Nishimura T, Shimazaki Y, Ohashi T, Kamada S, Sano T, Matsuda H
First Department of Surgery, Osaka University Medical School, Suita, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1994 Aug;42(8):1263-6.
A 8 month-old female was diagnosed with ventricular septal defect and patent ductus arteriosus associated with absent right pulmonary artery and scimitar syndrome. Cardiac catheterization revealed severe pulmonary hypertension as follows; 72 mmHg of mean pulmonary artery pressure, 0.56 Qp/Qs and 1.73 Pp/Ps. Temporary closure of the ductus reduced the mean pulmonary artery pressure from 72 to 40 mmHg. PDA was ligated and VSD was closed successfully. However, 4 months after initial operation she was readmitted due to infection of the hypoplastic right lung. Removal of the hypoplastic right lung was performed and postoperative course was uneventful.
一名8个月大的女性被诊断为室间隔缺损、动脉导管未闭，同时伴有右肺动脉缺如和弯刀综合征。心导管检查显示严重肺动脉高压，具体如下：平均肺动脉压72mmHg，肺循环血流量与体循环血流量比值0.56，肺血管阻力与体循环血管阻力比值1.73。暂时结扎动脉导管使平均肺动脉压从72mmHg降至40mmHg。成功结扎动脉导管未闭并闭合室间隔缺损。然而，初次手术后4个月，她因发育不良的右肺感染再次入院。切除了发育不良的右肺，术后过程顺利。
