Parenting stress mediates the association between cyanotic congenital heart disease and internalising problems in children and adolescents.

BACKGROUND: Children and adolescents with cyanotic congenital heart disease have been found to be at an increased risk of internalising problems. The underlying mechanisms, however, remain uncertain. AIMS: To examine the association between cyanotic congenital heart disease and internalising problems and assess whether parenting stress mediates this association. METHOD: The study sample was 699 children and adolescents (aged 2-17 years) with congenital heart disease (253 with cyanotic congenital heart disease and 446 with acyanotic congenital heart disease) in Taiwan. The Child Behavior Checklist and the Parenting Stress Index were used to assess internalising problems and parenting stress, respectively. A series of multiple regression models was conducted using the SPSS PROCESS procedure to test the association between types of congenital heart disease and internalising problems and the mediating role of parenting stress. A bootstrapping approach was applied to determine the significance of mediation. RESULTS: Compared with acyanotic congenital heart disease, cyanotic congenital heart disease was associated with increased levels of internalising problems (B=2.52, P<0.01), and this association was mediated by parenting stress (B=0.97, 95% bias-corrected bootstrap confidence interval 0.24, 1.75). In particular, parents of children with cyanotic congenital heart disease reported significantly more parenting stress compared with parents of children with acyanotic congenital heart disease (B=4.63, P<0.01), which contributed to elevated levels of internalising problems in the offspring (B=0.21, P<0.001). CONCLUSION: Cyanotic congenital heart disease conferred risks for internalising problems in children and adolescents, and this association was mediated by parenting stress. Interventions to decrease internalising problems in children and adolescents with cyanotic congenital heart disease may be more effective by targeting parenting stress.

Responses of mothers of children with CHD: quality of life, anxiety and depression, parental attitudes, family functionality.

Introduction The aim of this study was to evaluate the anxiety and depression status, family functions, parenting attitudes, and quality of life in the mothers of children with CHD. METHOD: The study enrolled 120 mothers: 40 of children with cyanotic CHD, 40 of children with non-cyanotic CHD, and 40 of healthy controls. Short Form-36 for quality of life, Hospital Anxiety-Depression Scale for anxiety and depression, Family Assessment Device for the detection of problems affecting family functions, and Parental Attitude Research Instrument for measuring child-rearing attitudes were used in the study. RESULTS: Statistically significant decreases were found in the general health standards of mothers of non-cyanotic children (p=0.035) and in the emotional and physical role difficulty of mothers of cyanotic children (p=0.006, p=0.010). When anxiety and depression levels of the parents were examined, the anxiety level of the cyanotic group was found to be significantly higher than that of the other groups (p=0.031). When family behaviours were assessed, there was a statistically significant decrease in role status in the families having a child with cyanotic CHD (p=0.035). In the Parental Attitude Research Instrument test, the husband and wife incompatibility sub-scale was found to be statistically significantly lower in the cyanotic CHD group (p=0.030). CONCLUSION: When there is a diseased person in the family, the focus should not be solely on the problems of the patient but also on preventive methods to be implemented in order to protect the mental health of all family members.

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.

Evaluation of depression and anxiety in parents of children undergoing cardiac catheterization.

OBJECTIVE: This study aimed to determine pre-procedure depression and anxiety levels among a group of parents whose children had congenital heart disease and were undergoing angiography. METHODS: The study comprised parents of 73 congenital heart disease patients undergoing angiography. The Beck Depression Inventory (BDI) and the Beck Anxiety Inventory (BAI) were used to evaluate the depression and anxiety scores. RESULTS: Sixty-one patients (83.6%) had acyanotic congenital heart disease, and 25 patients (34.2%) were undergoing diagnostic angiography. BDI scores among the mothers determined that 8 (11%) had mild, 14 (19.2%) moderate, and 10 (13.7%) severe depression. Their BAI scores showed that 16 (21.9%) had mild, 8 (11%) moderate, and 13 (17.8%) severe anxiety. BDI scores for the fathers showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe depression. Their BAI scores showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe anxiety. A comparison of mothers of cyanotic patients and those of acyanotic patients in terms of depression and anxiety levels revealed a statistically significant difference (p=0.050 and 0.043, respectively). CONCLUSION: Angiography was associated with increased levels of depression and anxiety in parents of children with congenital heart diseases. In comparison to parents of patients with acyanotic congenital heart disease, mothers of patients with cyanotic congenital heart disease had significantly higher levels of depression and anxiety.

