Matsuhisa Hironori, Yoshimura Naoki, Niimi Hideki, Ichida Fukiko
First Department of Surgery, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan.
Interact Cardiovasc Thorac Surg. 2010 Jun;10(6):1037-8. doi: 10.1510/icvts.2009.228569. Epub 2010 Mar 2.
A four-month-old male with Taussig-Bing anomaly and multiple ventricular septal defects underwent an open-heart palliative procedure. He suffered from massive postoperative gastrointestinal bleeding. Heterozygous Arg402Stop-related factor VII deficiency was detected by genomic examinations. When he was 14 months old, a subsequent open-heart surgery with replacement therapy of recombinant factor VIIa was performed without any bleeding or thromboembolic complications. Although heterozygous factor VII deficiency is generally recognized as clinically asymptomatic, this latent bleeding disorder can appear perioperatively or postoperatively in patients who undergo cardiopulmonary bypass procedures. Consequently, the prophylactic replacement therapy with recombinant factor VII is recommended during cardiac operations.
一名患有陶西格-宾畸形和多处室间隔缺损的4个月大男性接受了心脏姑息手术。他术后出现大量胃肠道出血。通过基因检测发现了与杂合性Arg402Stop相关的因子VII缺乏症。当他14个月大时，随后进行了心脏直视手术并采用重组因子VIIa替代疗法，未出现任何出血或血栓栓塞并发症。虽然杂合性因子VII缺乏症通常被认为临床上无症状，但这种潜在的出血性疾病可能在接受体外循环手术的患者围手术期或术后出现。因此，建议在心脏手术期间进行重组因子VII预防性替代治疗。
