Daily life and psychosocial functioning of adults with congenital heart disease: a 40-53 years after surgery follow-up study.

INTRODUCTION: Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, knowledge about their psychosocial functioning is limited. METHODS: Longitudinal cohort study of patients (n = 204, mean age: 50 years, 46.1% female) who were operated during childhood (< 15 years) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot or transposition of the great arteries. Psychosocial functioning was measured every 10 years, using standardized and validated questionnaires. Results were compared with the general Dutch population and over time. RESULTS: After a median follow-up of 45 [40-53] years adults with CHD had a significantly lower educational level, occupation level and employment rate, but better health-related quality of life and emotional functioning compared with normative data. Patients with moderate/severe defects reported significantly more self-perceived physical restrictions and lack of physical strength due to their CHD. Compared to 2011, in 2021 patients considered their CHD as more severe and they felt more often disadvantaged. CONCLUSIONS: Overall, despite a lower education, occupation level and employment rate, our sample of patients with CHD had a positive perception of their life and  their psychosocial functioning was even better than the norm. Although the quality of life was very good, their view on their disease was more pessimistic than 10 years ago, especially for patients with moderate/severe CHD.

Impact of congenital heart surgery on quality of life in children and adolescents with surgically corrected Ventricular Septal Defect, Tetralogy of Fallot, and Transposition of the Great Arteries.

OBJECTIVE: To describe the impact of CHD surgery in early childhood on quality of life in children aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. METHOD: A cross-sectional survey study of quality of life survey on 161 children and adolescents aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. The international Paediatric Quality of Life 4.0 quality of life questionnaires were applied and collected for assessment from patients and parents. The endpoints were total, physical, emotional, social, and school quality of life scores. RESULTS: The quality of life total and school scores was significantly lower in children with CHD than their healthy peers. There was no significant difference in quality of life between the three CHD groups. All three CHD groups had a significantly lower total (7.7-13.2%, p<0.001) and school scores (21.1-31.6%, p<0.001) than the control group. The tetralogy of Fallot group was the only group that had significantly lower scores in the physical subscale (p<0.001) than the controls. CONCLUSION: Children and adolescents with surgically corrected CHD show losses in quality of life in total and school scores compared to healthy controls. The tetralogy of Fallot group was the only CHD group that had significantly lower physical score than the controls.

Longitudinal Associations between Neurodevelopment and Psychosocial Health Status in Patients with Repaired D-Transposition of the Great Arteries.

OBJECTIVE: To examine associations between measurements of neurodevelopment and psychosocial health status at age 8 and 16 years in patients with repaired dextro-transposition of the great arteries. STUDY DESIGN: In the 16-year follow-up of the Boston Circulatory Arrest Study, 137 parents completed the Child Health Questionnaire-Parent Form-50, of whom 135 had completed the Child Health Questionnaire-Parent Form-50 when their child was age 8 years. Psychosocial and physical summary scores were used to assess change in health status from age 8 to 16 years. A comprehensive battery of neurodevelopmental testing was performed at ages 8 and 16 years to examine associations with adolescent health status. RESULTS: Lower psychosocial summary scores of 16 year old subjects with dextro-transposition of the great arteries were highly associated with numerous concurrent domains of neurodevelopmental function, most notably with higher (worse) scores on the Conners' Attention Deficit Hyperactivity Disorder/Diagnostic and Statistical Manual-4th Edition Scales (parent: r = -0.62, P < .001; adolescent: r = -0.43, P < .001) and the Behavior Rating Inventory of Executive Function Global Executive Composite (parent: r = -0.66, P < .001; adolescent: r = -0.39, P < .001). Psychosocial and physical summary scores tracked from ages 8 to 16 years (r = 0.44 and 0.47, respectively, P < .001 for each). Higher (worse) scores of multiple attention measures at age 8 years predicted worse psychosocial summary scores at age 16 years. CONCLUSIONS: Attention deficits at age 8 years were highly predictive of worse psychosocial health status in adolescence. Further studies are needed to assess whether treatment of childhood attention deficit hyperactivity disorder could improve adolescent well-being.

Neurocognitive and Psychological Outcomes in Adults With Dextro-Transposition of the Great Arteries Corrected by the Arterial Switch Operation.

