To save content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about saving content to .
To save content items to your Kindle, first ensure firstname.lastname@example.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Children with congenital heart disease and their families are at risk of psychosocial problems. Emotional and behavioural problems, impaired school functioning, and reduced exercise capacity often occur. To prevent and decrease these problems, we modified and extended the previously established Congenital Heart Disease Intervention Program (CHIP)–School, thereby creating CHIP-Family. CHIP-Family is the first psychosocial intervention with a module for children with congenital heart disease. Through a randomised controlled trial, we examined the effectiveness of CHIP-Family.
Ninety-three children with congenital heart disease (age M = 5.34 years, SD = 1.27) were randomised to CHIP-Family (n = 49) or care as usual (no psychosocial care; n = 44). CHIP-Family consisted of a 1-day group workshop for parents, children, and siblings and an individual follow-up session for parents. CHIP-Family was delivered by psychologists, paediatric cardiologists, and physiotherapists. At baseline and 6-month follow-up, mothers, fathers, teachers, and the child completed questionnaires to assess psychosocial problems, school functioning, and sports enjoyment. Moreover, at 6-month follow-up, parents completed program satisfaction assessments.
Although small improvements in child outcomes were observed in the CHIP-Family group, no statistically significant differences were found between outcomes of the CHIP-Family and care-as-usual group. Mean parent satisfaction ratings ranged from 7.4 to 8.1 (range 0–10).
CHIP-Family yielded high program acceptability ratings. However, compared to care as usual, CHIP-Family did not find the same extent of statistically significant outcomes as CHIP-School. Replication of promising psychological interventions, and examination of when different outcomes are found, is recommended for refining interventions in the future.
Tricuspid valvectomy is a rare surgical intervention, and knowledge regarding long-term outcome in children is lacking. We report a favourable outcome 11 years after tricuspid valvectomy in early infancy without subsequent surgery or other cardiac interventions. Specific criteria for timing of re-intervention are lacking. Application of adult tricuspid and pulmonary regurgitation recommendations is helpful but has limitations.
Myocardial deformation by speckle tracking echocardiography provides additional information on left ventricular function. Values of myocardial deformation (strain and strain rate) depend on the type of ultrasound machine and software that is used. Normative values for QLAB (Philips) are scarce, especially for children. It is important to evaluate the influence of age and body size on myocardial deformation parameters, since anthropometrics strongly influence many standard echocardiographic parameters. The aim of this study was to provide comprehensive normal values for myocardial deformation of the left ventricle using a Philips platform and to evaluate the association with anthropometric and standard echocardiographic parameters.
Healthy children between 1 and 18 years of age were prospectively examined using a standard echocardiographic protocol. Short-axis and apical four-chamber, two-chamber, and three-chamber views were used to measure peak systolic circumferential and longitudinal strain and systolic and early diastolic strain rate of the left ventricle using dedicated software.
A total of 103 children were included with a mean age of 10.8 and inter-quartile range 7.3–14.3 years. Global circumferential strain values (±SD) were −24.2±3.5% at basal, −25.8±3.5% at papillary muscle, and −31.9±6.2% at apex levels. Global left ventricular longitudinal strain values were −20.6±2.6% in apical four-chamber view, −20.9±2.7% in apical two-chamber, and −21.0 ±2.7% in apical three-chamber. Age was associated with longitudinal strain, longitudinal systolic and early diastolic strain rate, but not with circumferential strain.
Normal values for left ventricular deformation parameters in children are obtained using a Philips platform. Age partly explains normal variation of strain and strain rate.
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).
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.
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.
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.
If parents/patients reported a need for psychosocial care, referral to adequate mental health-care professionals was arranged.
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.
Results show that psychosocial care is rated as (very) important by both parents and children during an extremely stressful period of their life.
The Fontan circulation is the optimal treatment for patients with univentricular hearts. These patients are at high risk of circulatory failure. There is no consensus on the optimal drug treatment for the prevention of failure of the Fontan circulation. The aim of this systematic review was to provide an overview of evidence for drug therapy used in the prevention of Fontan circulatory failure.
