Carter J E, Feldman T, Carroll J D
University of Chicago Hospital, Hans Hecht Hemodynamics Laboratory, IL 60637.
Eur Heart J. 1994 Apr;15(4):575-6. doi: 10.1093/oxfordjournals.eurheartj.a060545.
A 60-year-old man with Noonan's syndrome presented with generalized weakness, dyspnoea, and paresthesias. He was known to have pulmonic stenosis and atrial septal defect with right-to-left shunting and had refused therapy for many years. On presentation there was cyanosis and the haematocrit was 63%. After phlebotomy he was treated with balloon valvuloplasty. There was marked reduction in the transpulmonic gradient and oximetric evidence for reversal of his right-to-left atrial septal defect flow. After 18 months follow-up he remained system free and had no rise in haematocrit, consistent with no return of right-to-left shunting.
一名患有努南综合征的60岁男性，出现全身乏力、呼吸困难和感觉异常。已知他患有肺动脉狭窄和房间隔缺损伴右向左分流，多年来一直拒绝治疗。就诊时出现发绀，血细胞比容为63%。放血后，他接受了球囊瓣膜成形术治疗。经肺动脉梯度明显降低，血氧测定显示其房间隔缺损右向左分流逆转。经过18个月的随访，他没有出现任何症状，血细胞比容也没有升高，这表明右向左分流没有复发。
