Razzouk A J, Chinnock R E, Gundry S R, Johnston J K, Larsen R L, Baum M F, Mulla N F, Bailey L L
Department of Surgery, Loma Linda University School of Medicine, California, USA.
Ann Thorac Surg. 1996 Jul;62(1):1-7; discussion 8. doi: 10.1016/0003-4975(96)00295-0.
Hypoplastic left heart syndrome is a lethal malformation. For the last 10 years, orthotopic cardiac transplantation has been our preferred treatment for infants with hypoplastic left heart syndrome.
One hundred seventy-six infants with hypoplastic left heart syndrome were entered into a cardiac transplant protocol between November 1985 and November 1995. Interventional procedures to stent the ductus arteriosus or enlarge the interatrial communication were performed in 8 and 35 patients, respectively. Thirty-four patients (19%) died during the waiting period, and 142 infants underwent cardiac transplantation. Age at cardiac transplantation ranged from 1.5 hours to 6 months (median, 29 days). The majority of grafts were oversized, and the median graft ischemic time was 273 minutes (range, 60 to 576 minutes). The implantation procedure used a period of hypothermic circulatory arrest ranging from 23 to 110 minutes (median, 53 minutes). Repair of other significant defects included interrupted aortic arch and total or partial anomalous pulmonary venous connection.
There were 13 early and 22 late deaths. Patient actuarial survival at 1 month and at 1, 5 and 7 years was 91%, 84%, 76%, and 70% respectively. Half of the late deaths were due to rejection. Severe graft vasculopathy was confirmed in 8 patients. Retransplantation was performed in 5 patients for graft vasculopathy 4 and rejection 1. Lymphoblastic leukemia developed in 1 patient 3 years after cardiac transplantation.
Cardiac transplantation can be performed in infants with hypoplastic left heart syndrome with good operative and intermediate-term results. Improved survival can be achieved with increased donor availability, better management of rejection, and control of graft vasculopathy.
左心发育不全综合征是一种致命的畸形。在过去10年中，原位心脏移植一直是我们治疗左心发育不全综合征婴儿的首选方法。
1985年11月至1995年11月期间，176例左心发育不全综合征婴儿进入心脏移植方案。分别对8例和35例患者进行了介入操作以扩张动脉导管或扩大房间隔交通。34例患者（19%）在等待期间死亡，142例婴儿接受了心脏移植。心脏移植时的年龄为1.5小时至6个月（中位数为29天）。大多数移植物尺寸偏大，移植物中位缺血时间为273分钟（范围为60至576分钟）。植入手术采用了23至110分钟（中位数为53分钟）的低温循环停止时间。其他重要缺陷的修复包括主动脉弓中断和完全或部分肺静脉异位连接。
有13例早期死亡和22例晚期死亡。患者1个月、1年、5年和7年的实际生存率分别为91%、84%、76%和70%。一半的晚期死亡是由于排斥反应。8例患者确诊有严重的移植物血管病变。5例患者因移植物血管病变4例和排斥反应1例进行了再次移植。1例患者在心脏移植3年后发生淋巴细胞白血病。
左心发育不全综合征婴儿可以进行心脏移植，手术效果和中期结果良好。通过增加供体来源、更好地管理排斥反应和控制移植物血管病变，可以提高生存率。
