Moodie D S, Gill C C, Sterba R, Stewart R, Ratliff N B
Ann Thorac Surg. 1986 Sep;42(3):307-11. doi: 10.1016/s0003-4975(10)62741-5.
From February, 1983, to November, 1984, 11 infants with hypoplastic left heart syndrome were managed medically prior to operative intervention. Only 2 of the 11 infants appeared to be in mild distress at birth. Despite relatively normal Apgar scores, 9 of the infants were seen in shock. All patients were managed medically with fluid restriction, diuretics, and the institution of prostaglandin E1. There was a marked improvement in arterial pH from a mean value of 7.13 prior to prostaglandin to 7.42 following prostaglandin infusion. There was also a dramatic decrease in the serum creatinine level from a mean pretreatment level of 1.5 mg/dl to 0.7 mg/dl following infusion. Ten of the 11 infants were operated on at a mean age of 4 days with a Norwood or conduit procedure. Three infants who died at 3, 13, and 24 days of age had sustained myocardial infarctions prior to operation. Three patients also had multiple infarcts of the liver. Three patients are alive 23, 22, and 6 months after operation, and are growing and developing normally. The ability to maintain the infant with hypoplastic left heart syndrome in a stable hemodynamic condition has made surgical palliation a realistic option for this otherwise fatal anomaly. Nevertheless, preoperative infarctions of the heart and viscera continue to play a major role in mortality.
从1983年2月至1984年11月，11例左心发育不全综合征婴儿在手术干预前接受了药物治疗。11例婴儿中只有2例出生时似乎有轻度窘迫。尽管阿氏评分相对正常，但9例婴儿出现休克。所有患者均接受了限制液体、使用利尿剂以及应用前列腺素E1的药物治疗。动脉血pH值从使用前列腺素前的平均值7.13显著改善至输注前列腺素后的7.42。血清肌酐水平也从输注前的平均1.5mg/dl急剧降至0.7mg/dl。11例婴儿中有10例在平均4日龄时接受了诺伍德手术或管道手术。3例分别在3日龄、13日龄和24日龄死亡的婴儿在手术前发生了心肌梗死。3例患者还出现了肝脏多发梗死。3例患者在术后23个月、22个月和6个月存活，生长发育正常。将左心发育不全综合征婴儿维持在稳定血流动力学状态的能力使得手术姑息治疗成为这种原本致命异常情况的现实选择。然而，术前心脏和内脏梗死仍然是死亡率的主要影响因素。
