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OBJECTIVES: To assess the occurrence of a wide range of behavioural, emotional, and post-traumatic stress problems in children and adolescents, long term after septic shock caused by Neisseria meningitidis (MSS). DESIGN: This study included 6- to 17-year-old patients who survived MSS and were admitted to the PICU of the Medical Centre between 1988 and 2001. To assess behavioural, emotional, and post-traumatic stress problems, the Child Behaviour Checklist (CBCL), the Teacher's Report Form (TRF), and the Youth Self-Report (YSR) were used. METHODS: Parents of 89 MSS children, aged 6-17 years, completed the CBCL. Teachers of 65 same-aged MSS children completed the TRF, and 45 11- to 17-year-old MSS children completed the YSR. These data were compared with those from the normative reference groups. RESULTS: Overall, the proportions of MSS children scoring in the deviant range for problem behaviour were comparable to the proportions in the reference groups, according to parents', teachers', and self-reports. As to the level of emotional and behavioural problems, mothers of the MSS children reported more somatic complaints regarding their children in comparison with the reference groups. Severity of illness was not a significant predictor of behavioural, emotional, and post-traumatic stress problems. Age at the time of illness was a significant predictor of behavioural, emotional, and post-traumatic stress problems in MSS children, indicating that the younger the child at the time of illness, the more problems were reported by parents at follow-up. CONCLUSION: Overall, the results showed long-term behavioural, emotional, and post-traumatic stress outcomes for MSS children, which were comparable to those in the general population. [ABSTRACT FROM AUTHOR]

Objective: This study systematically reviewed recent findings on neurocognitive functioning and health-related quality of life (HRQoL) of children after pediatric intensive care unit admission (PICU)., Data Sources: Electronic databases searched included Embase, Medline Ovid, Web of Science, Cochrane CENTRAL, and Google Scholar. The search was limited to studies published in the last five years (2015-2019)., Study Selection: Original studies assessing neurocognitive functioning or HRQoL in children who were previously admitted to the PICU were included in this systematic review., Data Extraction: Of the 3649 identified studies, 299 met the inclusion criteria based on title abstract screening. After full-text screening, 75 articles were included in the qualitative data reviewing: 38 on neurocognitive functioning, 33 on HRQoL, and 4 on both outcomes., Data Synthesis: Studies examining neurocognitive functioning found overall worse scores for general intellectual functioning, attention, processing speed, memory, and executive functioning. Studies investigating HRQoL found overall worse scores for both physical and psychosocial HRQoL. On the short term (≤ 12 months), most studies reported HRQoL impairments, whereas in some long-term studies HRQoL normalized. The effectiveness of the few intervention studies during and after PICU admission on long-term outcomes varied., Conclusions: PICU survivors have lower scores for neurocognitive functioning and HRQoL than children from the general population. A structured follow-up program after a PICU admission is needed to identify those children and parents who are at risk. However, more research is needed into testing interventions in randomized controlled trials aiming on preventing or improving impairments in critically ill children during and after PICU admission., (© 2022. The Author(s).)

Background: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated., Objective: To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents., Methods: A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires., Results: Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients., Conclusion: Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage.

Background: Dilated cardiomyopathy (DCM) in children is an important cause of severe heart failure and carries a poor prognosis. Adults with heart failure are at increased risk of anxiety and depression and such symptoms predict adverse clinical outcomes such as mortality. In children with DCM, studies examining these associations are scarce., Aims: We studied whether in children with DCM: (1) the level of emotional and behavioral problems was increased as compared to normative data, and (2) depressive and anxiety problems were associated with the combined risk of death or cardiac transplantation., Methods: To assess emotional and behavioral problems in children with DCM, parents of 68 children, aged 1.5-18 years (6.9±5.7 years), completed the Child Behavior Checklist., Results: Compared to normative data, more young children (1.5-5 years) with DCM had somatic complaints (24.3% vs. 8.0%; p < .001), but fewer had externalizing problems (5.4% vs. 17.0%; p = .049). Overall internalizing problems did not reach significance. Compared to normative data, more older children (6-18 years) showed internalizing problems (38.7% vs. 17.0%; p = .001), including depressive (29.0% vs. 8.0%; p < .001) and anxiety problems (19.4% vs. 8.0%; p = .023), and somatic complaints (29.0% vs. 8.0%; p < .001). Anxiety and depressive problems, corrected for heart failure severity, did not predict the risk of death or cardiac transplantation., Conclusion: Children of 6 years and older showed more depressive and anxiety problems than the normative population. Moreover, in both age groups, somatic problems were common. No association with outcome could be demonstrated.

Background: Transcatheter aortic valve implantation (TAVI) has become the standard treatment for patients with severe symptomatic aortic stenosis (AS) considered at very high risk for surgical aortic valve replacement. The purpose of this sub-study was to evaluate long-term (> 4 years) health-related quality of life (QoL) in octogenarians who underwent TAVI., Methods: A single center observational registry in twenty patients who underwent frame analysis assessment ≥ 4 years after TAVI. Health-related QoL was evaluated, using the Short Form-36 (SF-36), the EuroQoL-5D (EQ-5D) and the visual analogue score (EQ-VAS) questionnaires., Results: The mean SF-36 subscale scores at follow-up were physical functioning 40.8 ± 26.3, role physical functioning 67.7 ± 34.9, vitality 54.6 ± 21.6, general health 52.1 ± 20.4, social functioning 63.8 ± 37.7, role emotional functioning 70.2 ± 36.0, mental health 73.2 ± 23.3 and bodily pain 80.9 ± 22.9. The mean EQ-VAS score > 4 years after TAVI was 64.7 ± 15.1. With respect to functional class, 80% of the patients were in NYHA class I/II at follow-up compared to 15% prior to TAVI., Conclusions: This sub-study reports a significant improvement in functional class (NYHA) in a selected group of very elderly patients > 4 years after TAVI. Furthermore, all patients showed a satisfactory QoL despite their age and multiple comorbidities. In addition, our study reveals a lower QoL when compared with the general age matched Dutch population.

Background: Preoperative anxiety in children is highly prevalent and is associated with adverse outcomes. Existing psychosocial interventions to reduce preoperative anxiety are often aimed at distraction and are of limited efficacy. Gradual exposure is a far more effective way to reduce anxiety. Virtual reality (VR) provides a unique opportunity to gradually expose children to all aspects of the operating theater., Objective: The aims of our study are (1) to develop a virtual reality exposure (VRE) tool to prepare children psychologically for surgery; and (2) to examine the efficacy of the VRE tool in a randomized controlled trial (RCT), in which VRE will be compared to care as usual (CAU)., Methods: The VRE tool is highly realistic and resembles the operating room environment accurately. With this tool, children will not only be able to explore the operating room environment, but also get accustomed to general anesthesia procedures. The PREoperative Virtual reality Intervention to Enhance Wellbeing (PREVIEW) study will be conducted. In this single-blinded RCT, 200 consecutive patients (aged 4 to 12 years) undergoing elective day care surgery for dental, oral, or ear-nose-throat problems, will be randomly allocated to the preoperative VRE intervention or CAU. The primary outcome is change in child state anxiety level between baseline and induction of anesthesia. Secondary outcome measures include child's postoperative anxiety, emergence delirium, postoperative pain, use of analgesics, health care use, and pre- and postoperative parental anxiety., Results: The VRE tool has been developed. Participant recruitment began March 2017 and is expected to be completed by September 2018., Conclusions: To our knowledge, this is the first RCT evaluating the effect of a VRE tool to prepare children for surgery. The VRE intervention is expected to significantly diminish preoperative anxiety, postoperative pain, and the use of postoperative analgesics in pediatric patients. The tool could create a less stressful experience for both children and their parents, in line with the modern emphasis on patient- and family-centered care., Trial Registration: Netherlands Trial Registry: NTR6116; http://www.trialregister.nl/trialreg/admin/rctview.asp?TC=6116 (Archived by WebCite at http://www.webcitation.org/6ryke7aep)., (©Robin Eijlers, Jeroen S Legerstee, Bram Dierckx, Lonneke M Staals, Johan Berghmans, Marc P van der Schroeff, Rene MH Wijnen, Elisabeth MWJ Utens. Originally published in JMIR Research Protocols (http://www.researchprotocols.org), 11.09.2017.)

