Cardiovascular disease (CVD) is excessively prevalent and premature in bipolar disorder (BD), even after controlling for traditional cardiovascular risk factors. The increased risk of CVD in BD may be subserved by microvascular dysfunction. We examined coronary microvascular function in relation to youth BD.

Participants were 86 youth, ages 13–20 years (n = 39 BD, n = 47 controls). Coronary microvascular reactivity (CMVR) was assessed using quantitative T2 magnetic resonance imaging during a validated breathing-paradigm. Quantitative T2 maps were acquired at baseline, following 60-s of hyperventilation, and every 10-s thereafter during a 40-s breath-hold. Left ventricular structure and function were evaluated based on 12–15 short- and long-axis cardiac-gated cine images. A linear mixed-effects model that controlled for age, sex, and body mass index assessed for between-group differences in CMVR (time-by-group interaction).

The breathing-paradigm induced a significant time-related increase in T2 relaxation time for all participants (i.e. CMVR; β = 0.36, p < 0.001). CMVR was significantly lower in BD v. controls (β = −0.11, p = 0.002). Post-hoc analyses found lower T2 relaxation time in BD youth after 20-, 30-, and 40 s of breath-holding (d = 0.48, d = 0.72, d = 0.91, respectively; all pFDR < 0.01). Gross left ventricular structure and function (e.g. mass, ejection fraction) were within normal ranges and did not differ between groups.

Youth with BD showed evidence of subclinically impaired coronary microvascular function, despite normal gross cardiac structure and function. These results converge with prior findings in adults with major depressive disorder and post-traumatic stress disorder. Future studies integrating larger samples, prospective follow-up, and blood-based biomarkers are warranted.

Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation.

The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults’ post-health care transition.

A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed.

Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations.

The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.

Post-traumatic stress disorder occurs in parents of infants with CHD, contributing to psychological distress with detrimental effects on family functioning and well-being. We sought to determine the prevalence and factors associated with post-traumatic stress disorder symptoms in parents whose infants underwent staged palliation for single ventricle heart disease.

A large longitudinal multi-centre cohort study evaluated 215 mothers and fathers for symptoms of post-traumatic stress disorder at three timepoints, including post-Norwood, post-Stage II, and a final study timepoint when the child reached approximately 16 months of age, using the self-report questionnaire Impact of Event Scale – Revised.

The prevalence of probable post-traumatic stress disorder post-Norwood surgery was 50% of mothers and 39% of fathers, decreasing to 27% of mothers and 24% of fathers by final follow-up. Intrusive symptoms such as flashbacks and nightmares and hyperarousal symptoms such as poor concentration, irritability, and sudden physical symptoms of racing heart and difficulty breathing were particularly elevated in parents. Higher levels of anxiety, reduced coping, and decreased satisfaction with parenting were significantly associated with symptoms of post-traumatic stress disorder in parents. Demographic and clinical variables such as parent education, pre-natal diagnosis, medical complications, and length of hospital stay(s) were not significantly associated with symptoms of post-traumatic stress disorder.

Parents whose infants underwent staged palliation for single ventricle heart disease often reported symptoms of post-traumatic stress disorder. Symptoms persisted over time and routine screening might help identify parents at-risk and prompt referral to appropriate supports.

Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.

We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design.

Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field.

Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.

A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice.

A web-based survey was distributed via e-mail to over 2000 paediatric cardiologists, intensivists, cardiothoracic surgeons, and cardiac advance practice nurses during three distribution periods. The results were analysed using McNemar’s test for paired data and Fisher’s exact test.

The response rate was 31.5% (69% cardiologists and 65% with >10 years of experience). Among respondents familiar with trial results, 74% reported current practice consistent with trial findings versus 48% before trial publication (p<0.001); 19% used angiotensin-converting enzyme inhibitor in this population “almost always” versus 36% in the past (p<0.001), and 72% reported a change in management or improved confidence in treatment decisions involving this therapy based on the trial results. Respondents familiar with trial results (78%) were marginally more likely to practise consistent with the trial results than those unfamiliar (74 versus 67%, p=0.16). Among all respondents, 28% reported less frequent use of angiotensin-converting enzyme inhibitor over the last 3 years.

Within 5 years of publication, the majority of respondents was familiar with the Infant Single Ventricle Trial results and reported less frequent use of angiotensin-converting enzyme inhibitor in single-ventricle infants; however, 28% reported not adjusting their clinical decisions based on the trial’s findings.

A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.

We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).

A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.

After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles.

Foetuses were included who had a left ventricle that was 2–4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models.

From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean −3.8 versus −4.2 intervention group, p = 0.04, odds ratio 2.8).

The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography.

To determine the gross motor skills of school-aged children after the Fontan procedure and compare the locomotor and object control skills with normative data.

This study followed a cross-sectional design.

This study was based on hospital outpatient visit, with accelerometry conducted at home.

This study included 55 patients, including 22 girls in the age group of 6–10 years, 5.1 years after Fontan.

Test of Gross Motor Development – Version 2, daily activity by accelerometer, medical history review, child and parent perceptions of activity.

