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.

An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease.

OBJECTIVE: Prenatal exposures are known to alter fetal neurodevelopment and autonomic control. We aimed to explore the correlation between fetal autonomic activity, measured by fetal heart rate variability, and 18-month developmental outcome in subjects with congenital heart disease. STUDY DESIGN: From 2010 to 2013, 5 fetuses with hypoplastic left heart syndrome, 9 with transposition of the great arteries and 9 with tetralogy of Fallot were included in this prospective cohort study. A maternal abdominal fetal electrocardiogram monitor recorded fetal heart rate at 34 to 38 weeks gestational age. We assessed associations between fetal heart rate parameters including interquartile range and s.d. of the fetal RR intervals and 18-month Bayley Scales of Infant Development-III scores using Pearson's correlation coefficient. Multivariable regression modeling identified predictors of neurodevelopmental scores. RESULTS: Fetal heart rate variability parameters at 34 to 38 weeks gestational age correlated with 18-month Cognition (r=0.47, P=0.03) and Motor scores (r=0.66, P=0.001). The interquartile range of the fetal RR intervals predicted Cognition (ß=0.462, P=0.028, R2=0.282) and Motor (ß=0.637, P<0.001, R2=0.542) scores. CONCLUSIONS: In fetuses with congenital heart disease, low heart rate variability at 34 to 38 weeks gestational age predicts diminished 18-month Cognitive and Motor performance. Prenatal autonomic activity may serve as a marker of early childhood development in these high-risk patients.

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.

Hippocampal damage and memory impairment in congenital cyanotic heart disease.

Neonatal hypoxia can lead to hippocampal atrophy, which can lead, in turn, to memory impairment. To test the generalizability of this causal sequence, we examined a cohort of 41 children aged 8-16, who, having received the arterial switch operation to correct for transposition of the great arteries, had sustained significant neonatal cyanosis but were otherwise neurodevelopmentally normal. As predicted, the cohort had significant bilateral reduction of hippocampal volumes relative to the volumes of 64 normal controls. They also had significant, yet selective, impairment of episodic memory as measured by standard tests of memory, despite relatively normal levels of intelligence, academic attainment, and verbal fluency. Across the cohort, degree of memory impairment was correlated with degree of hippocampal atrophy suggesting that even as early as neonatal life no other structure can fully compensate for hippocampal injury and its special role in serving episodic long term memory. © 2017 Wiley Periodicals, Inc.

Processing speed, executive function, and academic achievement in children with dextro-transposition of the great arteries: Testing a longitudinal developmental cascade model.

OBJECTIVE: To establish executive function (EF) structure/organization and test a longitudinal developmental cascade model linking processing speed (PS) and EF skills at 8-years of age to academic achievement outcomes, both at 8- and 16-years, in a large sample of children/adolescents with surgically repaired dextro-transposition of the great arteries (d-TGA). METHOD: Data for this study come from the 8- (n = 155) and 16-year (n = 139) time points of the Boston Circulatory Arrest Study and included WISC-III, Trail Making Test, Test of Variables of Attention, and WIAT/WIAT-II tasks. RESULTS: A 2-factor model (Working Memory/Inhibition and Shifting) provided the best fit for the EF data, χ²(3) = 1.581, p = .66, RMSEA = 0, CFI = 1, NNFI = 1.044). Working Memory/Inhibition and Shifting factors were not correlated. In the structural equation model, PS was directly related to both EF factors and Reading at 8 years, and was indirectly related to Math and Reading achievement, both concurrently and longitudinally, via its effects on Working Memory/Inhibition. Shifting at 8 years was significantly associated with Math (but not Reading) at 16 years. CONCLUSIONS: The academic difficulties experienced by children and adolescents with d-TGA may be driven, at least in part, by underlying deficits in processing speed and aspects of executive function. Intervention efforts aimed at bolstering these abilities, particularly if implemented early in development, may prove beneficial in improving academic outcomes and, perhaps by extension, in reducing the stress and diminished self-confidence often associated with academic underachievement. (PsycINFO Database Record

Fetal Growth and Neurodevelopmental Outcome in Congenital Heart Disease.

