|Year : 2018 | Volume
| Issue : 3 | Page : 91-96
Postcardiac surgery neurodevelopmental evaluation in children with congenital heart disease
Moustafa M Abdel Raheem1, Naji Y Safieldeen2
1 Department of Pediatrics, Faculty of Medicine, El Minia University, Minya, Egypt
2 Department of Internal Medicine, Faculty of Medicine, King Khalid University, Abha City, Saudi Arabia
|Date of Web Publication||15-Mar-2019|
Dr. Moustafa M Abdel Raheem
Assistant Professor of Pediatrics, Faculty of Medicine, El- Minia University, El-Minia City
Source of Support: None, Conflict of Interest: None
Background Congenital heart disease (CHD) is one of the most common congenital anomalies. It may carry risk of neurodevelopment (ND) delay. Open heart surgery has been widely introduced to those patients. The outcome of this surgery on patient’s development is unclear.
Objective The aim was to evaluate ND outcomes in children with CHD who underwent open heart surgery.
Patients and methods Fifty children with CHD were enrolled in this study: 23 were surgically repaired (group 1) and 27 had no surgical intervention (group 2). Twenty normal children of matched age and sex were enrolled in the study as controls (group 3). Bayley-III test was used to compare the ND parameters in all groups.
Results ND aspects showed lower significant differences in groups 1 and 2 when matched with the control group (P<0.05). However, there were no significant differences in ND parameters between groups 1 and 2 in gross motor and receptive communication parameters (P>0.05).
Conclusion CHD carries the risk of ND delay, and exposure to open heart surgery increases this risk.
Keywords: cardiac surgery, congenital heart disease, neurodevelopment
|How to cite this article:|
Abdel Raheem MM, Safieldeen NY. Postcardiac surgery neurodevelopmental evaluation in children with congenital heart disease. Alex J Pediatr 2018;31:91-6
|How to cite this URL:|
Abdel Raheem MM, Safieldeen NY. Postcardiac surgery neurodevelopmental evaluation in children with congenital heart disease. Alex J Pediatr [serial online] 2018 [cited 2019 Mar 23];31:91-6. Available from: http://www.ajp.eg.net/text.asp?2018/31/3/91/254218
| Introduction|| |
Congenital heart disease (CHD) is considered one of the most common congenital anomalies and leads to significant childhood morbidity and mortality. Its incidence ranges from 4 to 12 per 1000 live births . Survival rates of children with CHD have been increased owing to recent lines of treatment, including noninvasive imaging, medical treatment, cardiac catheterization, advanced surgical techniques, progress in cardiopulmonary bypass, and postsurgical intensive care . Many studies showed different neurodevelopmental (ND) disabilities in infants and children with CHD. There is high prevalence and low severity affection of motor integration, skills, visual attention, executive function, and behavioral changes . American Heart Association and American Academy of Pediatrics clarified guidelines that classified patients with CHD as those at high risk for ND delay and suggested behavioral assessment and adaptive function screening among these children ,. One of the most reliable and commonly used tests for ND evaluation is the 3rd edition of Bayley Scales of Infant and Toddler Development Screening Test (Bayley-III). Its first description was in 1969, revised in 1993, and finally standardized in 2006 . Bayley-III test is constructed to evaluate cognitive, language, and global motor developmental milestones in both infants and children. Two subscores for language scale are used for language evaluation, expressive and receptive communications, and another two subscales are used to evaluate motor development, fine and gross motor .
| Aim|| |
This study was designed to evaluate ND aspects of children with CHD who underwent open heart surgery.
| Patients and methods|| |
In this comparative cross-sectional study, 50 infants and children with CHD and 20 as controls were included. The participants of the study were classified into three groups:
- Group 1: −23 patients with CHD who underwent open heart surgery, with an age range between 13 and 39 months and mean age of 24.1±12.9 months.
- Group 2: 27 patients with CHD without surgical intervention, with an age range between 15 and 38 months, and mean age of 22.3±13.5 months.
- Group 3 (control group): 20 healthy infants and children of matched age and sex, with an age range of 12–34 months and mean age 24.3±11.6 months.
Patients were selected from those coming for regular follow-up in Pediatric Cardiology Clinic, Almahla Hospital for Children, Abha City, Saudi Arabia. Controls were selected from those who were hemodynamically stable attending the well baby clinic at the same hospital. The study was approved by the hospital research ethical committee. The study was conducted between January 2016 and October 2016. A written consent was taken from parents of the participant according to criteria of research ethical committee of Almahla Hospital for Children.
Patients with CHD who underwent open heart surgery with postoperative duration of 6–12 months before the study (group 1) and those with CHD, hemodynamically unstable with no previous surgical history (group 2) were included. According to clinical and echo findings, the unstable hemodynamic status was clarified for children who were in need for medical treatment, surgical operation, or invasive intervention.
