred to as CHD) with pulmonary hypertension is one of the major diseases that seriously affect the physical and mental health of children in our country. The complicated pathogenesis is still not completely understood. However, the establishment of animal models of pathophysiological conditions of congenital heart disease, the pathological data of lung tissue and the biochemical indexes of blood molecular level are increasing day by day in recent years, and great progress has been made. This review summarizes the research progress of pathogenesis of congenital heart disease and PH in the world in recent years in order to understand its mechanism and guide clinical treatment.