Background: Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals. We included 90 children (median age 3.8 years, interquartile range (IQR) 0.9-12.3) whose parents completed 515 questionnaires. At the same visit, physicians completed the New York University Pediatric Heart Failure Index. Compared with Dutch normative data, quality of life was severely impaired at diagnosis (0-4 years: 7/10 subscales and 4-18 years: 8/11 subscales) and ⩾1 year after diagnosis (3/10 and 6/11 subscales). Older children were more impaired (p<0.05). After a median follow-up of 3 years (IQR 2-4), 15 patients underwent transplantation. Using multivariable time-dependent Cox regression, "physical functioning" subscale and the Heart Failure Index were independently predictive of the risk of death and heart transplantation (hazard ratio 1.24 per 10% decrease of predicted, 95% confidence interval (CI) 1.06-1.47 and hazard ratio 1.38 per unit, 95% CI 1.19-1.61, respectively)., Conclusion: Physical impairment rated by parents and heart failure severity assessed by physicians independently predicted the risk of death or heart transplantation in children with dilated cardiomyopathy.

Objective: The objective was to estimate the age of onset (AOO) for all anxiety disorders and for specific subtypes. Gender differences in the AOO of anxiety disorders were examined, as were the influence of study characteristics on reported AOOs., Methods: Seven electronic databases were searched up to October 2014, with keywords representing anxiety disorder subtypes, AOO, and study design. The inclusion criteria were studies using a general population sample that provided data on the AOO for all anxiety disorders, or specific anxiety disorders, according to DSM-III-R, DSM-IV, or ICD-10 criteria., Results: There were 1028 titles examined, which yielded 24 studies meeting the inclusion criteria. Eight studies reported the AOO and gender. Meta-analysis found a mean AOO of all anxiety disorders of 21.3 years (95% CI 17.46 to 25.07). Separation anxiety disorder, specific phobia, and social phobia had their mean onset before the age of 15 years, whereas the AOO of agoraphobia, obsessive-compulsive disorder, posttraumatic stress disorder, panic disorder, and generalized anxiety disorder began, on average, between 21.1 and 34.9 years. Meta-analysis revealed no difference in the AOO between genders. A prospective study design and higher developmental level of the study country were associated with an earlier AOO., Conclusions: Results from this meta-analysis indicate that anxiety disorder subtypes differ in the mean AOO, with onsets ranging from early adolescence to young adulthood. These findings suggest that prevention strategies of anxiety disorders should be directed towards factors associated with the development of anxiety disorder subtypes in the age groups with the greatest vulnerability for developing those disorders.

Introduction: Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to investigate the wish to conceive and the concerns about cardiovascular complications during pregnancy in women with TS., Methods: The patient association for TS invited all members of ≥18 years old (n = 344) to complete a specifically developed, disease-specific questionnaire, including questions about fertility, wish to conceive, attempts and concerns. Results were compared with previously published results of this questionnaire in women with congenital heart disease., Results: The questionnaire was completed by 89 women (median age 30.1 years, Q1-Q3 = 22.9-39.4). Of them, 51% had 45, X0-monosomy and 38% had ≥1 cardiac abnormality. Seventeen women (19%) had attempted to become pregnant and 12 of them succeeded to become pregnant. Women who had not undertaken attempts to conceive (81%), considered themselves mainly too young or had no partner. Of the total sample, 58% were concerned about the influence of pregnancy on their cardiovascular status. This was higher (75%) in the sample of women with TS and cardiac abnormalities, than in women with congenital heart disease from a previously published cohort (21%), (p < .001). There were no differences in concerns about pregnancy complications between women with TS who respectively had or had not attempted to become pregnant., Discussion: Women with TS, especially those with cardiac abnormalities, show serious concerns about the risks pregnancy may have. Patients should be timely counseled and specifically asked about their concerns. Psychosocial care should be provided when necessary.

Background: Cardiac rehabilitation (CR) is recommended as secondary prevention in primary percutaneous coronary intervention (pPCI) patients. This study was conducted to expand the knowledge about age-effects of CR in pPCI patients. The aim of this study was to compare changes in subjective health status (SHS) during and after CR between patients <60years and patients ≥60years, who underwent pPCI after myocardial infarction., Methods: Between 2009 and 2011, in total 282 pPCI patients who participated in CR were included. Patients completed the Short Form 12 (SF-12) questionnaire at baseline (pre-CR), 3months (post-CR) and 12months follow-up. Patients were divided into two age-groups, <60years versus ≥60years. To compare improvements in SHS between groups, Generalized Estimating Equations (GEE) analyses were performed., Results: The mean physical component summary (PCS) score improved over time in both groups and even reached mean levels of the normative Dutch population. The improvement on the PCS score was equal in both age groups. The mental component summary (MCS) score also improved in both groups. Patients <60years reported on average more improvement on the MCS score than patients ≥60years (Exp(B) 1.019; 95%CI 1.009-1.030; P<0.001). However, mean levels of the normative Dutch population were not reached by patients <60years., Conclusion: Even though pPCI patients <60years reported more improvement on the MCS score, mean levels of the normative Dutch population were not reached. Therefore, a tailored CR program with more focus on their mental status, may be beneficial in younger patients., (Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.)

Objective: The aim of this study was to investigate the psychosocial needs of both parents of children with CHD (aged 0-18 years) and patients themselves (aged 8-18 years) in the week before cardiac surgery or a catheter intervention. Patients Eligible participants included all consecutive patients (0-18 years) scheduled to undergo cardiac surgery or a catheter intervention in our hospital between March, 2012 and July, 2013. Psychosocial needs were assessed using a disease-specific questionnaire designed for this study, consisting of a 83-item parent version and a 59-item child version (for children ⩾8 years), each covering five domains: physical/medical, emotional, social, educational/occupational, and health behaviour; two items assessed from whom and in what format psychosocial care was preferred. Quality of life was also assessed. Interventions If parents/patients reported a need for psychosocial care, referral to adequate mental health-care professionals was arranged., Results: More than 40% of participating parents and >50% of participating children reported a need for psychosocial care on each of the five domains. Needs for psychosocial care for parents themselves were highest for those with children aged 0-12 years. Parents and patients report clear preferences when asked from whom and in what format they would like to receive psychosocial care. Quality of life was relatively high for both parents and patients. Psychosocial care interventions in our hospital increased significantly after the implementation of this study., Conclusions: Results show that psychosocial care is rated as (very) important by both parents and children during an extremely stressful period of their life.