Brain volumes predict neurodevelopment in adolescents after surgery for congenital heart disease.

Patients with complex congenital heart disease are at risk for neurodevelopmental impairments. Evidence suggests that brain maturation can be delayed and pre- and postoperative brain injury may occur, and there is limited information on the long-term effect of congenital heart disease on brain development and function in adolescent patients. At a mean age of 13.8 years, 39 adolescent survivors of childhood cardiopulmonary bypass surgery with no structural brain lesions evident through conventional cerebral magnetic resonance imaging and 32 healthy control subjects underwent extensive neurodevelopmental assessment and cerebral magnetic resonance imaging. Cerebral scans were analysed quantitatively using surface-based and voxel-based morphometry. Compared with control subjects, patients had lower total brain (P = 0.003), white matter (P = 0.004) and cortical grey matter (P = 0.005) volumes, whereas cerebrospinal fluid volumes were not different. Regional brain volume reduction ranged from 5.3% (cortical grey matter) to 11% (corpus callosum). Adolescents with cyanotic heart disease showed more brain volume loss than those with acyanotic heart disease, particularly in the white matter, thalami, hippocampi and corpus callosum (all P-values < 0.05). Brain volume reduction correlated significantly with cognitive, motor and executive functions (grey matter: P < 0.05, white matter: P < 0.01). Our findings suggest that there are long-lasting cerebral changes in adolescent survivors of cardiopulmonary bypass surgery for congenital heart disease and that these changes are associated with functional outcome.

Impact of fetal development on neurocognitive performance of adolescents with cyanotic and acyanotic congenital heart disease.

BACKGROUND: Our aims were to evaluate the neurocognitive performance in adolescents with congenital heart disease (CHD) and to determine whether parameters of fetal development evaluated in neonates, such as head circumference, length, weight, and Apgar scores, are somehow related to their neurocognitive performance. METHODS: We evaluated 77 CHD patients (43 males) aged from 13 to 18 years old (mean = 15.04 ± 1.86), 46 cyanotic, 23 with tetralogy of Fallot (TF), 23 with transposition of the great arteries (TGA) and 31 acyanotic with ventricular septal defect (VSD) enrolled in this study. The control group included 16 healthy children (11 males) ages ranging from 13 to 18 years old (mean = 15.69 years ± 1.44 years). All assessment measures for CHD patients were once obtained in a tertiary hospital; the control group was evaluated in school. Neuropsychological assessment included Wechsler's Digit Test, direct and reverse (WDD and WDR) and Symbol Search, Rey's Complex Figure (RCF), BADS's Key Searching Test, Color-Word Stroop Test (CWS), Trail Making Test (TMT), and Logical Memory Task (LMT). We evaluated some fetal parameters, such as head circumference, weight and length assessed at birth, and neonatal parameters, such as Apgar scores at 1 and 5 minutes after birth. We also registered some surgical parameters, such as the age at first operation and the number of surgeries. RESULTS: CHD patients compared with control group showed lower scores on every test, except for logical memory task. Patients with VSD when compared with patients with TF and TGA showed better results in all neuropsychological tests, although the only significant differences were in RCF, copy (F = 4936; P = .010). Several correlations were apparent between fetal/neonatal parameters and neuropsychological abilities in each type of CHD. However, head circumference at birth stands as the main correlation with cognitive development later on in all kinds of CHD (WDD: rho = .339, P = .011; RCF, copy: rho = 0.297, P = .027; CWS, interference: rho = 0.283, P = .036; TMT-A: rho = -0.321, P = .017). We analyzed the predicting relevance of several variables to cognitive performance of adolescents with CHD and confirmed that "cyanosis" stands as the main predictor (â = -4.758; t = -2.622; P = .011). CONCLUSIONS: Adolescents with CHD have worse neuropsychological performance than the control group, mainly the cyanotic patients. Fetal circulation seems to have impact on cerebral and somatic growth, predicting cognitive impairment in adolescents with CHD.