BACKGROUND: Neurodevelopmental impairments have frequently been described in children and adolescents with dextro-transposition of the great arteries (d-TGA). The arterial switch operation (ASO) to correct d-TGA has been used for more than 30 years, and more than 90% of these patients now reach adulthood. However, very little is known about their long-term functional outcomes. The present study investigated neurocognitive outcomes and the prevalence of psychiatric disorders in adults with d-TGA corrected by ASO. METHODS: Neurocognitive functioning was comprehensively assessed (general intellectual functioning, language, attention, visual-spatial skills, executive functions, memory) in 67 adults (59.7% men) with d-TGA (aged 22.9 ± 3.4 years) and in 43 healthy individuals. The prevalence of psychiatric disorders, including depression and anxiety, was evaluated using a structured diagnostic interview. We also analyzed patient- and operative-related risk factors associated with outcomes. RESULTS: Compared with the general population and the control group, adults with d-TGA displayed reduced performance in tasks assessing attention, visual-spatial skills, executive functions, and memory (all p < 0.05). Compared with controls, patients had also a higher lifetime prevalence of depression (43% vs 19%, p = 0.008) and anxiety disorders (54% vs 33%, p = 0.025). Predictors of long-term outcomes included gender and parental socioeconomic and educational status (all p < 0.05). CONCLUSIONS: Adults who have undergone a neonatal ASO to correct d-TGA have an increased risk of cognitive deficits and psychiatric disorders. Evaluation of long-term neuropsychological and psychosocial outcomes in early adulthood is a crucial step to anticipate for adapted treatment strategies in adults with congenital heart disease.

Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries.

The objective of this study is to investigate cognitive and attentional function in adolescents and young adults with operated congenital heart disease. Previous research has indicated that children with congenital heart disease have deficits in broad areas of cognitive function. However, less attention has been given to survivors as they grow into adolescence and early adulthood. The participants were 18 non-syndromic adolescents and young adults with tetralogy of Fallot and d-transposition of the great arteries that required cardiac surgery before the age of 5 years, and 18 healthy, unaffected siblings (11-22 years of age for both groups). Cases with congenital heart disease and their siblings were administered Wechsler Intelligence scales and reported attention problems using the Achenbach System of Empirically Based Assessments. Cases were compared to both healthy siblings and established norms. Cases performed significantly lower than siblings on full scale IQ and processing speed, and significantly lower than norms on perceptual reasoning. Cases also reported more attention problems compared to both siblings and norms. Effect sizes varied with medium-to-large effects for processing speed, perceptual reasoning, working memory, and attention problems. Findings suggest that neurocognitive function may continue to be affected for congenital heart disease survivors in adolescence and young adulthood, and that comparisons to established norms may underestimate neurocognitive vulnerabilities.

Facial expression recognition and emotion understanding in children after neonatal open-heart surgery for transposition of the great arteries.

AIM: Theory of mind impairments are part of the cognitive morbidities associated with transposition of the great arteries (TGA). We sought to assess core components of social cognition in school-aged children with TGA. METHOD: Thirty-eight children with neonatal corrected TGA (27 males, 11 females; mean age 7y 3mo, SD 1y 2mo) and a comparison group (n=31; 24 males, 7 females; mean age 7y 4mo, SD 1y 1mo) participated in this study. All children completed measures of facial expression recognition, emotion comprehension, and second-order cognitive and affective false-belief tasks. The association of medical pre-, intra-, and postoperative variables with cognitive outcomes was explored. RESULTS: After controlling for potential covariates, children with TGA performed significantly less accurately in the mental category of the emotion comprehension battery (p=0.002) and on second-order affective false-belief tasks (p<0.05). Preoperative variables including an associated ventricular septal defect (p=0.02), a younger age at open-heart surgery (p=0.03), and a prenatal diagnosis of TGA (p=0.02) were significantly associated with better outcomes. INTERPRETATION: School-aged children with TGA display significant impairment on complex affective mental state understanding even though facial expression recognition was generally preserved. Preoperative factors may be important determinants for long-term outcomes after cyanotic congenital heart disease.

Long-term psychosocial outcome of adults with tetralogy of Fallot and transposition of the great arteries: a historical comparison.