Background: Poor subjective health status significantly predicted short-term mortality in patients with coronary artery disease (CAD). However, the relation between subjective health status and long-term mortality remains limited in patients treated with PCI. The aim of this study is to investigate the association between subjective health status and 14-year mortality in patients treated with percutaneous coronary intervention (PCI)., Methods: A consecutive cohort with 1111 patients treated for CAD who completed the SF-36 questionnaire was included between 2001 and 2002 as part of the RESEARCH registry., Results: After adjustment, physical functioning (HR: 1.96; 95% CI: 1.59-2.43), social functioning (HR: 1.53; 95% CI: 1.24-1.88), role limitations due to physical functioning (HR: 1.75; 95% CI: 1.41-2.16), role limitations due to emotional functioning (HR: 1.34; 95%CI: 1.08-1.67), mental health (HR: 1.52; 95% CI: 1.24-1.88), vitality (HR: 1.66; 95% CI: 1.35-2.03), bodily pain (HR: 1.63; 95% CI: 1.32-2.02) and general health (HR: 1.82; 95% CI: 1.49-2.23) were all associated with an increased risk of 14-year mortality., Conclusion: Physical and mental subjective health status as measured with the SF-36 appeared to be a strong predictor for 14-year mortality in post-PCI patients., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS)., Methods: Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years., Results: Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (<30 days), survival was 94% at 40 years. Of 46 eligible survivors, 29 participated in the in-hospital examination and 15 gave permission to use their hospital records (96% participation). Cumulative event-free survival was 68% after 40 years: 25% needed a reintervention, 12% underwent pacemaker implantation and 9% had supraventricular arrhythmias. Early reinterventions were mainly for residual PS, late reinterventions for pulmonary regurgitation. Subjective health status was good. Exercise capacity was normal in 74% (median 96 (82-107)% of expected workload). Right ventricular and left ventricular (LV) dysfunction was found in 13% and 41%, respectively. The use of a transannular patch and younger age at surgery were predictive for late events (HR 3.02 (95% CI 1.09 to 8.37) and HR 0.81/year (95% CI 0.66 to 0.98), respectively). Use of inflow occlusion compared with cardiopulmonary bypass showed a trend towards more reinterventions (HR 3.19 (95% CI 0.97 to 10.47))., Conclusions: Survival up to 40 years after successful PS repair is nearly normal. Subjective health status is good and there is a low incidence of arrhythmias. Reinterventions, however, are necessary in one-quarter and 40 years postoperatively several patients show LV dysfunction., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Children and adolescents with a congenital heart defect (ConHD) frequently undergo painful or frightening medical procedures and hospitalizations. They often need multiple invasive procedures at a very young age and require regular checkups during their entire life. From other pediatric populations, it is known that these kinds of experiences can result in acute stress reactions and even in post-traumatic stress disorder (PTSD) in the long-term. PTSD and also subthreshold PTSD can lead to serious (psychosocial) impairment. However, limited information is available about PTSD in children with ConHD. Therefore, the aim of this review is to provide a summary of the current literature on post-traumatic stress (PTS) in children and adolescents with ConHD describing the prevalence of PTSD and its predictors/correlates. This review indicates that a range of 12-31% of children undergoing cardiac surgery develop PTSD. A range of 12-14% shows elevated post-traumatic stress symptoms (PTSS). These findings are comparable to those of hospitalized children without ConHD. Noteworthy, most studies used varying self-report questionnaires to measure PTSD and only one study used a semistructured interview. Although all studies point in the same direction of elevated PTSD and PTSS, systematic research is necessary to be able to draw firm conclusions. At present, as far as we know, in most clinics treating patients with ConHD, there is no regular screening for PTS in children with ConHD. In the reviewed literature, there is strong consensus that screening for PTSS and (preventive) psychological care for children and adolescents with ConHD is urgently needed.

Objective: There are indications that type D personality and depression are associated in patients treated with percutaneous coronary intervention (PCI). However, at present it is unclear whether this relationship holds in the long term. This study's aim was to investigate the association between type D personality at 6 months post-PCI (baseline), and depression at 10-year follow-up. A secondary aim was to test the association between type D personality at baseline and anxiety at 10-year follow-up., Methods: A cohort of surviving consecutive patients (N = 534) who underwent PCI between October 2001 and October 2002. Patients completed the type D personality scale (DS14) measuring type D personality at baseline, and the Hospital Anxiety and Depression Scale (HADS) measuring anxiety and depression at baseline and at 10 years post-PCI., Results: At baseline, the prevalence of type D personality was 25 % (135/534). Type D personality patients were more often depressed (42 %) than non-type D personality patients (9 %). Response rate of anxiety and depression questionnaires at 10 years was 75 %. At 10-year follow-up, 31 % of type D personality patients were depressed versus 13 % of non-type D personality patients. After adjustments, baseline type D personality remained independently associated with depression at 10 years (OR = 3.69; 95 % CI [1.89-7.19]). Type D showed a similar association with anxiety at 10 years, albeit somewhat lower (OR = 2.72; 95 % CI [1.31-5.63])., Conclusions: PCI patients with type D personality had a 3.69-fold increased risk for depression and a 2.72-fold increased risk for anxiety at 10 years of follow-up., Competing Interests: Funding sources None Conflict of interests We wish to confirm that there are no known conflicts of interest associated with this publication

Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Sickle cell disease (SCD) is endemic in non-Western countries. Due to migration, the prevalence of SCD in the Netherlands has increased. Adherence to medical treatment is recognized as a major problem area. Therefore, new effective interventions to increase adherence are urgently needed., Methods/design: The TEAM study is an ongoing randomized controlled trial (RCT) to compare protocolized individual medical appointments (IMA's; care-as-usual) with protocolized group medical appointments (GMA's; novel intervention) in pediatric (n = 40) and adult (n = 60) patients. The study aims to assess the effectiveness of GMA's (over a three year period) on patients' self-efficacy, adherence, quality of life, morbidity, hospital admissions and satisfaction with the treating professional; as well as to test the cost-effectiveness of GMA's. In both the IMA and GMA groups structured assessments will be performed at baseline (start of the study), after 1.5 and after 3 years., Discussion: This is the first RCT to investigate the effectiveness of GMA's on self-efficacy and adherence in pediatric and adult patients with SCD, including a cost-effectiveness analysis., Trial Registration: NTR4750 (NL42182.000.12). Registered 13 August 2014.

Background: Low marital quality is associated with adverse health outcomes and lower personal well-being. Loneliness increases the risk of cardiovascular disease and mortality and predicts poor quality of life. The aim of this study was to investigate the association between marital quality and loneliness and subjective health status in primary percutaneous coronary intervention (pPCI) patients who underwent cardiac rehabilitation (CR)., Design/methods: In a prospective cohort study, pPCI patients that followed CR were included between 2009-2011. A total of 223 patients responded to the Short Form 12 (SF-12) (subjective health status), Maudsley Marital Questionnaire (MMQ-6) (marital quality) and University of California, Los Angeles - Revised (UCLA-R) questionnaires at baseline (pre-CR) and at three months (post-CR) or at 12 months follow-up. Subjective health status is displayed by a physical component summary (PCS) score and a mental component summary (MCS) score. Generalized estimating equation (GEE) analyses were performed to test improvements in subjective health status., Results: Changes over time in subjective health status scores were similar between patients with optimal marital quality vs patients with less optimal marital quality and non-lonely patients vs lonely patients. The MCS level at one-year follow-up of both patients with less optimal marital quality and lonely patients was lower compared with a healthy Dutch population (respectively; mean MCS score 47.3 (standard deviation (SD) 10.5); p = 0.013 and mean MCS score 46.1 (SD 11.2); p = 0.010)., Conclusion: Both patients with less optimal marital quality and lonely patients did not reach the MCS level of a healthy Dutch population. Therefore, extra care and support should be given to these patients in a CR programme., (© The European Society of Cardiology 2016.)