Exercise performance and quality of life is more impaired in Eisenmenger syndrome than in complex cyanotic congenital heart disease with pulmonary stenosis.

OBJECTIVE: Patients with cyanotic congenital heart disease without corrective surgery or palliation survive into adulthood, if they have a balanced pulmonary blood flow facilitated by pulmonary stenosis (PS) or Eisenmenger syndrome (ES). Both groups show cyanosis, diminished exercise performance and impaired quality of life. This study aimed to compare the functional outcome of those two cohorts directly. PATIENTS AND METHODS: In total fifty-eight cyanotic patients with cardiac shunts (28 male, 30 female, aged 14-55 years) were investigated, twenty-three of them with PS and thirty-five of them with ES. They completed the health related quality of life questionnaire SF-36 and performed a symptom limited cardiopulmonary exercise test. RESULTS: At exercise, oxygen saturation decreased severely and similarly in both groups (PS: 90% to 65% vs. ES: 87 % to 64%). Moreover, hemoglobin levels were comparable in both subgroups. Exercise capacity was markedly reduced, but more diminished in ES (PS: 20.3 (11.9;24.6) ml/min/kg vs. ES: 11.3 (9.7;14.5) ml/min/kg; p < 0.001) and ventilatory inefficiency expressed as V(E)/V(CO2) slope was more enhanced in ES (PS: 45.7 (37.6;52.9) vs. ES: 54.6 (43.4;68.7); p = 0.005). Oxygen saturation at rest was correlated to peak V(O2) (r = 0.436; p = 0.001) and V(E)/V(CO2) slope (r = -0.388; p = 0.003). Self estimated quality of life was poor, with worse results in physical and psychosocial domains in ES group. CONCLUSIONS: Despite similar cyanosis, patients with ES show less exercise performance, more ventilation-perfusion-mismatch and a worse quality of life compared to complex cyanotic congenital heart disease patients with PS. Moreover, oxygen saturation at rest predicts exercise capacity and ventilatory efficiency in this cohort.

Determinants of neuropsychological and behavioural outcomes in early childhood survivors of congenital heart disease.

AIMS: To evaluate the relative effect of cyanosis, surgical interventions and family processes on neuropsychological and behavioural outcomes in 4-year-old survivors of serious congenital heart disease (CHD). METHODS: 90 children with a range of cyanotic and acyanotic conditions, who underwent either corrective or palliative surgery, completed a neuropsychological and behavioural evaluation. Families of participants were also profiled by evaluation of maternal mental health, worry, social support, parenting style and family functioning. RESULTS: Compromised neuropsychological outcomes were associated with a combination of cyanotic conditions and open-heart surgery, but this was not exacerbated by having a complex, palliative, status. Both cyanotic and acyanotic conditions were associated with specific sensorimotor delays, regardless of method of the correction. Only children with complex conditions and palliative interventions seemed at risk of poor behavioural outcomes; indeed, children with cyanosis with complete repair showed favourable behavioural outcomes compared with controls. Multivariate analyses highlighted the sometimes greater relevance of family processes (eg parenting style, maternal mental health and worry), rather than disease or surgical factors, in predicting especially behavioural outcomes. CONCLUSIONS: The findings (1) suggest a more complex relationship between cyanosis, surgical methods of correction, neuropsychological and behavioural outcomes than previously charted, (2) highlight that family processes may be aetiologically more important than disease and surgical factors, and (3) indicate specific targets for secondary prevention programmes for this at-risk population.

Cyanotic episodes in a male child with fragile X syndrome.