OBJECTIVE: To make a historical comparison on the long-term psychosocial outcome of cardiothoracic surgery during childhood. METHODS: Adult patients operated for tetralogy of Fallot or transposition of the great arteries between 1980 and 1990 (recent sample) were compared with patients who underwent surgery and were investigated 10 years earlier (historical sample). In addition, atrial switch and arterial switch patients within the recent sample were compared. Psychosocial functioning was measured using standardised, validated psychological questionnaires. RESULTS: Although the recent sample of patients overall shows a favourable quality of life, impairments were found in income, living conditions, relationships, offspring, and occupational level. Compared with the historical sample, the recent sample showed no significant improvements on psychosocial functioning, except for a better educational level. The amount of educational problems, such as learning difficulties, was still high compared with normative data. Recently operated patients with transposition of the great arteries (arterial switch) scored significantly better on the Short Form-36 vitality scale (p = 0.02) compared with historical patients with transposition of the great arteries (atrial switch). CONCLUSIONS: Despite improvements in medical treatment over the past few decades, hardly any change was found in the psychosocial outcome of the recent patient sample compared with the historical patient sample. In particular, the percentage of patients needing special education and showing learning problems remained high, whereas income was low compared with normative data.

In adults with atrial switch operation for transposition of the great arteries low physical activity relates to reduced exercise capacity and decreased perceived physical functioning.

OBJECTIVE: Patients with Mustard/Senning repair for transposition of the great arteries (d-TGA) have an impaired exercise capacity, but information about physical activity levels is scarce. The aim of the study was to come to a better understanding of the interrelationships between determinants of exercise capacity, physical activity and self-perceived health status. METHODS AND RESULTS: We studied 39 d-TGA patients who underwent echocardiography and exercise testing, and who completed questionnaires about physical activity and perceived health status. All results were compared with normal values. Stepwise multiple regression analysis was used to assess the determinants of peak V02% and exercise duration. Peak VO2 averaged 64 +/- 14.1% of the predicted normal values, which is significantly lower than expected (P < 0.0001). Physical activity measures as well as perceived general health were significantly lower compared to a reference population (P < 0.05). Peak heart rate and peak oxygen pulse explained together 90% of the variability in exercise duration (model F = 162.72; P < 0.0001). Type of surgery, total energy expenditure and right ventricular contractility were independent predictors of peak VO2% explaining 43% of the variability (model F = 8.75; P = 0.0002). CONCLUSIONS: Impaired peak exercise performance in d-TGA patients results mainly from the inability to increase stroke volume and heart rate at higher exercise intensities. Senning repair and a well-preserved right ventricular function are related to a better peak V02%. Furthermore, an active lifestyle has a positive effect on exercise capacity and perceived physical functioning. Therefore it might be indicated to encourage d-TGA patients to adopt a more physically active lifestyle.

Quality of life and perceived health status in adults with congenitally corrected transposition of the great arteries.

OBJECTIVE: The purpose of this study is to assess perceived health status and quality of life in adults with congenitally corrected transposition of the great arteries who have not undergone anatomic repair. METHODS: Quality of life as measured by the satisfaction with life scale and linear analog scales and perceived health status as measured by the Short Form 36 Health Survey (version 1) were evaluated in 25 adults with congenitally corrected transposition and compared with a control group of 25 adults with mild, hemodynamically insignificant defects. RESULTS: Instruments were returned by 83% of patients (25/30; 11 male; mean age, 44.6 ± 16 years). Health status by the linear analog scale was significantly lower (P = .03) in subjects (median, 80; range, 15-100) than in controls (median, 85; range, 65-100). Quality of life by the satisfaction with life scale was also lower (P = .009) in subjects (mean, 24 ± 8) compared with controls. Age was negatively correlated with the Short Form 36 Health Survey physical functioning (r = -0.41, P = .04), bodily pain (r = -0.5, P = .01), and physical component (r = -0.56, P = .004) summary scores in adults with congenitally corrected transposition but not in controls. CONCLUSIONS: Adults with congenitally corrected transposition have lower reported health status and satisfaction with life than a control population, with perceived health status declining with advancing age.

Health-related quality of life in patients with congenitally corrected transposition of the great arteries.

