Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; Alberta Transplant Institute, University of Alberta, Edmonton, Alberta, Canada; Department of Medical Microbiology and Immunology, University of Alberta, Edmonton, Alberta, Canada.
Department of Pediatrics, Glenrose Rehabilitation Hospital, University of Alberta, Edmonton, Alberta, Canada.
J Heart Lung Transplant. 2018 Jun;37(6):740-748. doi: 10.1016/j.healun.2017.12.013. Epub 2017 Dec 20.
Children requiring heart transplantation (HTx) for congenital heart disease (CHD) or failing anatomically normal hearts (CMP) face different challenges pre-HTx. We compared the neurocognitive capabilities in pre-school-age children receiving HTx for CHD vs CMP and determined factors predicting outcomes.
Data were collected within a prospective multi-provincial project from children who underwent HTx ≤4 years of age between 1999 and 2011. At age 54 ± 3 months, we obtained scores from the Wechsler Preschool and Primary Scales of Intelligence for full-scale intelligence quotient (FSIQ) verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ), and from the Beery-Buktenica Developmental Test for visual-motor integration (VMI). Possible predictive factors were collected prospectively from transplant listing.
Of the 76 patients included in the study, 61 survived to assessment, 2 were lost to follow-up and 4 were excluded for genetic disorders or heart-lung transplant. The CHD patients (n = 32) had significantly more previous surgeries, more severe kidney injuries, more days on ventilator and in intensive care, broader human leukocyte antigen (HLA) sensitization, longer cardipulmonary bypass (CPB) times and higher inotropic scores than CMP patients (n = 23). Mean IQ scores for the HTx children were below population norms and significantly lower in children with CHD. Intellectual disability (FSIQ <70) was more common in the CHD group (p = 0.036). The lower VMI in CHD patients approached significance. Lower FSIQ and VMI were independently associated with higher pre-HTx creatinine and lactate, longer stay in intensive care and lower socioeconomic status.
Children post-HTx showed IQ and VMI scores within the borderline to low-average range, with CHD children ranging significantly lower. Low scores are associated with a more difficult pre- and peri-transplant course. Careful follow-up is required to warrant early detection of deficits and introduction of interventions and supportive measures.
因先天性心脏病（CHD）或解剖结构正常的心脏衰竭（CMP）而需要心脏移植（HTx）的儿童在 HTx 前面临不同的挑战。我们比较了接受 HTx 治疗 CHD 与 CMP 的学龄前儿童的神经认知能力，并确定了预测结果的因素。
该数据来自 1999 年至 2011 年期间≤4 岁接受 HTx 的多省前瞻性项目。在 54±3 个月龄时，我们获得了韦氏学龄前和初级智力量表的全量表智商（FSIQ）、言语智商（VIQ）和操作智商（PIQ），以及比耶尔-布克蒂卡发育测验的视觉运动整合（VMI）的评分。前瞻性地从移植登记中收集了可能的预测因素。
在研究的 76 名患者中，61 名存活至评估，2 名失访，4 名因遗传疾病或心肺移植而被排除。CHD 患者（n=32）手术次数更多，肾脏损伤更严重，呼吸机使用时间和重症监护时间更长，人白细胞抗原（HLA）致敏更广泛，体外循环（CPB）时间更长，正性肌力药物评分更高，与 CMP 患者（n=23）相比。HTx 儿童的平均智商得分低于人群正常值，CHD 儿童的得分明显更低。智力障碍（FSIQ<70）在 CHD 组更为常见（p=0.036）。CHD 患者较低的 VMI 接近显著水平。较低的 FSIQ 和 VMI 与较高的 HTx 前肌酐和乳酸、较长的重症监护时间和较低的社会经济地位独立相关。
HTx 后儿童的智商和 VMI 得分处于边缘到低平均范围，CHD 儿童的得分明显更低。低分数与更困难的术前和围手术期过程相关。需要仔细随访，以确保早期发现缺陷，并引入干预和支持措施。