Children with history of birth asphyxia, prematurity, hypoglycemia, and recurrent convulsions, and those with neurological disorders, previous history of cerebral embolic manifestations, chronic malnutrition, chronic systemic disease, or a genetic syndrome were excluded from the study.
All patients were subjected to thorough clinical history and examination. Anthropometric measurements, including height (or length), weight, head, and mid arm circumference, were assessed by the same well-trained physician. All measurements were plotted on Saudi growth charts curves. BMI (kg/m2) was calculated for each. Chest radiography and ECG were done for all patients. Echocardiographic evaluation was done by the same person for all included infants and children by Vivid 5 Echo (GE Healthcare Company, Norway) machine to confirm diagnosis and search for postoperative residual lesions.
Bayley-III test was used to evaluate cognitive, language, and global motor development for both patients and controls. Items included in Bayley cognitive scale are information processing, information processing speed, problem solving, number concepts in addition to play skills. Language scales include two aspects: receptive (hearing ability, understanding, and response) and expressive (communication skills). Motor subtests include movement quality, sensory and perceptual motor integration, and basic milestones. Each subscale’s range of Bayley-III test scores is 40–160 and has mean of 100 with SD of 15 . Infants with developmental delay on Bayley scales were identified as indicated by scores less than 85 on the cognitive, language, or global motor composite scores . Test was done and scored by well-experienced person in psychiatry unit.
SPSS 18 for Windows 10 (SPSS Inc., Chicago, Illinois, USA) was used for data analysis. Probability values of P less than or equal to 0.05 were considered to be significant. Data were expressed in mean, SD, and range with minimum and maximum values to compare between different groups. Categorical variables between groups were analyzed by χ2 contingency tables. Normality of distribution of continuous variable was tested by Shapiro–Wilk test. Normally distributed data were compared using independent T-test. Non- parametric variables were compared using Mann–Whitney U-test.
| Results|| |
A total of 70 children were included in this study. They were divided into three groups. Group 1 included 23 children who previously underwent open heart surgery group with postoperative duration of 6 to 12 months before the study; two (8.7%) cases of them were exposed to two trials of corrections. According to the data recorded in the follow-up sheets, all patients were exposed to anesthesia for a duration range of 2–4 h, and no cases were arrested during or after surgery and survived after resuscitation. Other surgical details were not available as operations were done in other centers not in our hospital. Group 2 included 27 children, representing the nonoperated CHD group, and group 3 was the control group (20 children).
[Table 1] shows some demographic data and anthropometric measurements of the studied groups. The three groups were age and sex matched (P>0.05). Mean values of the weight in the control group was significantly higher when matched with group 1 and group 2 (P=0.03 and 0.04, respectively), whereas there was no significant difference between group 1 and 2 (P=0.35).
The height (length) in the control group was significantly higher when matched with group 1 and group 2 (P=0.025 and 0.042, respectively) whereas there was no significant difference between groups 1 and 2 (P=0.091).
BMI mean value was significantly higher in the controls when matched with group 1 (previously operated children) (P=0.02). Head circumference measurements showed no significant differences between groups 1 and 2 (P=0.6), whereas it was significantly higher in controls when matched with groups 1 and 2 (P=0.04 and 0.04).
Echocardiographic diagnoses of children with CHD are shown in [Table 2]. There were 13 cyanotic cases in group 1, whereas there were 14 cyanotic cases in group 2, with no significant difference in distribution of cyanotic cases between the two groups (P=0.1).
|Table 2 Echocardiographic diagnosis of the two groups of congenital heart disease|
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[Table 3] and [Figure 1] show the results of mean scores of Bayley-III test in the different groups. Group 1 (previously operated patients) showed significantly lower values in all test parameters, i.e., cognitive, language, receptive communication, expressive communication, global, growth, and fine motor, when matched with the control group (P=0.002, 0.001, 0.01,0.02, 0.001, 0.004, and 0.02, respectively). Group 2 showed lower significant values in cognitive, language, receptive communication, expressive communication, and fine motor subtest (P=0.04, 0.035, 0.015, 0.03, and 0.01, respectively) when compared with the control group. Global and gross motor values were insignificantly less (P=0.06 and 0.3, respectively). When compared with group 2, previously operated patients (group 1) showed significant lower values in cognitive, language, expressive communication, global, and fine motor subtest (P=0.03, 0.04, 0.04, 0.02, and 0.04, respectively). Receptive communication and gross motor were lower but not statistically significant.