OBJECTIVE: The study's objectives were to survey the quality of life in patients with congenitally corrected transposition of the great arteries and to compare the responses of those who have undergone anatomic repair with those who have a systemic right ventricle. METHODS: Thirty-eight patients who underwent anatomic repair and 13 patients after either conventional repair or no surgical procedure were enrolled. Subjects completed the PedsQL 4.0 Generic and 3.0 Cardiac Modules. Scores were also compared with those of patients from the literature with at least moderately severe cardiovascular disease. Mean differences between groups were compared, and the association between clinical variables and score in the anatomic repair subgroup was measured. RESULTS: Caregivers of patients in the anatomic repair group reported similar scores compared with the non-anatomic repair group in all functional domains The anatomic repair group self-reported lower school function (63 vs 81, P = .02). On the Cardiac Module, patients in the anatomic repair group self-reported fewer problems related to residual heart disease (75 vs 63), appearance (81 vs 68), and treatment anxiety (74 vs 59), although the differences were not significant. Compared with patients with other heart disease, the anatomic repair group scored lower, with the largest differences in cognition and communication. Prolonged hospital stay and need for a pacemaker were associated with lower quality of life after anatomic repair. CONCLUSIONS: Patients in the anatomic repair group had similar quality of life compared with patients in the non-anatomic group, except in the domain of school functioning. Prolonged hospital stay and need for a pacemaker after anatomic repair may be risk factors for lower quality of life.

Long-term morbidity and quality of life after surgical repair of transposition of the great arteries: atrial versus arterial switch operation.

Since a growing number of patients after surgical repair of transposition of the great arteries (TGA) survive until adulthood the focus of attention has shifted to the management of associated long-term morbidity and quality of life (QoL). Therefore, we reviewed all patients that underwent surgical repair of TGA at our institution and compared long-term results after atrial and arterial switch operation. Between 1973 and 2000, a total of 302 patients underwent either atrial switch operation (n=222) or arterial switch operation (n=80). Mean follow-up was 14.5±10.1 years. The arterial switch repair was associated with a higher early mortality whereas long-term survival was comparable between both groups. Postoperative arrhythmias including loss of sinus rhythm and pacemaker implantation occurred significantly more often after atrial switch repair. There was a trend towards a more favourable outcome of the arterial switch group concerning freedom from re-interventions, severe systemic ventricular dysfunction and need for heart failure medication. However, also the arterial switch operation was associated with an increased incidence of loss of sinus rhythm and neo-aortic valve regurgitation during late follow-up. Health related QoL according to the SF-36 questionnaire was not significantly different between both groups and comparable to a healthy population.

Quality of life of grown-up congenital heart disease patients after congenital cardiac surgery.

BACKGROUND: Due to better early and long-term outcome, the increasing population of grown-ups with congenital heart disease (GUCH) brings up unexpected quality of life (QoL) issues. The cardiac lesion by itself is not always the major problem for these patients, since issues pertaining to QoL and psychosocial aspects often predominate. This study analyses the QoL of GUCH patients after cardiac surgery and the possible impact of medical and psychosocial complications. PATIENTS AND METHODS: A questionnaire package containing the SF-36 health survey (health related QoL), the HADS test (anxiety/depression aspects) and an additional disease specific questionnaire was sent to 345 patients (mean 26+/-11 years) operated for isolated transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and ventricular septal defect (VSD). The scores were compared with age- and gender-matched standard population data and in relation to the underlying congenital heart disease (CHD). RESULTS: In all SF-36 and HADS health dimensions the GUCH patients showed excellent scores (116+/-20), which are comparable to the standard population (100+/-15), regardless of the initial CHD (p=0.12). Eighty-two percent of the patients were found to be in NYHA class I and 83% patients declared that they do not consider their QoL to be limited by their malformation. Complications like reoperations (p=0.21) and arrhythmias (p=0.10) do not show significant impact on the QoL. The additional questionnaire revealed that 76% of adult patients have a fulltime job, 18% receive a full or partial disability pension, 21% reported problems with insurances, most of them regarding health insurances (67%), and 4.4% of adult patients declared to have renounced the idea of having children due to their cardiac malformation. CONCLUSION: QoL in GUCH patients following surgical repair of isolated TOF, TGA and VSD is excellent and comparable to standard population, this without significant difference between the diagnosis groups. However, these patients are exposed to a high rate of complications and special psychosocial problems, which are not assessed by standardized questionnaires, such as the SF-36 and HADS. These findings highlight the great importance for a multidisciplinary and specialized follow-up for an adequate management of these complex patients.

Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease.

