原发性肺动脉高压症较罕见,本室历年总计尸检2,700余例,仅见此一例。现将本例临床、病理结合文献介绍如下。临床资料:女性,24岁。因进行性心跳气急伴口唇发绀16年,反复昏厥半年,腹胀浮肿3个月而入院治疗。入院时检查:叩诊心浊音界向两侧扩大,心律齐,P_2>A_2,P_2亢进。左侧心前区胸廓略隆起,胸骨左缘可闻及Ⅴ°收缩期粗糙杂音,心尖区可闻及Ⅳ°收缩期杂音。两肺呼吸音粗糙,未闻及干湿性罗音。腹平软,肝肋下 Primary pulmonary hypertension is rare, the total calendar year autopsy 2,700 cases, only to see this case. Now this case clinical, pathological literature are described below. Clinical data: Female, 24 years old. Because of progressive heartbeat with lip cyanosis 16 years, repeated fainting six months, abdominal distension and edema 3 months admitted to hospital. Admission examination: Percussion heart voiced sound bound to expand on both sides, rhythm Qi, P_2> A_2, P_2 hyperthyroidism. The left anterior chest area slightly uplift the chest, left sternal border can be heard and Ⅴ rough contraction of the murmur, apical area can be heard and Ⅳ ° systolic murmur. Breathe sound rough between the two lungs, unheard-of and wet and dry rales. Abdomen soft, liver ribs