|Table 3 Mean scores of Bayley-III test in the studied groups of congenital heart disease and controls|
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|Figure 1 Mean scores of Bayley-III test in the studied groups of congenital heart disease and controls.|
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[Table 4] shows the difference of Bayley-III results between noncyanotic and cyanotic studied patients. Noncyanotics showed statistically significant higher values regarding cognitive, language, global, and gross motor when compared with cyanotic patients (P=0.04, 0.03, 0.04, and 0.02, respectively). Other parameters were insignificantly different ([Table 4]).
|Table 4 Mean scores of Bayley-III test in children with congenital noncyanotic and cyanotic heart disease|
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| Discussion|| |
With improvement of pediatric cardiology, open heart surgery techniques, and postoperative intensive care, there are higher survival rates in children with CHD especially in those who underwent open cardiac surgery . ND of postoperative surviving patients is affected by many heterogeneous factors. During cardiopulmonary bypass surgery, the candidate’s brain may be subjected to either focal or global ischemia attributed to gaseous and particulate microemboli or hypoperfusion .
In the current study, we compared ND status of post-open heart surgery patients with nonoperated patients with CHD and in comparison with normally age-matched and sex-matched children using Bayley-III test as an indicator, in a trial to verify the effect of surgical procedure on ND status in children with CHD. Results showed that postoperative patients had statistically significant lower values regarding weight, height, and head circumference when compared with nonoperated and control groups. BMI also showed statistically significant lower values between operated patient and controls. Mean BMI levels of the patient groups were affected owing to both weight and height (length) affection. Growth parameters in patients with CHD were also affected as recorded by Olgu et al.  and Costello et al. , who found that all anthropometric parameters were lower and remained an ongoing problem in these children ,. Feeding problems represent a very important factor in these findings as assumed by Kathleen et al. . They found that hospital stay associated with prolonged tube feeding had good evidence with growth parameters’ decrement.
Moreover, they found that starting with structured early feeding strategy had the ability to improve outcomes. Many other factors may affect physical growth in patients with CHD including affected birth weight and co-morbidities like recurrent chest infections and feeding difficulties, especially with chronic heart failure. Open heart surgery represents a strong burden on infant feeding and psychological status, hence it may affect physical growth . Time of cardiopulmonary bypass more than 200 min or prolonged postoperative hospital stay more than 30 days with prolonged maternal deprivation were found to carry high risk of postoperative suppression of growth parameters. These events are associated with an increased total energy expenditure ,.
In the present study, we found that all Bayley-III test parameters are affected in patients with CHD. Olgu et al.  also studied a group of patients with CHD, and they noticed that CHD itself is a risk factor in ND delay in their studied cases. Children with CHD have less physical ability for interaction with environment, which leads to limitation of their exploratory behavior in addition to an increased maternal protectiveness that limits the child’s social interaction. Hospital admissions for long time also interfere with psychomotor development through physical and human activities restriction . Khalil et al.  concluded from their meta-analytic study that there is an association between CHD and a relatively high prevalence of neuroimaging brain lesions.
These finding were CHD type dependent as they found that the prevalence of brain lesions depends on the type of CHD, varying from 34% in cases of TGA to 49% in cases of left-sided heart lesions. On comparison with nonoperated patients with CHD, our study showed that all Bayley-III test parameters are affected in previously operated patients with CHD. These findings are in agreement with Walker et al. , who studied the effect of early major surgery on development of these patients and they recorded similar results. Low-flow bypass and Deep Hypothermic Circulatory Arrest Technique used during correction of complex congenital heart anomalies in early life may themselves contribute to neurological damage in these patients . Moreover, the details of the conduct of cardiopulmonary bypass including poor management of arterial blood gas in relation to temperature and cooling-warming extent, may lead to potential brain injury postoperatively . Surgical burden was proved in the study by Bellinger et al.  on adolescents with corrected TGA and on corrected F4 patients as they exhibit higher rates of structural brain MRI abnormalities than controls .In the current study, we also found that cyanotic children had less scores in cognitive, language, global, and fine motor Bayley-III subtests, which is in agreement with Adam et al. , who demonstrated that children with critical cyanotic heart disease are at increased risk for executive function defects. This may be attributed to the fact that patients with cyanotic CHD have problems with inhibitory control, cognitive flexibility, working memory, and social adoption  which are related to postnatal perfusion and oxygenation disturbance in these patients . Patients with complex CHD are usually exposed to more time for surgery either for one setting or more which carry more exposure to general anesthesia with more risk on the brain . These ND changes may proceed to adult life as one-third of adults with CHD in North America develop mood or anxiety disorders .
Regarding these data, many factors affect post-open heart ND status, including the preoperative general condition and co-morbidities and nature of CHD. Type of operation, anesthesia time exposure, postoperative complications, and hospital stay are also important factors determining the ND outcome.
To our knowledge, previous studies did not compare the different three groups with each other.