Unlabelled: Very little is known about the psychological consequences of a cardiac arrest (CA) during childhood. Our aim was to assess long-term emotional and behavioral functioning, and its predictors, in survivors of CA in childhood. This long-term follow-up study involved all consecutive infants, children, and adolescents surviving CA in a tertiary-care university children's hospital between January 2002 and December 2011. Emotional and behavioral functioning was assessed with the Child Behavior Checklist (CBCL), Teacher's Report Form (TRF), and Youth Self-Report (YSR). Of the eligible 107 CA survivors, 52 patients, parents, and/or teachers filled out online questionnaires. Compared with normative data, parents and teachers reported significantly more attention and somatic problems (age range 6-18 years). Parents also reported more attention problems for age range 1.5-5 years. Twenty-eight percent of the children (n = 14) scored in the psychopathological range (i.e., for age range 1.5-18 years; p < 0.001) according to parent reports. Male gender, older age, and basic life support were significantly related to worse scores on the scales internalizing problems, externalizing problems, and total problems and subscale attention problems., Conclusion: Long-term deficits in attention and somatic complaints were reported. Attention problems after childhood CA can interfere with school performance. Long-term follow-up with neuropsychological assessment should be organized., What Is Known: • Critical illness has a significant influence on the presence of long-term emotional and behavioral problems. • Long-term emotional and behavioral problems have been described for various groups of critically ill children such as congenital heart disease, meningococcal septic shock, and neonatal asphyxia. What is new: • This is the first study that addresses long-term emotional and behavioral problems in a relatively large consecutive series of children and adolescents surviving cardiac arrest. • Long-term deficits in attention and somatic complaints were reported.

Background: We examined the longitudinal associations of autonomic nervous system (ANS) and hypothalamic-pituitary-adrenal (HPA) axis rest and reactivity measures with anxiety and depressive symptoms at one-year follow-up in children with anxiety disorders., Methods: In a clinical sample of 152 children with a primary DSM-IV anxiety disorder, aged 8 to 12 years, anxiety and depressive symptoms were assessed with the Multidimensional Anxiety Scale for Children and the Children's Depression Inventory at pre-treatment baseline and one year later, after treatment with cognitive behavioral therapy. At baseline, children participated in a 70min stress task. Salivary cortisol was measured directly prior to and 20min post stress task. Skin conductance level (SCL), heart rate and high frequency heart rate variability (HRV) were continuously measured during rest and the stress task. To investigate if rest or reactivity measures predicted anxiety and depressive symptoms at one year follow-up, linear regression analyses were conducted for rest and reactivity measures of SCL, heart rate, HRV and cortisol separately., Results: Higher SCL reactivity predicted less decrease of anxiety symptoms at one-year follow-up. Cortisol reactivity showed a weak association with depressive symptoms at one-year follow-up: lower cortisol reactivity predicted less decrease in depressive symptoms., Limitations: Only self-reported anxiety and depressive symptoms were used. However, all predictors were objective biological measures, hence there is no risk of shared method variance bias., Conclusions: These findings suggest that pre-treatment HPA and ANS responsiveness to stress are predictive biomarkers for a lack of symptom improvement in children with a clinical anxiety disorder., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Introduction: Adolescents with inflammatory bowel disease (IBD) show a higher prevalence of depression and anxiety, compared to youth with other chronic diseases. The inflammation-depression hypothesis might explain this association, and implies that treating depression can decrease intestinal inflammation and improve disease course. The present multicentre randomised controlled trial aims to test the effectiveness of an IBD-specific cognitive-behavioural therapy (CBT) protocol in reducing symptoms of subclinical depression and anxiety, while improving quality of life and disease course in adolescents with IBD., Methods and Analysis: Adolescents with IBD (10-20 years) from 7 hospitals undergo screening (online questionnaires) for symptoms of depression and anxiety. Those with elevated scores of depression (Child Depression Inventory (CDI) ≥13 or Beck Depression Inventory (BDI) II ≥14) and/or anxiety (Screen for Child Anxiety Related Disorders: boys ≥26, girls ≥30) receive a psychiatric interview. Patients meeting criteria for depressive/anxiety disorders are referred for psychotherapy outside the trial. Patients with elevated (subclinical) symptoms are randomly assigned to medical care-as-usual (CAU; n=50) or CAU plus IBD-specific CBT (n=50)., Main Outcomes: (1) reduction in depressive and/or anxiety symptoms after 3 months and (2) sustained remission for 12 months., Secondary Outcomes: quality of life, psychosocial functioning, treatment adherence. In addition, we will assess inflammatory cytokines in peripheral blood mononuclear cells and whole blood RNA expression profiles. For analysis, multilevel linear models and generalised estimating equations will be used., Ethics and Dissemination: The Medical Ethics Committee of the Erasmus MC approved this study. If we prove that this CBT improves emotional well-being as well as disease course, implementation is recommended., Trial Registration Number: NCT02265588.

Purpose: It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents., Methods: We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form., Results: In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains., Conclusions: Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.

Background: Depression has been shown to be an independent risk factor for short-term mortality in patients with coronary artery disease (CAD). There are studies suggesting that depression might also be associated with long-term mortality. Anxiety has also been associated with mortality. This study aimed to further investigate the predictive value of depression and anxiety symptoms on all-cause mortality, 10 years after percutaneous coronary intervention (PCI)., Methods: The study population comprised a consecutive series of CAD patients (n = 1411) treated with PCI between September 2001 and October 2002 at the Erasmus Medical Centre, Rotterdam. The Hospital Anxiety and Depression Scale (HADS) was completed by 1112 patients at baseline to assess levels of depression and anxiety. The endpoint was defined as all-cause mortality., Results: The prevalence of depression and anxiety was 24.8% and 27.7%, respectively. The cumulative all-cause mortality rate in depressed patients was 37% versus 20% in non-depressed patients (log-rank p < 0.001). After adjustment, depression remained a predictor of all-cause mortality (hazard ratio (HR) 1.77; 95% confidence interval (CI) 1.36-2.29). Cumulative survival rates did not differ for anxious versus non-anxious patients (log-rank p = .79). However, after adjustment, anxiety was associated with an increased risk for all-cause mortality (HR 1.50; 95% CI 1.14-1.98). A sub-analysis showed that cumulative survival rates did not differ for depressed and anxious patients versus depressed but non-anxious patients (log-rank p = 0.46)., Conclusions: Depression is associated with an increased risk of 77% for all-cause mortality, 10 years post-PCI, independently of anxiety. Although anxiety was associated with all-cause mortality, it has no additional value in the case of co-occurring depression., (© The European Society of Cardiology 2015.)

Objective: To determine longitudinal changes in psychopathology in a cohort of patients 30-43 years after their first cardiac surgery for Congenital Heart Disease (CHD) in childhood, to compare outcomes of the 30- to 43-year follow-up with normative data, and to identify medical predictors for psychopathology., Methods: This study is the third follow-up of this cohort. The first and second follow-ups of this same cohort were conducted in 1990 and 2001, respectively. At all three follow-ups, psychopathology was assessed with standardised, parallel questionnaires. In 2011, subjective health status was assessed by the Short Form-36. Medical predictor variables were derived from medical examinations and medical records., Results: In this third follow-up, a total of 252 patients participated. Of these, 152 patients participated in all three follow-ups. Over a 30-year period, proportions of patients showing psychopathology decreased significantly. At the 30- to 43-year follow-up, overall outcomes on psychopathology for the CHD sample were similar or even better compared with normative groups. Subjective health status was also better compared with normative data. No differences were found between cardiac diagnostic groups. Medical variables that predicted the course of psychopathology over time were as follows: the scar, as judged by the patient, results of the first cardiac surgery, and the number of hospitalisations., Conclusions: Over a 30-year period, psychopathology decreased in patients with CHD. Levels of psychopathology in these patients, who are now aged between 30 and 54 years, were comparable or even better than normative data.