A 9-year-old male with a diagnosis of fragile X syndrome (FXS) was evaluated for cyanotic episodes of unknown etiology. Clinical observation revealed frequent episodes of hyperventilation lasting several minutes, only while the patient was awake. This was followed by apnea associated with cyanosis and oxygen desaturation. Polysomnogram confirmed episodic central apnea temporally associated with hypocapnia, only during the awake state. Extensive evaluation failed to reveal other neurological, cardiac, gastrointestinal, or pulmonary etiologies for the events. The clinical observations and investigations allowed us to conclude that the patient's cyanotic episodes were caused by primary behavioral hyperventilation in the awake state. Similar behaviors have been reported in children with a variety of diagnoses but to our knowledge have not been previously reported in children with FXS. Treatment for this unusual behavior in FXS consists of reassurance and behavior modification to decrease the frequency and severity of the cyanotic episodes.

Breath-holding spells in somatoform disorder.

Breath-holding spells (BHS) are commonly seen in childhood. However, there are no case reports of BHS occurring in adolescents or young adults. We report two young adult cases and discuss the pathogensis, both physically and psychologically. BHS occurred for 1-2 minutes after hyperventilation accompanied by cyanosis in both cases. Oxygen saturation was markedly decreased. Each patient had shown distress and a regressed state psychologically. These cyanotic BHS occurred after hyperventilation, and we considered that a complex interplay of hyperventilation followed by expiratory apnea increased intrathoracic pressure and respiratory spasm. Breath-holding spells can occur beyond childhood.

Maternal infant bonding enhanced after atrial septostomy in cyanotic neonates in a general hospital.

UNLABELLED: Seventeen newborns in a general hospital had a successful atrial septostomy when indicated. Mostly done at the bedside under echocardiographical guidance, the successful introduction of this procedure enabled the infants to remain with mother to facilitate feeding and bonding prior to elective transfer to a children's hospital for corrective surgery. BACKGROUND: Atrial septostomy is a well-recognised intervention in the newborn to facilitate atrial mixing in transposition of the great arteries (TGA) or to decompress an atrium where the connecting AV valve is absent or stenosed, e.g. tricuspid atresia (TA). AIMS: To review the outcome of this procedure in a general hospital with appropriate neonatal and cardiological facilities. METHODS: Retrospective review over an 11-year period. RESULTS: Seventeen inborn infants had successful atrial septostomies, 11 with TGA and 6 with TA. All done under general anaesthesia, 15 were performed in the newborn nurseries, under echocardiographic guidance, and 2 in the catheter laboratory. No complications occurred. Eleven had a prenatal diagnosis made. All infants were able to be subsequently nursed by their mothers, affording prime time facilitating feeding and bonding. They were electively transferred to a children's hospital for corrective surgery. CONCLUSIONS: Atrial septostomy can be safely performed in a general hospital with appropriate neonatal and cardiological expertise. Such intervention allows for elective transfer of the infant for corrective surgery, allowing the infant and mother to be initially cared for at the one hospital, thereby facilitating maternal contact, feeding and bonding, doing away with the added stress of emergency transfer.

Are cyanosed adults with congenital cardiac malformations depressed?

OBJECTIVE: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. DESIGN: Prospective study of consecutive patients. SETTING: Single institution, tertiary referral centre. PATIENTS: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. ASSESSMENT: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. RESULTS: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p = 0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p = 0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. CONCLUSIONS: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability.

OBJECTIVE: To evaluate changes in cognitive and academic functioning following cardiac surgery in children with congenital heart disease. DESIGN: A prospective cross sectional study in which patients were assessed immediately before treatment and 12 months later. PATIENTS: Three groups of children aged 3.5-17 years: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group. MAIN OUTCOME MEASURES: Intelligence quotient and measures of academic attainment, evaluated with the British Ability Scales. RESULTS: Preoperatively, children with cyanotic lesions showed deficits in comparison with those with acyanotic lesions. Postoperatively, children with cyanotic lesions showed a deterioration in performance and achieved significantly lower scores than those with acyanotic lesions. While there were significant differences between the congenital heart disease and bone marrow transplantation groups preoperatively, these were no longer apparent at follow up. CONCLUSIONS: In contrast to previously published findings, the present results suggest that cardiac surgery does not result in early postoperative improvements in cognitive function for children with congenital heart disease. The nature of the cardiac lesion continues to affect cognitive and academic performance, even after surgery.

