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原发性肺动脉高压是指原因不明的肺血管阻力增大、肺动脉压力增高,常引起右心室肥大。原发性肺动脉高压是少见的,1951年Dresdale首次对本病征进行了系统的描述,迄今各国报道的总数仅数百例,国内也有报道。女性多于男性,其比例为1.7:1。典型病例在20～40岁。
Primary pulmonary hypertension refers to the unexplained pulmonary vascular resistance increased pulmonary artery pressure, often causing right ventricular hypertrophy. Primary pulmonary hypertension is rare. In 1951, Dresdale first described the symptoms of the disease systematically. Up to now, only a few hundred cases have been reported in various countries and reported in China. More women than men, the ratio of 1.7: 1. Typical cases in 20 to 40 years old.