Background: Computed Tomography (CT) is the gold standard to assess bronchiectasis and trapped air in cystic fibrosis (CF) lung disease, but has the disadvantage of radiation exposure. Magnetic Resonance Imaging (MRI) is a radiation free alternative., Objective: To validate MRI as outcome measure by: correlating MRI scores for bronchiectasis and trapped air with clinical parameters, and by comparing those MRI scores with CT scores., Methods: In patients with CF (aged 5.6-17.4 years), MRI and CT were alternated annually during routine annual check-ups between July 2007 and January 2010. Twenty-three children had an MRI performed 1 year prior to CT, 34 children had a CT 1 year prior to MRI. Bronchiectasis and trapped air were scored using the CF-MRI and CF-CT scoring system. CF-MRI scores were correlated with clinical parameters: FEV1 , Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms measured on the Cystic Fibrosis Questionnaire-Revised (CFQ-R), using Spearman's correlation coefficient. MRI and CT scores were compared using intra-class correlation coefficients (ICC) and Bland-Altman plots., Results: Fifty-seven patients who had an MRI, CT and CFQ-R during the study period were included. CF-MRI bronchiectasis correlated with FEV1 , Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms. CF-MRI trapped air only correlated with FEV1 and Pseudomonas aeruginosa. ICCs between MRI and CT bronchiectasis and trapped air were 0.41 and 0.35 respectively. MRI tended to overestimate bronchiectasis compared to CT., Conclusion: The associations between CF-MRI scores and several important clinical parameters further contributes to the validation of MRI. MRI provides different information than CT., (© 2015 Wiley Periodicals, Inc.)

Background: Evidence is accumulating that mindfulness training has favorable effects on psychological outcomes, but studies on physiological outcomes are limited. Patients with heart disease have a high incidence of physiological and psychological problems and may benefit from mindfulness training. Our aim was to determine the beneficial physiological and psychological effects of online mindfulness training in patients with heart disease., Methods: The study was a pragmatic randomized controlled single-blind trial. Between June 2012 and April 2014 we randomized 324 patients (mean age 43.2 years, 53.7% male) with heart disease in a 2:1 ratio (n = 215 versus n = 109) to a 12-week online mindfulness training in addition to usual care (UC) compared to UC alone. The primary outcome was exercise capacity measured with the 6 minute walk test (6MWT). Secondary outcomes were other physiological parameters (heart rate, blood pressure, respiratory rate, and NT-proBNP), subjective health status (SF-36), perceived stress (PSS), psychological well-being (HADS), social support (PSSS12) and a composite endpoint (all-cause mortality, heart failure, symptomatic arrhythmia, cardiac surgery, and percutaneous cardiac intervention). Linear mixed models were used to evaluate differences between groups on the repeated outcome measures., Results: Compared to UC, mindfulness showed a borderline significant improved 6MWT (effect size, meters: 13.2, 95%CI: -0.02; 26.4, p = 0.050). There was also a significant lower heart rate in favor of the mindfulness group (effect size, beats per minute: -2.8, 95%CI: -5.4;-0.2, p = 0.033). No significant differences were seen on other outcomes., Conclusions: Mindfulness training showed positive effects on the physiological parameters exercise capacity and heart rate and it might therefore be a useful adjunct to current clinical therapy in patients with heart disease., Trial Registration: Dutch Trial Register 3453.

Most infants born with congenital heart disease (CHD) are now expected to reach adulthood. However, adults with CHD of moderate or great complexity remain at elevated risk of heart failure, arrhythmias, additional surgeries and interventional procedures, and premature mortality. This creates a need for lifelong specialized cardiac care and leads to 2 sets of potential challenges: (1) the transition from pediatric to adult care and (2) the psychosocial implications of coping with a chronic and often life-shortening medical condition. Many adolescents struggle with the transition to adult care, and mood and anxiety disorders are not uncommon in the adult setting., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To assess long-term health status and health-related quality of life in survivors of cardiac arrest in childhood and their parents. In addition, to identify predictors of health status and health-related quality of life., Design: This medical follow-up study involved consecutive children surviving cardiac arrest between January 2002 and December 2011, who had been admitted to the ICU. Health status was assessed with a medical interview, physical examination, and the Health Utilities Index. Health-related quality of life was assessed with the Child Health Questionnaires and Short-Form 36., Setting: A tertiary care university children's hospital., Patients: Of the eligible 107 children, 57 (53%) filled out online questionnaires and 47 visited the outpatient clinic (median age, 8.7 yr; median follow-up interval, 5.6 yr)., Interventions: None., Measurements and Main Results: Of the participants, 60% had an in-hospital cardiac arrest, 90% a nonshockable rhythm, and 50% a respiratory etiology of arrest. Mortality rate after hospital discharge was 10%. On health status, we found that 13% had long-term neurologic deficits, 34% chronic symptoms (e.g., fatigue, headache), 19% at least one sign suggestive of chronic kidney injury, and 15% needed special education. Health Utilities Index scores were significantly decreased on most utility scores and the overall Health Utilities Index mark 3 score. Compared with Dutch normative data, parent-reported health-related quality of life of cardiac arrest survivors was significantly worse on general health perception, physical role functioning, parental impact, and overall physical summary. On patient reports, no significant differences with normative data were found. Parents reported better family cohesion and better health-related quality of life for themselves on most scales. Patients' health status, general health perceptions, and physical summary scores were significantly associated with cardiac arrest-related preexisting condition., Conclusions: Considering the impact of cardiac arrest, the overall outcome after cardiac arrest in childhood is reasonably good. Prospective long-term outcome research in large homogeneous groups is needed.