The purpose of the current study was to assess the psychosocial outcomes of preschool-aged survivors (ages 3-6 years) of hypoplastic left heart syndrome (HLHS; n=13) and transposition of the great arteries (TGA; n=13). Parents completed the following measures: Pediatric Quality of Life Inventory, Impact on the Family Scale, Parenting Stress Index, Parent Behavior Checklist, and Child Behavior Checklist. Quality of life scores did not differ from those of healthy controls. Parents of children with HLHS reported more negative impact of the child's illness on the family and more parenting stress than parents of children with TGA. Parents of both groups of children were more permissive in their parenting style than parents of healthy controls. Children with HLHS had higher rates of attention and externalizing behavior problems than children with TGA. The results highlight the need for practitioners working with these children and families to ask about parental stress, family functioning, and behavioral expectations for the child in the context of routine medical/cardiac follow-up.

In the footsteps of senning: lessons learned from atrial repair of transposition of the great arteries.

The Senning operation has evolved from being the initial surgical correction that allowed survival in complete transposition of the great arteries to an integral part of the anatomic repair of congenitally corrected transposition. In patients with complete transposition, the Senning operation has given satisfactory initial and long-term surgical results, but the potential for right ventricular failure and atrial arrhythmias have drastically reduced its indications in the current era. The long-term follow-up and pertinent postoperative issues of the Senning operation will be reviewed, along with its newfound role in the anatomic repair of congenitally corrected transposition.

Visual-spatial skills in children after open-heart surgery.

This study was part of a randomized clinical trial comparing the central nervous system effects of the two vital organ-support methods used in infant cardiac surgery: total circulatory arrest and low-flow cardiopulmonary bypass. The extent to which visual-spatial deficits are (1). associated with surgical and perioperative variables, (2). attributable to visual-perceptual, motor control, or metacognitive deficits, and (3). associated with adaptive difficulties at home or school was evaluated. The subjects were 155 8-year-old children with D-transposition of the great arteries who underwent the arterial switch operation before 3 months of age. As part of a comprehensive evaluation, the Rey-Osterrieth Complex Figure (ROCF) was administered. ROCF copy productions were classified as having a Basal Organization Level of 1 (low) or 2 or greater. A five-category clinical rating was also assigned. More than half of the children in the cohort (52%) had copy productions scored at Level 1, more than twice the expected frequency. The risk of having a low score was not associated with vital organ support method or other surgical variables. On the basis of comparisons of the relative fits of nested logistic regression models, poor visual-perceptual abilities were more predictive of having a Level 1 score than either motor control or metacognitive deficits. Children with poor copy production scores had lower mathematics scores, but not lower reading scores or poorer parent and teacher ratings of adaptive competence. The percentage of children receiving remedial school services was associated with ROCF clinical rating, ranging from 58% in the worst category to 8% in the best category. Visual-spatial deficits are common among children after infant heart surgery and seem to reflect visual-perceptual rather than motor control or metacognitive deficits. In addition, these deficits do not seem to be clearly associated with the intraoperative methods or postoperative events evaluated.

Long term behavioural outcome after neonatal arterial switch operation for transposition of the great arteries.

AIMS: To evaluate behavioural outcome and quality of life in children aged 8-14 years after neonatal arterial switch operation for transposition of the great arteries. METHODS: Sixty children operated as neonates with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass were evaluated at age 7.9-14.3 years by the Child Behaviour Checklist (CBCL) and the Inventory for the Assessment of the Quality of Life in Children and Adolescents (IQCL). RESULTS: Parent reported behavioural outcome on all CBCL problem and competence scores was worse, whereas quality of life on self reported IQCL scores was not reduced compared to the normal population. On multivariate analysis, severe preoperative hypoxia was related to parent reported social problems; peri- and postoperative cardiocirculatory insufficiency was associated with internalizing, externalizing, attention, and total behavioural problems. Reduced expressive language was associated with total behavioural problems, and poor academic achievement was related to parent reported deficits in school performance. Impaired neurological status and reduced endurance capacity both predicted self reported stress by illness. CONCLUSIONS: The neonatal arterial switch operation with combined circulatory arrest and low flow bypass is associated with parent reported long term behavioural impairment, but not with self reported general reduction in quality of life. This discrepancy may be a result of different perception of illness. In our experience, increased risk of long term psychosocial maladjustment after neonatal corrective cardiac surgery is related to the presence of neurological impairment and reduced endurance capacity.

40 years after the first atrial switch procedure in patients with transposition of the great arteries: long-term results in Toronto and Zurich.

