Cardiopulmonary exercise testing in children following surgery for tetralogy of Fallot

The purpose of this study was to compare the cardiopulmonary responses at rest and during exercise of children following surgical correction of tetralogy of Fallot. The physiologic measurements assessed at rest and during maximal treadmill exercise included diffusing capacity of the lung for carbon monoxide, heart rate, oxygen consumption, minute ventilation, and cardiac output. Twenty children with repaired tetralogy of Fallot served as the study group, and 20 normal boys and girls served as controls. Subjects with tetralogy of Fallot had significantly lower values than those of control subjects for maximal cardiac output and somewhat lower values for diffusing capacity of the lung for carbon monoxide and heart rate. We conclude that the exercise cardiac function of children with surgically corrected tetralogy of Fallot is somewhat diminished compared with that of control children. This information is necessary to establish safe, yet effective exercise guidelines for this select population.     

小儿体外循环心内直视术后处理

先天性心脏病患儿由于其重要器官发育不成熟 ,各种代偿功能低下 ,在经受手术创伤及体外循环后所表现的脏器功能紊乱尤为明显 ,这类患儿的术后处理具有特殊性。现将我科自 1 993年 1月至2 0 0 3年 2月收治的体重 1 5kg以下小儿先天性心脏病术后处理体会报告如下。1　临床资料1 .     

Psychological adjustment and quality of life in children and adolescents following open-heart surgery for congenital heart disease: a systematic review

Background  

Children with congenital heart defects (CHD) requiring open-heart surgery are a group at high risk for health-related sequelae. Little consensus exists regarding their long-term psychological adjustment (PA) and health-related quality of life (QoL). Thus, we conducted a systematic review to determine the current knowledge on long-term outcome in this population.     

Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects

OBJECTIVES: To determine the prevalence of persistent developmental impairments in children with congenital heart defects and to identify factors that enhance risk for an adverse outcome. STUDY DESIGN: Eligible infants (n = 131) <2 years of age requiring open heart surgery were recruited prospectively. Subjects were assessed during surgery and again 12 to 18 months later with standardized developmental assessments and formal neurologic examinations. RESULTS: Mean age at follow-up testing was 19.1 +/- 6.6 months. Assessments indicated that 41% had abnormal neurologic examinations. Gross and/or fine motor delays were documented in 42%, and 23% demonstrated global developmental delay. Univariate and multiple regression models identified the following factors increasing the risk for persistent developmental deficits: preoperative and acute postoperative neurodevelopmental status and microcephaly, type of heart lesion, length of deep hypothermic circulatory arrest, age at surgery, and days in the intensive care unit (P <.05). CONCLUSIONS: Children with congenital heart defects commonly have ongoing neurologic, motor, and developmental deficits well after surgical correction. The cause is multifactorial and includes brain injury before, during, and after heart surgery.     

Psychological adjustment of families of children and adolescents treated for leukemia

This report describes a follow-up study in which the prevalence of emotional and behavioral problems in a group of 42 children and adolescents treated for leukemia is compared with the prevalence of problems in a matched control group selected from the general population. The prevalence of problems among the siblings of the two groups, and the adjustment of the two groups of families, are also examined. The Achenbach Child Behavior Checklist, for completion by parents, and the Achenbach Child Behavior Checklist and Rutter B2 Behavioural Scale, for completion by teachers, are used to identify both social competence and behavioral problems. In addition, the Family Concept Inventory is used to evaluate the adjustment of families. The leukemic children and adolescents were found to have significantly more problems and less social competence, particularly in school-related activities, than either the control group or their own siblings. There was no difference in the prevalence of problems between the two groups of siblings, nor between the two groups of families. It is suggested that careful prospective studies are needed to identify the cause of the problems experienced by the leukemic children and adolescents so that programs may be developed to prevent their emergence.     