All patients were operated upon in centers away from our hospital. They came to our hospital’s echocardiography clinic for follow-up. We did not attend the surgery or postoperative events so many data about operation or postoperative events were difficult to collect.
In conclusion, children with CHD have risk of ND delay. This risk increases with exposure to open heart surgery and complexity of the defect. ND evaluation and follow-up is recommended in these patients to detect early changes.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Egbe A, Uppu S, Stroustrup A, Lee S, Ho D, Srivastava S. Incidence and sociodemographics of specific congenital heart disease in the United States of America: an evaluation of hospital discharge diagnoses. Pediatr Cardiol 2014;35:975–982.
Marelli AJ, Ionesscu-Ittu R, Mackie AS, Guo I, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in general population from 2000-2010. Circulation 2014; 130:749–756.
Bellinger DC, Wypij D, Rivkin MJ, DeMaso DR, Robertson RL, Dunbar-Masterson C et al.
Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation 2011;124:1361–1369.
Sananes R, Manlhiot C, Kelly E, Hornberger LK, Williams WG, MacGregor D et al.
Neurodevelopmental outcomes after open heart operations before 3 months of age. Ann Thorac Surg 2012;93:1577–1583.
Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW et al.
Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012;126:1143–1172.
Bayley N. Bayley scales of infant and toddler development-third edition screening test manual. Oxford: Psych Corp; 2006.
Anderson JA, Burnett A. Assessing developmental delay in early childhood concerns with the Bayley-III scales. Clin Neuropsychol 2017;31:371–381.
Komur M, Ozen S, Okuyaz C, Makharoblidze K, Erdogan S. Neurodevelopmental evaluation in children with congenital hypothyroidism by Bayley-III. Brain Dev 2013;35:392–397.
Mahle WT, Tavani F, Zimmerman RA, Nicolson SC, Galli KK, Gaynor JW et al.
An MRI study of neurological injury before and after congenital heart surgery. Circulation 2002;106:109–114.
Olgu H, Guliz G, Gulcin B, Derya K, Khatuna M, Cetin O. Evaluation of neurodevelopment using Bayley-III in children with cyanotic or hemodynamically impaired congenital heart disease. Congenit Heart Dis 2015;10:537–541.
Costello CL, Gellatly M, Daniel J, Justo RN, Weir K. Growth restriction in infants and young children with congenital heart disease. Congenit Heart Dis 2015;10:447–456.
Kathleen A, Raymond G, James S, George M, Laurel B, Yumei C et al.
Risk and prevalence of developmental delay in young children with congenital heart disease. Pediatrics 2014;133:570–577.
Jillian CT, Irving SY, Papas MA, Hollowell C, Ravishankar C, Marino BS et al.
Total energy expenditure of infants with congenital heart disease who have undergone surgical intervention. Ped Cardiol 2015;36:1670–1679.
Dittrich H, Bührer C, Grimmer I, Dittrich S, Abdul-Khaliq H, Lange P. Neurodevelopment at 1 year of age in infants with congenital heart disease. Heart 2003;89:436–441.
Khalil A, Suff N, Thilaganathan B, Hurrell A, Cooper D, Carvalho J. Brain abnormalities and neurodevelopmental delay in congenital heart disease: systematic review and meta-analysis. Ultrasound Obstet Gynecol 2014;43:14–24.
Walker K, Halliday R, Badawi N, Stewart J, Holland AJ. Early developmental outcomes following major noncardiac and cardiac surgery in term infants: a population-based study. J Pediatr 2012;161:748–752.
Dean B, Stephen A, Laura K, Chandra R. Neurological monitoring for congenital heart surgery. Anesth Analg 2004;99:1365–1375.
Wernovsky G, Newburger J. Neurologic and developmental morbidity in children with complex congenital heart disease. J Pediatr 2003;142:6–8.
Bellinger D, Rivkin M, DeMaso D, Robertson R, Stopp C, Dunbar-Masterson C et al.
Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging. Cardiol Young 2014;11:1–10.
Adam RC, Matthew TW, David RM, Jane WN, David CB. Executive function in children and adolescents with critical cyanotic congenital heart disease. J Int Neuropsychol Soc 2015;20:34–49.
Diamond A. Executive functions. Ann Rev Psychol 2013;64:135–168.
Jevtovic-Todorovic V, Absalom AR, Blomgren K, Bambrink A, Crosby G, Culley DJ et al.
Anaesthetic neurotoxicity and neuroplasticity: an expert group report and statement based on the BJA. Salzburg Seminar. Br J Anaesth 2013;111:143–151.
Kovacs AH, Utens EM. More than just the heart. Cardiol Clin 2015;33:625–634.
[Table 1], [Table 2], [Table 3], [Table 4]