We evaluated differences in growth between fetuses with and without congenital heart disease (CHD) and tested associations between growth and early childhood neurodevelopment (ND). In this prospective cohort study, fetuses with hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and tetralogy of Fallot (TOF) and controls had biparietal diameter (BPD), head (HC) and abdominal circumference (AC), femur length (FL), and estimated fetal weight (EFW) recorded serially during pregnancy at 18-26 weeks GA (F1), at 27-33 weeks GA (F2), and at 34-40 weeks GA (F3). CHD subjects underwent Bayley Scales of Infant Development-III ND testing at 18 months. Differences between CHD fetuses and controls were assessed using t tests and generalized linear modeling. Correlations between biometry and ND informed regression modeling. We enrolled 41 controls and 68 fetuses with CHD (N = 24 HLHS, N = 21 TGA, N = 23 TOF), 46 of whom had ND scores available. At 18-26 weeks, CHD fetuses were smaller than controls in all biometric parameters. Differences in growth rates were observed for HC, BPD, and AC, but not for FL or EFW. Cognitive score correlated with HC/AC at F2 (r = -0.33, P = 0.04) and mean HC/AC across gestation (r = -0.35, P = 0.03). Language correlated with FL/BPD at F2 (r = 0.34, P = 0.04). In stepwise linear regression, mean HC/AC predicted Cognition (B = -102, P = 0.026, R (2) = 0.13) and FL/BPD at F2 predicted Language score (B = 127, P = 0.03, R (2) = 0.12). Differences in growth between CHD fetuses and controls can be measured early in pregnancy. In CHD fetuses, larger abdominal relative to head circumference is associated with better 18-month neurodevelopment.

Maternal-infant interaction and autonomic function in healthy infants and infants with transposition of the great arteries.

The quality of maternal-infant interaction is a critical factor in the development of infants' autonomic function and social engagement skills. In this secondary data analysis, relationships among infant and maternal affect and behavior and quality of dyadic interaction, as measured by the Parent-Child Early Relational Assessment, and infant autonomic function, as measured by heart rate variability, were examined during feeding at 2 weeks and 2 months of age in 16 healthy infants and in 15 infants with transposition of the great arteries (TGA). Contrary to previous research, at 2 weeks infant age, mothers of infants with TGA had significantly higher scores in affect and behavior than did mothers of healthy infants. The affect and behavior and quality of dyadic interaction of infants with TGA also did not differ from that of healthy infants. Although infants' social engagement skills did not differ by health condition (TGA or healthy), these skills did differ by parasympathetic nervous system function: infants better able to suppress vagal activity with challenge had more positive and less dysregulated affect and behavior, regardless of health status. These findings suggest that maternal-infant interactions for some cardiac disease subgroups may not differ from healthy dyads. Additional research is required to identify both healthy and ill infants with delayed autonomic maturation and to develop and test interventions to enhance critical interactive functions.

Executive functions development in 5- to 7-year-old children with transposition of the great arteries: a longitudinal study.

This longitudinal study investigates executive functions (EF) in children with transposition of the great arteries (TGA) compared to typically developing children at a key age period between 5 and 7 years. We explored the presence and evolution of specific impairments on three core EF components (inhibition, working memory, and cognitive flexibility). Ninety children were evaluated for three consecutive years. Results demonstrated significant delays in inhibition and cognitive flexibility despite normal working memory. Impairments did not systematically worsen with age. EF impairments after TGA are dynamic and may affect selective components. Cyanotic congenital heart disease is associated with altered EF development.

Psychiatric disorders and function in adolescents with d-transposition of the great arteries.

OBJECTIVE: To compare adolescents with d-transposition of the great arteries (d-TGA) with healthy adolescents with respect to prevalence of psychiatric disorders and global psychosocial functioning. STUDY DESIGN: Subjects, consisting of 139 adolescents with d-TGA (16.1 ± 0.5 years) and 61 healthy adolescents (15.3 ± 1.1 years) without known risk factors for brain disorders, underwent a battery of assessments, including semistructured psychiatric interviews; self-report measures of depressive, anxiety, and disruptive behavior symptoms; and brain magnetic resonance imaging. Previous cognitive functioning and parental stress assessments at age 8 as well as parental post-traumatic stress at age 16 years were explored as potential risk factors predictive of overall psychiatric functioning. RESULTS: Compared with healthy adolescents, adolescents with d-TGA had higher lifetime prevalence of structured interview-derived attention-deficit/hyperactivity disorder (19% vs 7%, P = .03), along with reduced global psychosocial functioning (80.6 ± 11.2 vs 87.2 ± 7.1, P < .001) as well as significant increases in self-reported depressive (P = .01), anxiety (P = .02), and disruptive behavior symptoms (parent P < .001 and adolescent P = .03). Nevertheless, these youth scored in the nonclinical range on all self-report measures. Level of global psychosocial functioning was positively related to cognitive functioning (P < .001) and negatively related to parental stress (P = .008). CONCLUSIONS: Although adolescents with d-TGA demonstrate significant resilience to known neuropsychological and academic deficits, they show increased rates of attention-deficit/hyperactivity disorder and reduced psychosocial functioning. Impaired cognitive functioning and parental stress at younger age emerged as significant risk factors for psychiatric impairment.