[Acrocyanosis: changing concepts and nosological limitations]. / Acrocyanose, évolution des concepts et limites nosologiques.

Acrocyanosis is undoubtedly the most commonplace acrosyndrome, both in terms of pathogenesis and prognosis. Patients experience functional impairment and an esthetic prejudice that must not be neglected. Adopting the nosological classifications described for Raynaud's syndrome, primary acrocyanosis must be distinguished from exceptional secondary phenomena that have a radically different clinical course. Primary acrocyanosis is generally observed in a young woman who appears thin or has recently lost weight. No paroxysmal episode (syncope, cyanosis, suspicious event involving the fingers) is found. The physical examination is negative and no complementary explorations are needed. Current pathophysiological hypotheses remain insufficient but suggest that vasospasticity rather than hemorheology is involved. The hypothesis that a thermoregulation disorder could be associated with weight loss deserves further study. Symptomatic care relies on dietary and hygiene counseling, emphasizing the importance of warm clothing. The psychological element must also be considered even in the most common forms.

Controlled study of preschool development after surgery for congenital heart disease.

AIM: Research into intellectual impairment among children with congenital heart disease has focused mainly on older children. This study was designed to determine whether previous findings are applicable to preschool children. METHODS: Three groups of children under 31/2 years old were assessed immediately before treatment and 12 months later: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group. RESULTS: Although the means of the three groups were within the normal range, preoperatively the cardiac and transplant groups showed deficits compared with the healthy controls. Postoperatively, continuing developmental deficits were significant only in the children with cyanotic lesions. CONCLUSIONS: Conclusions about intellectual development in older children with congenital heart disease do not apply to preschool children. Before corrective surgery, chronic illness itself appears to be the predominant influence on development. Postoperatively, cyanotic and acyanotic lesions are associated with different short term outcomes.

Efeitos pasicológicos no sintoma cianose em crianças do sexo feminino portadoras de cardiopatia congênita / Psychological effects of cyanosis in female children with congenital cardiopathies - eight female children observations

A cianose aparece como um dos principais sintomas em crianças portadoras de cardiopatia congênita do tipo cianogênica. O objetivo deste estudo foi analisar os efeitos da cianose no desenvolvimento psicológico infantil feminino. Foram atendidas 8 crianças do sexo feminino com média 8,1 anos, portadoras de cardiopatia congênita do tipo cianogênica. Foram utilizadas técnicas projetivas (desenho livre) e entrevistas abertas. De acordo com os resultados, 75 'por cento' utlizaram a cor roxa como principal em seus desenhos e 25 'por cento' também se utilizaram da mesma cor, mas com menor evidência. Em relaçäo às entrevistas, observou-se que 100 'por cento' demonstraram desagrado muito significativo por serem cianóticas, apresentando introversäo, auto-estima e autoconfiança rebaixadas, além da auto-imagem distorcida com sentimentos de insegurança e inferioridade, notando-se comportamento agressivo. Por meio deste estudo, concluiu-se que os efeitos da cianose interferem no desenvolvimento emocional da criança.

Care of the adult with cyanotic congenital heart disease.

In adults with cyanotic congenital heart disease (CHD), medical considerations apply to nonsurgical survivors and to postsurgical patients. The special needs of these groups present a unique challenge for the adult care practitioner. Clinical management revolves around the physiologic consequences of a right-to-left shunt. The care of the cyanotic CHD patient must encompass not only the physiologic variables that set these patients apart from the "typical" adult with acquired cardiovascular disease but also the lifelong psychosocial impact of their congenital heart program.

Behavior profile of children with severe breath-holding spells.

Thirty-two children with severe breath-holding spells and 42 control children were studied by using the Child Behavior Checklist. No single behavioral profile could identify children with breath-holding spells, and there were no group differences with respect to individual behavior categories, broad-band profiles, or total scores. No correlation was found between frequency of breath-holding spells and profile scores.