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肺动脉高压以存在毛细血管前肺高压,无左侧心脏疾病、肺部疾病、慢性血栓栓塞疾病为特征。在组织病理学方面,肺动脉高压则以肺小动脉内层平滑肌细胞和内皮细胞增殖为特征。肺动脉高压可以是原发性、遗传性或与其他疾病相关,如结缔组织疾病和先天性心脏病等。以往的研究发现,肺高压特别是肺动脉高压是镰状细胞病较常见的并发症[1]。
Pulmonary hypertension is characterized by the presence of pre-capillary pulmonary hypertension, left heart disease, lung disease, and chronic thromboembolic disease. In histopathology, pulmonary hypertension is characterized by proliferation of endothelial smooth muscle cells and endothelial cells in the pulmonary arterioles. Pulmonary hypertension can be primary, hereditary or associated with other diseases such as connective tissue disease and congenital heart disease. Previous studies have found that pulmonary hypertension, especially pulmonary hypertension, is a more common complication of sickle cell disease [1].