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.

Autonomic nervous system function, child behavior, and maternal sensitivity in three-year-old children with surgically corrected transposition.

OBJECTIVES: Explore relationships among autonomic nervous system (ANS) function, child behavior, and maternal sensitivity in three-year-old children with surgically corrected transposition of the great arteries (TGA) and in children healthy at birth. BACKGROUND: Children surviving complex congenital heart defects are at risk for behavior problems. ANS function is associated with behavior and with maternal sensitivity. METHOD: Child ANS function (heart rate variability) and maternal sensitivity (Parent-Child Early Relational Assessment) were measured during a challenging task. Mother completed the Child Behavior Checklist. Data were analyzed descriptively and graphically. RESULTS: Children with TGA had less responsive autonomic function and more behavior problems than healthy children. Autonomic function improved with more maternal sensitivity. CONCLUSION: Alterations in ANS function may continue years after surgical correction in children with TGA, potentially impacting behavioral regulation. Maternal sensitivity may be associated with ANS function in this population. Continued research on relationships among ANS function, child behavior, and maternal sensitivity is warranted.

Long-term outcome and quality of life after arterial switch operation: a prospective study with a historical comparison.

AIM.: The study aims to describe the long-term cardiological and psychological results of our first surgical cohort of arterial switch operation (ASO) patients and compare the results with our earlier series of Mustard patients. METHODS.: Twenty-four survivors of ASO operated in our center (1985-1990) were evaluated by electrocardiography, echocardiography, magnetic resonance imaging, exercise testing, 24-hour Holter-monitoring, and health-related quality of life questionnaire. The results were compared with 58 adult Mustard patients who were evaluated in 2001 using the same study protocol. RESULTS.: Arterial switch operation was performed at a median age of 13 days and Mustard operation at 2 years. Median follow-up was 22 years (range 20-25) and 25 years (22-29), respectively. After ASO, survival was better (P =.04). The event-free survival after 22 years was 77% after ASO vs. 44% after Mustard (P =.03). Good systemic ventricular function was present in 93% after ASO vs. 6% after Mustard (P <.01). Exercise capacity in ASO was 85% of predicted, compared with 72% in Mustard patients (P =.01). Aortic regurgitation was found in 21% of ASO patients vs. 16% in Mustard patients. Arterial switch patients vs. Mustard patients reported significantly better quality of life and less somatic complaints. CONCLUSION.: The progression made in surgical treatment for transposition of the great arteries from Mustard to ASO has had a positive impact on survival, cardiac function, exercise capacity, and also self-reported quality of life and somatic complaints. Longer follow-up is warranted to monitor aortic regurgitation.

Persistent superior exercise performance and quality of life long-term after arterial switch operation compared to that after atrial redirection.

BACKGROUND: In the end of the last century arterial switch operation (ASO) replaced atrial redirection according to Senning or Mustard as the surgical technique for infants with transposition of the great arteries (TGA). Better survival with the new technique has been shown. However, exercise performance and quality of life have not yet been compared directly. METHODS AND RESULTS: All consecutive patients aged sixteen years or older that had undergone ASO for TGA at our institution and had performed a cardiopulmonary exercise test as part of their routine follow-up were included into the study. Those twenty-eight patients (20 male, median age 18.8 years) were matched for age and gender with twenty-eight patients (20 male, median age 18.9 years) who underwent atrial redirection in the same surgical period at our institution. Peak oxygen uptake was higher in patients after ASO (median 38.1 [quartiles 30.3; 44.5] ml/kg/min vs. 29.8 [23.5; 33.9] ml/kg/min; p<.0001) representing 92.0% predicted and 66.1% predicted (p<.0001), respectively. In addition, patients after ASO presented a better ventilatory efficiency ( [Formula: see text] slope, p=.029), ventilatory threshold (p=.006), peak O2 pulse (p=.0001), and oxygen saturation (p=.016), as well as a superior blood pressure (p=.046) response to exercise. Self-estimated quality of life was rather good in both groups but with better results in the physical functioning and general health domains in patients after ASO. CONCLUSIONS: Adolescents and young adults with TGA still have an improved exercise performance and quality of life in the long-term follow-up after ASO than those patients after atrial redirection.

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.