We searched the Embase database for articles that reported drug therapy in Fontan patients. Studies published between 1997 and 2014 were included if efficacy or safety of medication was assessed, drug therapy aimed to prevent or treat failure of the Fontan circulation, and if the full text was available. Case reports were excluded.
A total of nine studies were included with a total of 267 Fontan patients; four studies evaluated the medication sildenafil, one iloprost, three bosentan, and one enalapril. Among all, two sildenafil studies reported improvement in exercise capacity, one in exercise haemodynamics, and one in ventricular performance. In the largest study of bosentan, an increase in exercise capacity was found. Enalapril did not result in improvements.
The studies analysed in this review suggest that bosentan, sildenafil, and iloprost may improve exercise capacity at the short term. Given the limitations of the studies, more, larger, placebo-controlled studies with longer follow-up periods are needed to better understand which drug therapies are effective in the prevention of failure of the Fontan circulation.
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.
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.
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.
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.
The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references.
A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995. In all, 42 patients were included and underwent a cross-sectional evaluation including echocardiography, magnetic resonance imaging, exercise testing, and 24-hour ambulatory electrocardiography. Patients were matched with healthy controls for gender, body surface area, and age.
The mean age at surgery was 4.6 plus or minus 2.8 years, and the mean age at follow-up was 16 plus or minus 3 years. There were no residual intracardiac lesions. The mean right ventricular end-systolic volume was significantly larger in patients (142 ± 26 millilitres) than in references (137 ± 28 millilitres; p = 0.04). In 25% of the patients, right ventricular end-systolic volume was larger than the 95th percentile for references. No relevant arrhythmias were detected. Exercise testing did not reveal differences with healthy references: maximal power (169 ± 43 Watt patients versus 172 ± 53 controls; p = 0.8), maximal oxygen uptake (38 ± 8 versus 41 ± 13 millilitres per minute per kilogram; p = 0.1).
After surgical closure of secundum-type atrial septal defect, right ventricular end-systolic volume is increased. These findings have no impact on rhythm status or exercise capacity at this stage of follow-up, but may have implications for the timing of surgery or the technique of closure if confirmed in longer follow-up.
In patients with coronary arterial disease, stress imaging is able to demonstrate abnormalities in the motion of the ventricular walls, and abnormalities in coronary arterial perfusion not apparent at rest. It can also provide information on prognostic factors. In patients with congenitally malformed hearts, stress imaging is used to determine contractile reserve, abnormalities of mural motion, and global systolic function, but also to assess diastolic and vascular function. In most of these patients, stress is usually induced using pharmacological agents, mainly dobutamine given in varying doses. The clinical usefulness of abnormal responses to the stress induced in such patients has to be addressed in follow-up studies. The abnormal stress might serve as surrogate endpoints, predicting primary endpoints at an early stage, which are useful for stratification of risk in this population of growing patients. We review here the stress imaging studies performed to date in patients with congenitally malformed hearts, with a special emphasis on echocardiography and cardiac magnetic resonance imaging.
To assess the level of psychological distress and styles of coping in both mothers and fathers of children who underwent invasive treatment for congenital cardiac disease at least 7 years and 6 months ago.
The General Health Questionnaire and the Utrecht Coping List were completed by parents of children with 4 different cardiac diagnoses.
Overall, in comparison with reference groups, parents of children treated for congenital heart disease showed lower levels of distress, manifested as lower levels of somatic symptoms, anxiety and sleeplessness and serious depression. Mothers of children with congenital heart disease reported significantly more somatic symptoms than fathers.
Further, compared to reference groups more favourable outcomes on coping were found; parents in our sample showed a weaker tendency to use styles of coping such as reassuring thoughts and less often expressed negative emotions (anger, annoyance). Mothers in our sample appeared to seek social support more often compared to fathers.
Overall, lower levels of psychological distress and few differences in styles of coping compared to reference groups were found in parents of children treated for congenital heart disease. We need to remain alert however, for individual parents at risk of adjusting poorly.
We report on the management of Candida endocarditis in a 5-month old infant. The orifice of the tricuspid valve was totally obstructed, and the tension apparatus of the valve destroyed. Excision of the valve led to severe failure of the right heart. The combined use of anti-failure treatment, and reduction of right ventricular afterload with oxygen, nitric oxide and sildenafil, proved successful.