原发性肺动脈高压症为一稀見疾病,以往临床上常难确診,多数于屍检获得到診断。近十余年来,右心导管检查术广泛使用后,发现逐漸增多。目前国内对此病尚少专题报告。本文就所見一病例,結合文献略加討論。病例报告患者,男,13岁,学生(住院号23850),于1961年10月20日入院,主诉自幼发育不良,体力衰弱,行动缓慢,易疲劳,智力也较差。11岁前即有輕度心跳气短現象,家人未予重視,自11岁起,每負重走路后,即有明显心跳气短,并伴有口唇青紫,此外輕常有輕度咳嗽,休息后可缓解。近一年来症状加重,又出現胸闷感和暈厥現象,每次 Primary pulmonary hypertension is a rare disease, often clinically difficult to diagnose the past, the majority of autopsy obtained diagnosis. In the past ten years or so, the widespread use of right heart catheterization found that gradually increased. At present, there are still few special reports on this disease in our country. This article has seen a case, combined with the literature a little discussion. Case report The patient, male, 13 years old, student (hospital number 23850), was admitted to hospital on October 20, 1961. He complained of poor growth, weakness, slow movement, fatigue, and poor intelligence. 11 years before that there is a mild shortness of breath shortness of breath, the family did not pay attention, since the age of 11, after each weight walk, there is a clear shortness of breath and shortness of breath, accompanied by lips bruising, in addition mild mild cough, rest can ease . In the past year, the symptoms have increased, chest tightness and syncope have appeared again, each time