Reich Bettina, Heye Kristina, Tuura Ruth, Beck Ingrid, Wetterling Kristina, Hahn Andreas, Hofmann Karoline, Schranz Dietmar, Akintürk Hakan, Latal Beatrice, Knirsch Walter
Pediatric Heart Center, University Hospital, Giessen, Germany.
Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland; Children's Research Center, University Children's Hospital, Zurich, Switzerland.
Semin Thorac Cardiovasc Surg. 2017 Dec 5. doi: 10.1053/j.semtcvs.2017.09.014.
Neurodevelopmental impairment and impaired quality of life constitute a major source of morbidity among children with complex congenital heart disease, in particular for single-ventricle (SV) morphologies. Risk factors and quality of life determining clinical and neurodevelopmental outcome at 2 years of age are examined. In a 2-center cohort study, 48 patients with SV morphology (26 hypoplastic left heart syndrome and 22 other types of univentricular heart defect) have been examined before Fontan procedure between 2010 and 2015. Patients were assessed with the Bayley Scales of Infant and Toddler Development, Third Version (Bayley-III), and the Preschool Children Quality of Life (TAPQOL) questionnaire. A total of 44 patients underwent hybrid procedure (n = 25), Norwood procedure (n = 7), or shunt or banding procedure (n = 12) as first surgery before subsequent bidirectional cavopulmonary anastomosis (n = 48). Median cognitive, language, and motor composite scores on the Bayley-III were 100 (range 65-120), 97 (68-124), and 97 (55-124), respectively. The language composite score was significantly below the norm (P = 0.025). Risk factors for poorer neurodevelopmental outcome were prolonged mechanical ventilation, longer days of hospital stay, and more reinterventions (all P < 0.05). Parents reported a good quality of life for their children. Children undergoing Fontan procedure show a favorable development and good quality of life. More complicated postoperative course and reinterventions constitute risk factors for impaired neurodevelopment. Improving postoperative management and implementing routine follow-up assessments aremeasures to further improve the neurodevelopmental outcome of this high-risk patient population.
神经发育障碍和生活质量受损是复杂先天性心脏病患儿发病的主要原因，尤其是单心室（SV）形态的患儿。本文研究了2岁时决定临床和神经发育结局的危险因素及生活质量。在一项双中心队列研究中，对2010年至2015年间48例SV形态的患者（26例左心发育不全综合征和22例其他类型的单心室心脏缺陷）在Fontan手术前进行了检查。采用贝利婴幼儿发展量表第三版（Bayley-III）和学龄前儿童生活质量（TAPQOL）问卷对患者进行评估。共有44例患者在随后的双向腔肺吻合术（n = 48）之前接受了混合手术（n = 25）、诺伍德手术（n = 7）或分流或束带手术（n = 12）作为首次手术。Bayley-III上的认知、语言和运动综合得分中位数分别为100（范围65 - 120）、97（68 - 124）和97（55 - 124）。语言综合得分显著低于正常水平（P = 0.025）。神经发育结局较差的危险因素包括机械通气时间延长、住院天数增加和再次干预次数增多（均P < 0.05）。家长报告其子女生活质量良好。接受Fontan手术的儿童显示出良好的发育和生活质量。术后病程更复杂和再次干预是神经发育受损的危险因素。改善术后管理并实施常规随访评估是进一步改善这一高危患者群体神经发育结局的措施。