缺血后处理对法洛四联症患儿的心肺保护作用

目的：缺血后处理能减轻缺血、再灌注损伤,但在心脏外科手术中的效果尚缺乏大组病例报道。该研究观察了缺血后处理对法洛四联症患儿的心肺保护作用。方法：将105例法洛四联症根治术患者随机分为常规组和处理组,常规组行常规手术,处理组给予缺血后处理,即间断夹闭升主动脉3次。术后以两组患者的ICU治疗时间、输血量、正性肌力药物评分、血流动力学、呼吸功能以及乳酸代谢作为评价指标。结果：与常规组比较,处理组ICU治疗时间明显缩短［（37±21）h vs （54±26）h］,输血量及正性肌力药物计分明显减少［（308±230）mL vs （526±515）mL和（5.9±5.0） vs （10.3±7.7）］,血流动力学和呼吸功能恢复更好,乳酸蓄积明显减少。结论：缺血后处理对法洛四联症根治术患儿有心肺保护作用。［中国当代儿科杂志,2009,11（4）：273-276］     

小儿先天性心脏病术后脓毒症发生的危险因素

目的：探讨小儿先天性心脏病（先心病）术后脓毒症发生的危险因素,以利于疾病早期认识和诊断,改善临床转归。方法回顾性分析我院胸外科重症监护室2012年1月至2015年4月间发生的52例先心病术后合并脓毒症患儿和1∶2配对的104例非脓毒症患儿的临床资料。对患儿年龄、性别、术前感染、延迟关胸、膈肌麻痹、二次开胸、长时间体外循环、留置多个有创导管方面进行Logistic回归分析,分析脓毒症发生的危险因素,计算OR值及其95％CI。结果先心病术后合并脓毒症患儿休克发生率高、功能受累脏器数目多,住院及ICU滞留天数长,病死率高,较非脓毒症患儿差异有统计学意义[25.32％vs.6.73％,（3.5±1.1）个vs.（1.1±0.7）个,（35.1±11.2）d vs.（11.3±3.1）d,（21.3±7.1）d vs.（7.1±2.3） d,19.23％ vs.4.81％,P＜0.05]。 Logistic回归分析显示术前合并感染、延迟关胸、二次开胸、留置多个有创导管,膈肌麻痹是小儿先天性心脏病术后脓毒症发生的危险因素, OR值（95％CI）分别为10.53（1.73,64.22）、26.66（2.69,263.83）、19.47（1.87,203.02）、4.99（1.361,8.31）、8.32（0.12,16.46）（P＜0.05）。结论小儿先心病术后脓毒症发生的危险因素有术前合并感染、延迟关胸、二次开胸、留置多个有创导管及膈肌麻痹。脓毒症患儿较非脓毒症患儿临床转归差。     

Quality of life in children with congenital heart defects

Quality of life was measured in children with congenital heart defects (CHDs) registered in a total population of infants born live in the period 1982–91 ( n = 22 810), using essential life spheres: external living conditions, interpersonal and personal conditions. In 200 children with CHD alive at the time the investigation was performed, 164 (82%) of the families answered a questionnaire addressing different dimensions of these quality of life spheres. Three subgroups of CHDs were investigated: CHDs spontaneously cured ( n = 80), CHDs treated by surgery ( n = 56), and CHDs with associated syndromes/ malformations ( n = 29). 301 (75%) out of 400 controls, matched for age and habitat (county), answered the same questionnaire. The children's ages at investigation were 2y 2 months-12y 2 months (median 6y 1 month). There were no statistically significant differences between the CHD groups and the controls for overall quality of life for any of the three life spheres ( p > 0. 05). In children with operated CHDs and CHDs associated with syndromes/malformations, quality of life was influenced at some aspects of the external as well as at the interpersonal and personal levels. A trend existed for a higher subjective experience of quality of life in the total CHD group as well as in all the subgroups. It is speculated that this may represent development of coping mechanisms and recalibration of values of life.     

Developmental screening of children diagnosed with congenital heart defects

Children with congenital heart disease (CHD) are more likely than normal children to have developmental delays. The development of 64 children with CHD less than 6 years old was screened with the Denver II. Thirty-five of the 64 children had CHD that required surgical or catheter intervention. These 35 children were significantly less likely than other children with CHD to be normal on developmental screening (46% vs 86%, respectively). Thirty-four percent of children with more severe CHD were referred for early intervention. As research shows the efficacy of early intervention, results indicate the need for early developmental evaluation of children with CHD of hemodynamic significance.     