Background: Many patients with congenital heart disease do not meet current public health guidelines to participate in moderate-to-vigorous physical activity for ≥60 minutes per day. They are less fit than their healthy peers. We hypothesized that exercise training would increase cardiopulmonary fitness and daily physical activity in these patients. We therefore assessed effects of an exercise training program on cardiopulmonary fitness and daily physical activity in patients with corrected tetralogy of Fallot (ToF) or Fontan circulation., Methods: In a multicenter prospective controlled trial, patients with ToF or Fontan circulation (age 10-25 years) were randomized, 56 patients to the exercise group and 37 to the control group. The exercise group participated in a 12-week standardized aerobic exercise training program. The control group continued lifestyle as usual. Cardiopulmonary exercise testing and activity measurements were performed before and after 12 weeks., Results: Peak oxygen uptake increased in the exercise group by 5.0% (1.7 ± 4.2 mL/kg per minute; P = .011) but not in the control group (0.9 ± 5.2 mL/kg per minute; P = not significant). Workload increased significantly in the exercise group compared with the control group (6.9 ± 11.8 vs 0.8 ± 13.9 W; P = .047). Subgroup analysis showed a significant increase in pre-to-post peak oxygen uptake in the exercise group of ToF patients but not in the exercise group of Fontan patients. Percentage of measured time spent in moderate-to-vigorous activity at baseline was 13.6% ± 8.6%, which did not significantly change after training., Conclusions: Aerobic exercise training improved cardiopulmonary fitness in patients with ToF but not in patients with Fontan circulation. Exercise training did not change daily physical activity., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: Given the debate around limitations and controversies in type D personality studies, we aimed to evaluate the prognostic value of 'synergistically' analyzed type D personality (interaction z-scores negative affectivity NA, and social inhibition SI) on 10-year mortality and on 10-year subjective health status in percutaneous coronary intervention (PCI) patients., Methods: This prospective study comprised a cohort of 1190 consecutive patients who underwent PCI between October 2001 and September 2002 (73% male, mean age: 62years, range [27-90]years). At baseline, type D personality (DS14), and depression/anxiety (HADS) were assessed. Primary endpoint was 10year all-cause mortality; secondary endpoint was 10-year subjective health status (SF-36)., Results: After a median follow-up of 10.3years (IQR 9.8-10.8), 293 deaths of any cause (24.6%) were recorded. After adjustment for significant baseline characteristics, personality categories approached and dichotomously approached type D personality were associated with 10-year mortality, p<.05. Synergistically approached type D personality was not associated with all-cause mortality or subjective health status at 10years. In survivors, higher NA was associated with lower subjective health status. Type D was not associated with mortality after adjusting for continuous depression and anxiety in all approaches., Conclusions: Synergistically analyzed type D was not associated with 10-year all-cause mortality in PCI patients whereas dichotomous type D was. However, after adjustment for depression most of the findings had disappeared. Depression played an important role in this. Type D was not associated with 10-year subjective health status., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: Research into neuropsychological functioning of survivors of cardiac arrest (CA) in childhood is scarce. We sought to assess long-term neuropsychological functioning in children and adolescents surviving CA., Methods: Neuropsychological follow-up study involving all consecutive children surviving CA between January 2002 and December 2011. Intelligence (IQ), language, attention, memory, visual-spatial, and executive functioning were assessed with internationally validated, neuropsychological tests and questionnaires. Scores were compared with Dutch normative data., Results: Of 107 eligible children, 47 who visited the outpatient clinic (median follow-up interval: 5.6 years) were analyzed. Fifty-five percent had an in-hospital CA, 86% a non-shockable rhythm, and 49% a respiratory-related etiology. CA survivors scored significantly worse on full-scale IQ (mean = 87.3), verbal IQ (mean = 92.7), performance IQ (mean = 85.6), verbal comprehension index (mean = 93.4), perceptual organization index (mean = 83.8), and processing speed index (mean = 91.1), than the norm population (mean IQ = 100). On neuropsychological tests, compared with norms, respectively adjusted for IQ, significantly worse scores were found on visual memory, significantly better on verbal memory (recognition), and comparable outcomes on visual-motor integration, attention, other measures of verbal memory, and executive functioning. On questionnaires, parents reported better executive functioning than the norm, but teachers reported more problems in planning/organizing skills., Conclusions: Long-term neuropsychological assessment of CA survivors showed significant weaknesses, but also relatively intact functioning. As deficits in IQ, memory and executive functioning have significant impact on the child, long-term follow-up and neuropsychological support of CA survivors is warranted.

Background: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD)., Objectives: The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure., Methods: Patients who underwent surgical VSD closure during childhood between 1968 and 1980 were reexamined every 10 years. In 2012, we invited eligible patients to undergo another examination, which included electrocardiography, Holter monitoring, echocardiography, bicycle ergometry, measurement of N-terminal pro-B-type natriuretic peptide, and subjective health assessment., Results: Cumulative survival was 86% at 40 years. Causes of mortality were arrhythmia, heart failure, endocarditis, during valvular surgery, pulmonary hypertension, noncardiac causes, and unknown causes. Cumulative event-free survival after surgery was 72% at 40 years. Symptomatic arrhythmias occurred in 13% of patients and surgical or catheter-based reinterventions in 12%. Prevalence of impaired right ventricular systolic function increased from 1% in 2001 to 17% in 2012 (p=0.001). Left ventricular systolic function was impaired but stable in 21% of patients. Aortic regurgitation occurred more often in the last 20 years (p=0.039), and mean exercise capacity decreased (p=0.003). N-terminal pro-B-type natriuretic peptide (median: 11.6 pmol/l [interquartile range: 7.0 to 19.8 pmol/l]) was elevated (>14 pmol/l) in 38% of patients. A concomitant cardiac lesion, for example, patent ductus arteriosus, and aortic cross-clamp time were determinants of late events (hazard ratio: 2.84 [95% confidence interval: 1.23 to 6.53] and hazard ratio: 1.47 per 10 min [95% confidence interval: 1.22 to 1.99], respectively). Patients rated their subjective health status significantly better than a reference population., Conclusions: Survival up to 40 years after successful surgical VSD closure is slightly lower than in the general Dutch population. Morbidity is not negligible, especially in patients with a concomitant cardiac lesion., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: Investigating long-term psychosocial and emotional outcomes, 30-43 years after cardiothoracic surgery during childhood., Methods: Adult patients (N = 252, aged 33-55 years) all operated between 1968 and 1980 for atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot or transposition of the great arteries (TGA, all Mustard) were evaluated in the Erasmus MC. Psychosocial and emotional functioning were measured using standardized, validated questionnaires. Results in 2011 were compared with those found in 2001., Results: Compared with normative data, the total sample showed significant impairments on living conditions, relationships, occupational level and income, but a better quality of life and emotional functioning. Patients with moderate/complex ConHD reported significantly more physical restrictions and felt more often at a disadvantage because of their ConHD compared with those with simple ConHD. From 2001 to 2011, outcomes on biographical characteristics (living conditions, marital status and income) improved significantly, though still impairments were found., Conclusion: At 30-43 year follow-up, compared to normative data, patients had a better quality of life and emotional functioning, despite impairments on occupational level and income. Compared to patients with simple defects, patients with moderate/complex ConHD scored worse on multiple outcomes, which warrants special attention for these patients.

