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原发性肺动脉高压(PPH)是一少见的疾病,因病因不明而区别于继发性肺动脉高压,1987年美国做了多中心的广泛研究,对原发性肺动脉高压的流行病学、病因学有了进一步的了解,诊断和治疗水平也有所提高,预后已有一定的改观,但仍相当险恶。一、原发性肺动脉高压的定义和术语直到1950年心导管检查术问世、原发性肺动脉高压才得以明确诊断。“原发性(Primary)一词表示除肺血管病变以外,未发现引起肺动脉高压或肺血管硬化的任何其它原因。病理属致丛性肺动脉
Primary pulmonary hypertension (PPH) is a rare disease that differs from secondary pulmonary hypertension due to an unknown etiology. In 1987, the United States did a multicentre, extensive study of the epidemiology and etiology of primary pulmonary hypertension With further understanding, diagnosis and treatment have also improved, the prognosis has been some improvement, but still quite dangerous. First, the definition and terms of primary pulmonary hypertension Until the advent of cardiac catheterization in 1950, primary pulmonary hypertension was able to confirm the diagnosis. “The word” Primary "means that no other cause of pulmonary hypertension or pulmonary vascular sclerosis was found except for pulmonary vascular disease Pathology is caused by pulmonary plexus