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.

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.

Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging.

BACKGROUND: Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot. METHODS: In this single-centre cross-sectional study, we enrolled 91 adolescents (13-16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging. RESULTS: Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%). CONCLUSIONS: Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.

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.

Functional health status of adults with tetralogy of Fallot: matched comparison with healthy siblings.

BACKGROUND: Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. METHODS: All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. RESULTS: Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status (p < 0.001), were less likely to rate their health as good or excellent (p < 0.001), and had lower SF-36 scores for physical functioning and general health (p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. CONCLUSIONS: Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF.

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.

Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot.

OBJECTIVE: Although it is known that pediatric cardiac surgery holds risks for later development, few studies investigated the long-term development in children with tetralogy of Fallot. The purpose of this study was to define their intellectual capacities, neuropsychological profile, and behavioral functioning 6 to 12 years postoperatively. METHODS: Patients (n = 18; age, 8 years, 3 months +/- 1 year, 6 months) were examined with a short-form intelligence scale (Wechsler Intelligence Scale for Children, 3rd edition, Dutch version) and a neuropsychological assessment battery (NEPSY). Their parents completed a behavioral questionnaire. The patient group was compared with an acyanotic congenital heart disease group and a healthy control group. RESULTS: No significant differences between the patient group and the acyanotic group emerged. Compared with the healthy control group, children with tetralogy of Fallot showed significantly lower scores on the estimated Full Scale IQ (P < .05) and on the NEPSY domains Language (P < .01) and Sensorimotor Functioning (P < .01). Also, the subtests Tower (P < .05), Memory for Names (P < .05), Narrative Memory (P < .05), and Design Copy (P < .05) elicited group differences. Parental reports revealed significantly higher scores on attention problems (P < .05) and the total problem scale (P < .05), as well as significantly lower school performances than those of healthy peers (P < .01). CONCLUSIONS: In children with tetralogy of Fallot, we identified a lower estimated full-scale intelligence than in healthy peers and a neuropsychological profile characterized by primarily mild motor deficits and difficulties with language tasks. Parents of the children with tetralogy of Fallot indicated attention problems and rated the child's school competencies to be lower than in healthy control subjects.

Quality of life after repair of tetralogy of Fallot.

OBJECTIVE: To determine the quality of life in individuals with corrected tetralogy of Fallot. METHODS AND SUBJECTS: Questionnaires concerning quality of life were sent to all 87 surviving patients aged between 16 and 40 years who had undergone intracardiac repair of tetralogy of Fallot and follow-up in the Wessex Cardiothoracic Unit, and to 87 age and sex matched controls, with medically treated haemodynamically insignificant ventricular septal defects. RESULTS: The only significant difference found between the cases and controls was in requirements for schooling, where those with tetralogy of Fallot were more likely to require additional educational help at school (p = 0.044). For all other aspects of quality of life examined by the questionnaire, including social and genetic history, exercise ability, and health related quality of life, no significant differences were found. Different operative techniques, such as transjunctional patching, right ventriculotomy, and previous palliative shunting, did not affect the quality of life of our population with Tetralogy of Fallot, on average twenty years after their surgery, although the range of operative techniques was limited. Neither age at surgery, nor time since surgery, was correlated with measurements of quality of life. CONCLUSIONS: Those who have undergone surgical correction of tetralogy of Fallot have a normal quality of life, with few differences compared to controls.

Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot.

OBJECTIVE: To describe the long-term prognosis after repair of Tetralogy of Fallot with pulmonary stenosis beyond 20 years. METHODS: One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot at Rigshospitalet in Copenhagen between January 1960 and July 1977. Ninety seven patients had undergone a palliative operation prior to Tetralogy of Fallot repair. All the 125 patients who were discharged from the hospital were traced through the population register and the patients alive July 1997 were contacted by mail and/or telephone and questioned about use of medicine, professional status, family status and ability to perform sport activities. RESULTS: Sixty patients died in hospital and 125 patients, 78 males and 47 females, were discharged alive. Among operative survivors, median age at operation was 12.8 years (range 0.4-41 years). Thirteen patients required a reoperation, the main indication was failed VSD closure. There were 16 late cardiac deaths, out of which seven were sudden and unexpected and three were in immediate relation to reoperations. One hundred and nine patients were alive at follow-up. The mean follow-up time was 25.5 years (range 20-38 years). Sixteen percent used cardiac drugs, 89% were, or had been, working normally (all professions from academics to hard manual labors were represented), 53% (64% of women) had given birth after the repair and 51% performed sport activities regularly. No patients were lost to follow-up. CONCLUSIONS: The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.

[Psychological studies of families with children and adolescents following surgery for congenital heart defects (tetralogy of Fallot and aortic coarctation)]. / Psychologische Untersuchungen bei Familien mit Kindern und Jugendlichen nach Operation angeborener Herzfehler (Fallotscher Tetralogie und Aortenisthmusstenose).

Sixty-two children with operated congenital heart disease (34 with tetralogy of Fallot and 28 with coarctation) ranging in age from 9 to 16 years underwent a detailed psychological examination. The results were compared with children without structural heart disease and with test norm values. The aim of the study was to check findings on intelligence and explore aspects which had been hardly considered previously (for instance learning capability, concentration, mental work-up of disease etc.). We had assumed that there were differences between the Fallot group and that of coarctation, since the former had been handicapped physically prior to surgery. There were differences in terms of logical thinking, where children with coarctation turned out to be better. As far as other variables were concerned there was no significant difference (such as behavioural features), nor was there any difference between boys and girls. The results show that psychic "problems" of cardiac children do not resolve automatically after surgery, but there may be children with persisting psychological peculiarities who require guidance, occasionally even psychotherapy.

[Effect of timing of surgery on the intellectual and psychosocial development of children with tetralogy of Fallot]. / Der Einfluss des Operationszeitpunktes auf die intellektuelle und psychosoziale Entwicklung bei Kindern mit Fallotscher Tetralogie.

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.

[Psychosocial effects of heart disease in adolescents and adults with tetralogy of Fallot]. / Psychosoziale Auswirkungen des Herzfehlers im Adoleszenten- und Erwachsenenalter bei Tetralogie von Fallot.

The progress of heart surgery has brought about an important improvement in the prognosis of many congenital heart diseases. More and more attention is now being paid to the psychosocial effects of heart disease in patients who have undergone cardiac surgery. The results are presented of a study designed to assess the psychosocial situation of patients aged 18 years or more with tetralogy of Fallot. Variables assessed in the study include school, work, insurance, marriage, children, sport and physical activity, and emotional wellbeing.