Background: Exercise can improve physical fitness in children and adults with congenital heart disease. We hypothesized that exercise training would not lead to adverse cardiac remodelling in this population., Methods and Results: This multi-centre randomized controlled trial included children and young adults (10 to 25 years) with either corrected tetralogy of Fallot or Fontan circulation. The exercise-group was enrolled in a 12 week standardized aerobic dynamic exercise training program. The control-group continued their life-style and received care as usual. Both groups underwent cardiopulmonary exercise testing, cardiac magnetic resonance imaging (MRI), echocardiography and neurohormonal assessment, within 2 weeks before and 2 weeks after the intervention period. Fifty-six patients were randomized to the exercise-group and 37 to the control-group. We assessed changes between the pre- and the post-intervention period for the exercise group compared to the changes in the control-group. Peak load increased significantly in the exercise-group compared to the control-group (exercise-group 6.9 ± 11.8 W; control-group 0.8 ± 13.9 W; p=0.047). There were no adverse events linked to the study. Ventricular systolic parameters, cardiac dimensions and neurohormonal markers during follow-up did not change in patients allocated to the exercise-group and control-group. Although there were some isolated minor changes in inflow parameters, there was no consistent pattern of changes, indicating a lack of true change in the diastolic function., Conclusion: We demonstrated that no clinically relevant adverse cardiac remodelling occurred after 12 weeks of exercise training in patients with either corrected tetralogy of Fallot or Fontan circulation., Clinical Trial Registration: www.trialregister.nl, identification NTR2731., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: It is unclear whether sports participation in adults with repaired congenital heart disease is safe and has benefits., Methods: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis, Tetralogy of Fallot or Transposition of the Great Arteries in our center between 1968 and 1980 were included, and participated in our longitudinal follow-up study with serial evaluations in 2001 and 2011. At both time points patients filled in questionnaires on sports participation, subjective physical functioning and quality of life. Exercise testing, echocardiogram and 24-hour continuous ambulatory ECG-monitoring were performed in both 2001 and 2011. All clinical events (re-intervention, arrhythmia, heart failure) were prospectively recorded., Results: No relationship was found between practicing sports and the occurrence of sudden death, PVCs or SVTs. Patients with moderate/complex forms of ConHD practiced fewer hours of sports compared with the general Dutch normative population. Patients with both simple and moderate/complex ConHD who practiced sports showed a higher exercise capacity. More favorable subjective physical functioning was found for moderate/complex patients who practiced sports., Conclusions: Adults with repaired ConHD are less often involved in sports than the Dutch general population. The patients that were engaged in sports show a higher exercise capacity than those who did not. Sports participation in patients with ConHD was not associated with an increased incidence of adverse cardiac events., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: It is of debate whether or not childhood anxiety disorders (AD) can be captured by one taxonomic construct. This study examined whether perceived arousal (PA), autonomic nervous system (ANS) and hypothalamic-pituitary-adrenal (HPA) axis measures can distinguish children with different primary diagnoses of clinical anxiety disorders (AD) from each other, and from a general population reference group (GP)., Methods: The study sample consisted of 152 AD children (comparing separation anxiety disorder, generalized anxiety disorder, social phobia and specific phobia), aged 8- to 12-years, and 200 same-aged reference children. HPA-axis functioning was measured by a diurnal cortisol profile. ANS functioning was measured by continuous measures of skin conductance level in rest and during a mental arithmetic task and high frequency heart rate variability in rest. PA was assessed by a questionnaire., Results: The AD sample showed lower high frequency heart rate variability during rest, heightened anticipatory PA, higher basal and reactive skin conductance levels and lower basal HPA-axis functioning compared to the GP sample. The existence of three or more clinical disorders, i.e. a high clinical 'load', was associated with lower basal HPA-axis functioning, higher skin conductance level and lower posttest PA. Specific phobia could be discerned from social phobia and separation anxiety disorder on higher skin conductance level., Conclusions: Our findings indicated that children with AD have specific psychophysiological characteristics, which resemble the psychophysiological characteristics of chronic stress. A high clinical 'load' is associated with an altered ANS and HPA-axis functioning. Overall, ANS and HPA-axis functioning relate to AD in general, accept for specific phobia., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients, aged 10-15, were randomly allocated (stratified by age, gender, and congenital heart disease) to a 12-week period with either: (a) 3 times per week standardized exercise training or (b) care-as-usual (randomization ratio 2:1). Adolescents and their parents filled in online questionnaires at baseline and at 12-week follow-up. In this randomized controlled trail, primary analyses involved influence of parental mental health and parental social support for exercise on changes in the TNO/AZL Child Quality of Life Questionnaire Child Form at follow-up. Secondary analyses concerned comparing levels of parental characteristics with normative data. Compared with controls, adolescents in the exercise group reported a decrease in social functioning when their parents had more anxiety/insomnia or severe depression themselves. Adolescents also reported a decrease in social functioning when their parents showed poorer overall mental health themselves. Parents reported comparable or even better mental health compared with normative data. The effect of a standardized exercise program on HRQoL changes in adolescents with ToF or a Fontan circulation is moderated by parental mental health, more specifically by parental anxiety/insomnia and severe depression. The trial registration number of this article is NTR2731 ( www.trialregister.nl ).

Introduction: Cystic fibrosis (CF) lung disease is characterized by bronchiectasis and trapped air on chest computed tomography (CT)., Objective: We aim to validate bronchiectasis and trapped air as outcome measures by evaluating associations between changes in bronchiectasis, trapped air and patient-reported respiratory symptoms., Methods: A longitudinal cohort study has been conducted. CF patients (aged 6-19 years) who had two routine CTs and completed twice a Cystic Fibrosis Questionnaire-Revised within 2 years (referred to as T1 and T2 ), in the period of July 2007 to January 2012 were included. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and student's paired t tests were performed., Results: In total 40 patients were included with a median age at T1 of 12.6 years (range 6-17 years), and at T2 14.5 years (range 8-19 years). At T1 , bronchiectasis (r = -0.49, P < 0.01) and trapped air (r = -0.34, P = 0.04) correlated with CFQ-R Respiratory Symptoms Scores (CFQ-R RSS). At T2 similar correlations were found with the CFQ-R RSS. Over 2 years, there was significant progression in bronchiectasis (P = 0.03) and trapped air (P = 0.03), but not in CFQ-R RSS. Changes in bronchiectasis and trapped air were not associated with changes in CFQ-R RSS., Conclusion: Our results indicate that bronchiectasis and trapped are sensitive outcome measures in CF lung disease, showing a significant association with CFQ-R RSS at two-time points. However, progression of bronchiectasis and trapped air over 2 year does not necessarily correlate to changes in quality of life., (© 2014 Wiley Periodicals, Inc.)

Background: Prospective data on long-term survival and clinical outcome beyond 30 years after surgical correction of tetralogy of Fallot are nonexistent., Methods and Results: This longitudinal cohort study consists of the 144 patients with tetralogy of Fallot who underwent surgical repair at <15 years of age between 1968 and 1980 in our center. They are investigated every 10 years. Cumulative survival (data available for 136 patients) was 72% after 40 years. Late mortality was due to heart failure and ventricular fibrillation. Seventy-two of 80 eligible survivors (90%) participated in the third in-hospital investigation, consisting of ECG, Holter, echocardiography, cardiopulmonary exercise testing, N-terminal pro-brain natriuretic peptide measurement, cardiac magnetic resonance (including dobutamine stress testing), and the Short Form-36 questionnaire. Median follow-up was 36 years (range, 31-43 years). Cumulative event-free survival was 25% after 40 years. Subjective health status was comparable to that in the normal Dutch population. Although systolic right and left ventricular function declined, peak exercise capacity remained stable. There was no progression of aortic root dilation. A previous shunt operation, low temperature during surgery, and early postoperative arrhythmias were found to predict late mortality (hazard ratio, 2.9, 1.1, and 2.5, respectively). An increase in QRS duration and a deterioration of exercise tolerance and ventricular dysfunction did not predict mortality. Insertion of a transannular patch was a predictor for late arrhythmias (hazard ratio, 4.0; 95% confidence interval, 1.2-13.4)., Conclusions: Although many patients needed a reoperation or developed arrhythmias, late mortality was low, and the clinical condition and subjective health status of most patients remained good. Previous shunt, low temperature during surgery, and early postoperative arrhythmias were found to predict late mortality., (© 2014 American Heart Association, Inc.)

