患者 ,男 ,66岁。出生后家人未发现异常 ,会走路后喜蹲位 ,剧烈活动时有气短。但达 1 0岁后一般活动无明显受限 ,至 5 0岁还可作轻松的工作 ,上三楼无气短。近几年活动耐力明显下降 ,家人发现唇紫 ,冬季咳痰带血丝 ,为明确诊断而来院。病程中无昏厥抽搐史。体检 :有明显紫绀和 Patient, male, 66 years old. After the birth of his family did not find abnormalities, will like to squat after walking, intense shortness of breath. However, up to the age of 10, there was no obvious limitation on general activities. By the age of 50, work could be relaxed and no shortness of breath on the third floor. Activity endurance decreased significantly in recent years, the family found lip purple, winter sputum with bloodshot eyes, to a clear diagnosis from the hospital. No syncope in the course of convulsions history. Physical examination: obvious cyanosis and