法洛四联症术后心血管残余问题的研究进展

法洛四联症(Tetralogy of Fallot,TOF)是最常见的紫绀型先天性心脏病,约占先天性心脏畸形的10％。近年来,随着诊断技术、心脏外科和围手术期监护水平的综合提高,经手术TOF的预后明显改善,大部分患者可长期生存。然而,术后遗留的一些问题严重影响患者生活质量,已经引起重视。这些问题包括残余右室流出道梗阻(right ventricularoumow tract obstruction,RVOT0)、     

Neurologic status of newborns with congenital heart defects before open heart surgery

Controversy exists regarding the integrity of the nervous system in the newborn with a congenital heart defect who must undergo corrective or palliative open heart surgery. Neurodevelopmental sequelae have been primarily attributed to surgical procedures without standardized evaluation of the preoperative neurologic status. OBJECTIVE: To determine whether newborns with congenital heart defects demonstrate abnormalities in neurobehavioral status before surgery. STUDY DESIGN: In this prospective study, a standardized neonatal neurobehavioral assessment and a neurologic examination were conducted independently in a consecutive series of 56 neonates referred to our hospital for investigation of open heart surgery. RESULTS: Neurobehavioral and neurologic abnormalities were documented in greater than half of the cohort and included hypotonia, hypertonia, jitteriness, motor asymmetries, and absent suck. Poor state regulation (62%) and feeding difficulties (34%) also were commonly observed. Furthermore, 3 subjects had seizures, 35.7% were microcephalic, and 12.5% were macrocephalic. The overall likelihood of neurobehavioral abnormalities was not enhanced by indicators of cardiorespiratory compromise. Interestingly, newborns with acyanotic congenital heart defects were more likely to demonstrate neurologic compromise than were those with cyanotic defects. CONCLUSIONS: Findings suggest that the prevalence of neurobehavioral abnormalities before surgery in newborns with congenital heart defects has been underappreciated and would indicate that factors other than intraoperative procedures should be considered in the genesis of brain injury in this population.congenital heart defects, neurologic examination, newborn.     

Longer-term survival and health outcomes for children living with congenital heart defects

As mortality after major cardiac surgery in early childhood falls, it becomes clearer that there is a high prevalence of ‘non-cardiac’ problems in survivors. Patient support groups for cardiac children have long lobbied for better access to general paediatric expertise and upcoming organizational changes underwrite this. Motor and cognitive deficits as well as emotional and behavioural difficulties may need addressing; all impact the child's quality of life. Participation in exercise-based activities and quality of life correlate poorly with disease severity, except at the worst end of the spectrum of heart disease. Cardiac function as measured objectively poorly predicts children's activity in daily life and some children are actively limited by personal habit or family concern, which is often inappropriate. Enough is now known about the prevalence of problems that survivors of childhood cardiac surgery experience in the education system that support may be justified, even when the original cardiac condition is no longer an ongoing problem.     

Evaluation of oxidative stress in children with congenital heart defects

Background: A significant cause of death and chronic illness in childhood is caused by cardiovascular diseases, including congenital heart disease (CHD). This study aims to investigate the oxidative stress status and to establish its association with CHD in children. Methods: The study involves measurements of malondialdehyde (MDA), protein carbonyl (PCO), total anti‐oxidant capacity, high‐sensitive C‐reactive protein (hs‐CRP), fibrinogen and cytokine (interleukin [IL‐6] and tumor necrosis factor‐α) levels in 43 children with CHD and 30 healthy age‐matched children. Results: MDA, PCO, hs‐CRP, fibrinogen, IL‐6 and tumor necrosis factor‐α were significantly elevated while total anti‐oxidant capacity was significantly declined in patients compared with the controls. MDA was positively correlated with PCO, hs‐CRP, Qp/Qs and systolic pulmonary artery pressure. PCO was positively correlated with hs‐CRP, fibrinogen, IL‐6 and systolic pulmonary artery pressure. Conclusion: Oxidative stress and its association with other markers in children with CHD was established. To the best of our knowledge, this is the first time that PCO has been used as a biomarker in CHD and it may be employed as a new diagnostic biomarker in CHD and in the assessment of its severity.     

Effect of timing of surgery on the intellectual and psychosocial development of children with tetralogy of Fallot

Chronic hypoxemia in patients with Tetralogy of Fallot may influence intellectual and social development. Even though the physical situation is the most important factor for the time of operation, psychosocial aspects should be considered as well. 24 children underwent intelligence tests and questionnaires, 13 of them having had surgery in early infancy, 11 between 7 and 11 years of age. No significant difference was noted between the two groups concerning early childhood development and mean IQ. Early operation does appear to improve the social situation. Such children are able to participate in kindergarten, start school in time and pass through school without delay. These results suggest a possible beneficial relief of the stressed situation of the whole family.