The atrial switch procedure dramatically improved the prognosis of children with complete transposition of the great arteries (TGA). Overall actuarial survival was approximately 75% after 25 years and was better in patients with simple TGA than in those with complex TGA. Mortality by any cause (16%) and cardiovascular mortality (12% and 13%) were comparable in both centers. Progressive congestive heart failure and sudden death were the principal modes of death. Most of the survivors denied any symptoms or had mild limitations in their daily activities. However, long-term problems in this growing population of adults are challenging and include late arrhythmias (up to two thirds of the patients), systemic ventricular (SV) failure, systemic atrioventricular valve regurgitation and reoperations, such as baffle reconstruction, being the most frequent. Objective assessment of SV function obtained by echocardiography is difficult. It may include fractional area change and tricuspid annular motion. Survivors after an atrial switch procedure are unique and have a good quality of life. However, the definitive and true history of the RV supporting the systemic circulation is not as yet known.

Long-term psychological outcome of children after surgery for transposition of the great arteries.

To assess the psychological consequences of a single congenital heart defect, we tested intellectual function, self-perception, "body image", child psychiatric symptoms and the family climate in 21 boys and 10 girls, at a mean age of 13.2 y, on average 11.5 y after surgery for transposition of the great arteries. Where applicable, test norms were used for comparison. WISC-R IQ tended to be slightly lower than that of the general population. Self-perception, as reflected on the "I think I am" test, was normal. "Body image" as measured by the "Draw-a-man" test was poor in the boys, but did not show a relationship with any other test tapping mental health. Six children (19%) had clinically significant child psychiatric symptoms, which is slightly more than expected, and were overrepresented in patients with poorer cardiac function. Five of these represented "internalizing" disorders. The patients' families scored higher than expected on the family climate "chaos" subscale, which has been demonstrated to be associated with the development of psychiatric symptoms. Overall, however, the children and their families were regarded as socially and psychologically well-functioning.

Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.

BACKGROUND: Deep hypothermia with either total circulatory arrest or low-flow cardiopulmonary bypass is used to support vital organs during heart surgery in infants. We compared the developmental and neurologic sequelae of these two strategies one year after surgery. METHODS: Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a method of support consisting predominantly of circulatory arrest or a method consisting predominantly of low-flow bypass. Developmental and neurologic evaluations and magnetic resonance imaging (MRI) were performed at one year of age. RESULTS: Of the 171 patients enrolled in the study, 155 were evaluated. After adjustment for the presence or absence of a ventricular septal defect, the infants assigned to circulatory arrest, as compared with those assigned to low-flow bypass, had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (a 6.5-point deficit, P = 0.01) and a higher proportion had scores < or = 80 (i.e., 2 SD or more below the population mean) (27 percent vs. 12 percent, P = 0.02). The score on the Psychomotor Development Index was inversely related to the duration of circulatory arrest (P = 0.02). The risk of neurologic abnormalities increased with the duration of circulatory arrest (P = 0.04). The method of support was not associated with the prevalence of abnormalities on MRI scans of the brain, scores on the Mental Development Index of the Bayley Scale, or scores on a test of visual-recognition memory. Perioperative electroencephalographic seizure activity was associated with lower scores on the Psychomotor Development Index (P = 0.002) and an increased likelihood of abnormalities on MRI scans of the brain (P < 0.001). CONCLUSIONS: Heart surgery performed with circulatory arrest as the predominant support strategy is associated with a higher risk of delayed motor development and neurologic abnormalities at the age of one year than is surgery with low-flow bypass as the predominant support strategy.

Cognitive development of children following early repair of transposition of the great arteries using deep hypothermic circulatory arrest.

Twenty-eight children who underwent corrective cardiac surgery in early infancy had developmental evaluations to explore whether cardiopulmonary bypass perfusion variables are associated with later cognitive function. All had transposition of the great arteries repaired by the arterial switch operation using deep hypothermic circulatory arrest. The mean duration of deep hypothermic circulatory arrest was 64 +/- 10 minutes (mean +/- SD). Median age at repair was 4 days (range 1 to 125 days). Tests of development were administered at age 7 to 53 months: Bayley Scales for children younger than 30 months of age (n = 18) and McCarthy Scales for older children (n = 10). Overall cognitive development score was 101.2 +/- 11.1. Duration of deep hypothermic circulatory arrest was not associated with performance. However, for core cooling periods of less than 20 minutes' duration, shorter cooling periods were associated with lower scores (r = .85, n = 11, P less than .001). These data suggest that patients undergoing relatively long periods of deep hypothermic circulatory arrest may require some minimum time of cardiopulmonary bypass cooling to avoid central nervous system injury.