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目的比较特发性肺动脉高压(idiopathic pulmonary arterial hypertension,IPAH)与先天性心脏病相关性肺动脉高压(pulmonary arterial hypertension associated with congenital heart disease,PAH-CHD)患者小气道功能的差异。方法50例确诊为IPAH的患者、44例PAH-CHD患者及24位健康人群(对照组),进行常规肺功能检查(pulmonary function test,PFT)及脉冲振荡肺