Hövels-Gürich Hedwig H, Konrad Kerstin, Skorzenski Daniela, Nacken Claudia, Minkenberg Ralf, Messmer Bruno J, Seghaye Marie-Christine
Department of Pediatric Cardiology, Aachen University of Technology, Aachen, Germany.
Ann Thorac Surg. 2006 Mar;81(3):958-66. doi: 10.1016/j.athoracsur.2005.09.010.
The purpose of this prospective study was to assess whether neurodevelopmental status and exercise capacity of children 5 to 10 years after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy was different compared with normal children and influenced by the preoperative condition of hypoxemia or cardiac insufficiency.
Forty unselected children, 20 with tetralogy of Fallot and hypoxemia and 20 with ventricular septal defect and cardiac insufficiency, operated on with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass at a mean age of 0.7 +/- 0.3 years (mean +/- SD), underwent, at mean age 7.4 +/- 1.6 years, standardized evaluation of neurologic status, gross motor function, intelligence, academic achievement, language, and exercise capacity. Results were compared between the groups and related to preoperative, perioperative, and postoperative status and management.
Rate of mild neurologic dysfunction was increased compared with normal children, but not different between the groups. Exercise capacity and socioeconomic status were not different compared with normal children and between the groups. Compared with the normal population, motor function, formal intelligence, academic achievement, and expressive and receptive language were significantly reduced (p < 0.01 to p < 0.001) in the whole group and in the subgroups, except for normal intelligence in ventricular septal defect patients. Motor dysfunction was significantly higher in the Fallot group compared with the ventricular septal defect group (p < 0.01) and correlated with neurologic dysfunction, lower intelligence, and reduced expressive language (p < 0.05 each). Reduced New York Heart Association functional class was correlated with lower exercise capacity and longer duration of cardiopulmonary bypass (p < 0.05 each). Reduced socioeconomic status significantly influenced dysfunction in formal intelligence (p < 0.01) and academic achievement (p < 0.05). Preoperative risk factors such as prenatal hypoxia, perinatal asphyxia, and preterm birth, factors of perioperative management such as cardiac arrest, lowest nasopharyngeal temperature, and age at surgery, and postoperative risk factors as postoperative cardiocirculatory insufficiency and duration of mechanical ventilation were not different between the groups and had no influence on outcome. Degree of hypoxemia in Fallot patients and degree of cardiac insufficiency in ventricular septal defect patients did not influence the outcome within the subgroups.
Children with preoperative hypoxemia in infancy are at higher risk for motor dysfunction than children with cardiac insufficiency. Corrective surgery in infancy for tetralogy of Fallot or ventricular septal defect with combined circulatory arrest and low flow bypass is associated with reduced neurodevelopmental outcome, but not with reduced exercise capacity in childhood. In our experience, the general risk of long-term neurodevelopmental impairment is related to unfavorable effects of the global perioperative management. Socioeconomic status influences cognitive capabilities.
本前瞻性研究的目的是评估婴儿期接受法洛四联症或室间隔缺损矫正手术后5至10年的儿童，其神经发育状况和运动能力与正常儿童相比是否存在差异，以及是否受术前低氧血症或心脏功能不全状况的影响。
40名未经挑选的儿童，其中20名患有法洛四联症并伴有低氧血症，20名患有室间隔缺损并伴有心脏功能不全，平均年龄0.7 +/- 0.3岁（平均 +/- 标准差）时接受了联合深低温循环停搏和低流量体外循环手术，在平均年龄7.4 +/- 1.6岁时接受了神经状况、粗大运动功能、智力、学业成绩、语言和运动能力的标准化评估。对两组结果进行比较，并与术前、围手术期和术后状况及管理相关联。
与正常儿童相比，轻度神经功能障碍发生率有所增加，但两组之间无差异。与正常儿童相比以及两组之间，运动能力和社会经济状况无差异。与正常人群相比，整个组和各亚组的运动功能、形式智力、学业成绩以及表达性和接受性语言均显著降低（p < 0.01至p < 0.001），室间隔缺损患者的智力正常除外。法洛四联症组的运动功能障碍明显高于室间隔缺损组（p < 0.01），且与神经功能障碍、智力较低和表达性语言减少相关（各p < 0.05）。纽约心脏协会功能分级降低与运动能力降低和体外循环时间延长相关（各p < 0.05）。社会经济状况降低显著影响形式智力障碍（p < 0.01）和学业成绩（p < 0.05）。两组之间术前危险因素如产前缺氧、围产期窒息和早产，围手术期管理因素如心脏停搏、最低鼻咽温度和手术年龄，以及术后危险因素如术后心脏循环功能不全和机械通气时间均无差异，且对结果无影响。法洛四联症患者的低氧血症程度和室间隔缺损患者的心脏功能不全程度在亚组内不影响结果。
婴儿期术前有低氧血症的儿童比有心脏功能不全的儿童发生运动功能障碍的风险更高。婴儿期对法洛四联症或室间隔缺损采用联合循环停搏和低流量旁路进行矫正手术与神经发育结果降低相关，但与儿童期运动能力降低无关。根据我们的经验，长期神经发育损害的总体风险与围手术期整体管理的不利影响有关。社会经济状况影响认知能力。