Background: Children and adolescents operated upon for congenital heart disease (ConHD) may show reduced exercise capacity and physical activity, possibly associated with lowered self-esteem and quality of life (QoL). The studies into associations between these parameters have not been reviewed before., Objective: Review of studies into associations between exercise capacity, physical activity, respectively exercise training, and psychosocial functioning of ConHD youngsters., Data Sources: PubMed, Embase and reference lists of related articles., Study Selection: Articles published between January 2000 and December 2012 into exercise capacity and/or physical activity, and a measure of psychosocial functioning in children with ConHD., Data Extraction: Two investigators independently reviewed the identified articles for eligibility, and one author extracted the data., Results: Although exercise capacity was strongly related to physical domains of parent-reported and self-reported QoL, it was almost never associated with psychosocial domains of QoL. Physical activity was rarely associated with physical or psychosocial domains of QoL. Remarkably, self-reported depressive symptoms were associated with both physical and psychosocial QoL. The few studies into exercise-training programmes showed promising results in QoL and emotional and behavioral problems, but they contained methodological flaws., Conclusions: No clear relationships were found between exercise capacity, physical activity, and QoL in children and adolescents with ConHD. Therefore we recommend assessing QoL separately, preferably both self-reported and parent-reported. Since depressive symptoms were associated with reduced physical and psychosocial QoL, screening on these symptoms is also recommended., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Objective: The aim of this study was to evaluate the effects of a standardized exercise program on sports enjoyment and leisure-time spending in adolescents with congenital heart disease and to know what the moderating impact of their baseline health behavior and disease knowledge is., Methods: Included were 93 patients, aged 10 to 25, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology, of 5 participating centers of pediatric cardiology in The Netherlands. They were randomly allocated, stratified for age, gender, and type of congenital heart disease to a 12-week period with either: (1) three times per week standardized exercise training or (2) care as usual (randomization ratio 2:1). At baseline and after 12 weeks, participants completed Web-based questionnaires and were interviewed by phone., Outcome Measures: Primary analyses tested changes from baseline to follow-up in sports enjoyment and leisure-time spending in the exercise group vs. control group. Secondary analyses concerned the moderating influence of baseline health behavior and disease knowledge on changes from baseline to follow-up, and comparison with normative data., Results: At follow-up, the exercise group reported a decrease in passive leisure-time spending (watching television and computer usage) compared with controls. Exercise training had no effect on sports enjoyment and active leisure-time spending. Disease knowledge had a moderating effect on improvement in sports enjoyment, whereas health behavior did not. Compared with normative data, patients obtained similar leisure time scores and lower frequencies as to drinking alcohol and smoking., Conclusions: Exercise training decreased passive, but not active, leisure-time spending. It did not influence sports enjoyment., (© 2013 Wiley Periodicals, Inc.)

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.

Aims: To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair., Methods and Results: This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status was obtained of 86 patients. Median follow-up was 35 (IQR 34-38) years. Cumulative survival was 84% after 10 years, 80% after 20 years, 77% after 30 years, and 68% after 39 years. Cumulative survival free of events (i.e. heart transplantation, arrhythmias, reintervention, and heart failure) was 19% after 39 years. Reinterventions were mainly required for baffle-related problems. Supraventricular and ventricular arrhythmias occurred in 28 and 6% of the patients, respectively. Pacemaker and/or ICD implantation was performed in 39%. Fifty survivors participated in the current in-hospital investigation including electrocardiography, 2D-echocardiography, cardiopulmonary-exercise testing, NT-proBNP measurement, Holter monitoring, and cardiac magnetic resonance. Right ventricular systolic function was impaired in all but one patient at last follow-up, and 14% developed heart failure in the last decade. NT-proBNP levels [median 31.6 (IQR 22.3-53.2) pmol/L] were elevated in 92% of the patients. Early postoperative arrhythmias were a predictor for late arrhythmias [HR 3.8 (95% CI 1.5-9.5)], and development of heart failure [HR 8.1 (95% CI 2.2-30.7)]. Also older age at operation was a predictor for heart failure [HR 1.26 (95% CI 1.0-1.6)]., Conclusion: Long-term survival after Mustard repair is clearly diminished and morbidity is substantial. Early postoperative arrhythmias are a predictor for heart failure and late arrhythmias., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Purpose: To evaluate effects of an exercise program on health-related quality of life (HRQoL) in children and adolescents with tetralogy of Fallot (ToF) or a Fontan circulation., Methods: Stratified, randomized, controlled intervention study conducted in five participating centers of pediatric cardiology in The Netherlands. In total, 93 patients, aged 10-25 years, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology were included. They were randomly allocated with a ratio of 2:1 to: (1) a 12-week period with an exercise program for 3 times per week or (2) to a control group. Randomization was stratified by age, gender, and cardiac diagnosis. At baseline and follow-up after 12 weeks, all participants completed Web-based age-appropriate HRQoL questionnaires. Primary analyses involved change in HRQoL during follow-up. Secondary analyses concerned influence of cardiac diagnosis and comparison with normative data., Results: Forty-eight (86%) and 32 (86%) patients in the exercise-group and control-group respectively completed all questionnaires at baseline and follow-up. Compared with the control-group, children, aged 10-15 years, in the exercise-group improved significantly on self-reported cognitive functioning, p < .05, r = .30, and parent-reported social functioning, p < .05, r = .30. Youngsters aged 16-25 years did not change their HRQoL. Cardiac diagnosis had no influence on pre/post changes. Children and youngsters in this study reported comparable or better HRQoL than norm groups., Conclusions: Participation in an exercise program improved HRQoL of children with ToF or a Fontan circulation, especially in those with low baseline QoL., (Copyright © 2014 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study was to investigate whether the objective medical parameters related to congenital heart disease and patients' ratings of cardiac disease severity were related to psychological well-being and illness behaviour during the pre-operative period., Methods: A total of 143 patients (63 male patients; 16-73 years old) with congenital heart disease evaluated the severity of their cardiac condition using a numerical rating scale ranging from 0, indicating the least severe condition, to 100, indicating the most severe condition. Psychological well-being was assessed using the Psychological General Well-Being Index (total score ≤ 60 indicating severe distress) and illness behaviour using the Illness Behavior Questionnaire., Results: Pre-operative psychological well-being was not related to the objective medical parameters reflecting cardiac disease severity. In contrast, total psychological well-being scores correlated significantly with patients' subjective ratings of disease severity (p < 0.001). When compared with the reference values from the Italian population, the mean scores of the patients on psychological well-being were similar. As regards the Illness Behavior Questionnaire, the scores on denial were higher and those on hypochondria were lower compared with other hospitalised patients., Conclusions: This study shows that the perception of cardiac disease severity, and not the medical parameters in congenital heart disease, is related to the patients' pre-operative psychological state. Thus, more importance needs to be given to assessing the patients' pre-operative perception and psychological state independently of cardiac severity. Targeted interventions with regard to the cardiac condition are recommended.

Objectives: To investigate whether analgesic and sedative drug use during PICU treatment is associated with long-term neurodevelopmental outcome in children who survived meningococcal septic shock., Design: This study concerned a secondary analysis of data from medical and psychological follow-up of a cross-sectional cohort of all consecutive surviving patients with septic shock and purpura requiring intensive care treatment between 1988 and 2001 at the Erasmus MC-Sophia Children's Hospital. At least 4 years after PICU admission, these children showed impairments on several domains of neuropsychological functioning. In the present study, type, number, and dose of sedatives and analgesics were retrospectively evaluated., Setting: Tertiary care university hospital., Patients: Seventy-seven meningococcal septic shock survivors (median age, 2.1 yr)., Interventions: None., Measurements and Main Results: Forty-five patients (58%) received one or more analgesic and/or sedative drugs during PICU admission, most commonly benzodiazepines (n = 39; 51%), followed by opioids (n = 23; 30%). In total, 12 different kinds of analgesic or sedative drugs were given. The use and dose of opioids were significantly associated with poor test outcome on full-scale intelligence quotient (p = 0.02; Z = -2.28), verbal intelligence quotient (p = 0.02; Z = -2.32), verbal intelligence quotient subtests (verbal comprehension [p = 0.01; Z = -2.56] and vocabulary [p = 0.01; Z = -2.45]), and visual attention/executive functioning (Trial Making Test part B) (p = 0.03; Z = -2.17). In multivariate analysis adjusting for patient and disease characteristics, the use of opioids remained significant on most neuropsychological tests., Conclusions: The use of opioids during PICU admission was significantly associated with long-term adverse neuropsychological outcome independent of severity of illness scores in meningococcal septic shock survivors.