Forbess J M, Visconti K J, Bellinger D C, Jonas R A
Department of Cardiovascular Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Circulation. 2001 Sep 18;104(12 Suppl 1):I127-32. doi: 10.1161/hc37t1.094816.
Previous studies of patients after the Fontan operation have reported IQ scores lower than population norms. In the past decade, changes have occurred both in surgical methods used and in the patient population undergoing Fontan palliation. The present study examined the impact of these changes on neurodevelopmental outcomes after Fontan.
Neuropsychological tests were administered to 27 five-year-old children after Fontan. Mean age at repair was 2 years 4 months. The present sample was compared with an earlier Fontan group (EFG) of 133 patients who underwent surgery in the 1970s and 1980s. Mean age at repair for the EFG was 7 years 3 months. Compared with EFG, the present study sample was younger at Fontan (P=0.0001) and more likely to have undergone a Norwood procedure (P=0.02), a pre-Fontan bidirectional cavopulmonary anastomosis (P<0.001), and Fontan fenestration (P=0.001). Although mean full-scale, verbal, and performance IQ scores were within 1 SD (15 points) of the population mean of 100 (93+/-16, 95+/-15, and 91+/-17, respectively), mean full-scale and performance IQ scores were significantly lower than this population mean (P=0.03 and P=0.01, respectively).
Compared with a historical cohort of Fontan patients from this institution, a staged approach to Fontan earlier in life is not detrimental to neurodevelopmental outcome. Neurodevelopmental outcomes in children after Fontan are in the normal range, but performance remains lower than the general population.
既往对接受Fontan手术的患者的研究报告称，其智商得分低于人群平均水平。在过去十年中，Fontan姑息手术所采用的手术方法以及接受该手术的患者群体均发生了变化。本研究探讨了这些变化对Fontan手术后神经发育结局的影响。
对27名接受Fontan手术的5岁儿童进行了神经心理学测试。修复时的平均年龄为2岁4个月。将本研究样本与20世纪70年代和80年代接受手术的133例早期Fontan手术患者组成的早期Fontan组（EFG）进行比较。EFG修复时的平均年龄为7岁3个月。与EFG相比，本研究样本接受Fontan手术时年龄更小（P = 0.0001），更有可能接受了诺伍德手术（Norwood procedure）（P = 0.02）、Fontan手术前双向腔肺吻合术（P < 0.001）以及Fontan开窗术（P = 0.001）。尽管全量表、言语和操作智商的平均得分在人群平均得分100的1个标准差（15分）范围内（分别为93±16、95±15和91±17），但全量表和操作智商的平均得分显著低于该人群平均得分（分别为P = 0.03和P = 0.01）。
与该机构的一组Fontan手术历史队列相比，在生命早期采用分期Fontan手术方法对神经发育结局并无不利影响。Fontan手术后儿童的神经发育结局在正常范围内，但表现仍低于一般人群。