Being involved in active team sports increased locomotor percentile score by 10.3 points (CI: 4.4, 16.1). Preference for weekend outdoor activities (6.9, CI: 2.0, 11.8), performing at least 30 minutes of moderate-to-vigorous physical activity daily (24.5, CI: 7.3, 41.8), and reporting that parents seldom criticise the child's physical activity (21.8, CI: 8.9, 34.8) were also associated with higher locomotor percentile scores (p < 0.01). Object control percentile scores were higher (p < 0.03) with involvement in formal instruction (5.9, CI: 1.1, 10.6) and being restricted to “activities within comfortable limits” (27.6, CI: 7.7, 47.5). Older chronological age (r = 0.28), a more complicated medical history (r = 0.36), and older age at Fontan (r = 0.28) were associated with greater skill delay (p < 0.04).

Children after Fontan attain basic motor skills at a later age than their peers, and deficits continue for more complex skills as age increases, suggesting a need for longitudinal monitoring of gross motor skill development through the elementary school years. Future research might investigate whether a gross motor skill rehabilitation programme can provide these children with the motor skills needed to successfully participate in a physically active lifestyle with peers.

To identify child and parental factors associated with screen time in 3-year-old children.

Observational study.

Participants were recruited from a large primary-care paediatric group practice in Toronto, Canada.

Healthy 3-year-old children were included. A questionnaire was completed by their parents on screen time. Descriptive statistics and linear regression models were used to assess associations between child screen time and selected factors. Multivariable models included factors from the univariate analysis with P < 0·1. Estimated effects and 95 % CI are reported.

A total of 157 children were enrolled (91 % recruitment). The mean screen time per weekday was 104 min (similar for weekend day). In all, 10 % of children had a television (TV) in their bedroom; 59 % consumed at least one meal while watching TV; and 81 % of parents had household rules about screen time. Controlling for maternal education and age, eating lunch and dinner in front of the screen and mother being employed were associated with an increase in child weekday screen time of 96 (95 % CI 30, 192), 42 (95 % CI 12, 90) and 36 (95 % CI 6, 72) min/d, respectively. Eating lunch in front of the screen and an increase of 1 h of parental screen time were associated with an increase of 78 (95 % CI 36, 132) and 12 (95 % CI 6, 18) min/d in child weekend screen time. Family rules decreased child weekend screen time by 30 (95 % CI 6, 54) min/d.

Interventions that include these important parental factors should be evaluated for their effectiveness in reducing screen time.

Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.

A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls.

Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case–control differences for patients at moderate altitude were greater for peak oxygen consumption (−29% against −13%, p = 0.04), anaerobic threshold (−36% against −5%, p = 0.001), and oxygen pulse (−35% against −18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively).

Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.

Functional health is becoming an important part of outcome assessment following congenital heart surgery.

The Child Health Questionnaire was used to evaluate self-reported functional health in a cohort of children operated on for congenital heart disease between 1996 and 2002, now aged 10–20 years. A total of 288 schoolchildren served as controls. The association between demographic and clinical factors such as the Risk Adjusted Classification for Congenital Heart Surgery, the Aristotle Basic Complexity Score, physical and psycho-social domains was explored by multivariate analysis.

In total 239 children who were operated on (response rate 68%, mean age at assessment 13.1 years, 50% male children) participated. There were no differences between children operated on for congenital heart disease and controls in nine out of thirteen domains. In multivariate analysis, male gender was positively associated with physical, mental and general health. Higher education of the parents was also associated with better scores for family activities, physical, emotional and general health. In contrast, living with a single parent was negatively associated with mental health. Category 4 in the Risk Adjusted Classification for Congenital Heart Surgery was associated with worse scores in all behaviour domains. The Aristotle Basic Complexity Score was not associated with any domain.

Functional health in children operated for congenital heart disease was overall similar to other children of the same age. Male gender of the child, education of the parents, living with a single parent, and category 4 in the Risk Adjusted Classification for Congenital Heart Surgery were important factors for functional health.

Challenges inherent in researching rare congenital cardiac lesions led to creation of the Congenital Heart Surgeons’ Society Data Center (Data Center) two decades ago. The Data Center pools experiences from up to 60 institutions, and over 4,700 children have been prospectively recruited within nine diagnostic inception cohorts. This report describes the operations of our research database, with particular focus on analytic strategies employed.

A procedural log is created of all investigations and interventions, and reports from enrolling institutions are subsequently obtained. Cross-sectional follow-up is undertaken annually by the Data Center. All data are linked to the individual child, and quality control mechanisms ensure that completeness and accuracy are maximised. Specific advantages of Data Center analytic approaches include multi-phase parametric hazard analysis, re-sampling techniques for reliable risk factor identification, competing risks methodology, and propensity-adjusted comparisons. Virtues of applying these techniques to a research database are illustrated by clinically pertinent questions that have been addressed in place of what would be difficult through randomised trials.

The Data Center is a cost-effective, versatile tool for researching congenital cardiac surgical outcomes. Research databases are ideally suited to in-depth investigations of survival and functional outcomes. Multi-center propensity-adjusted analyses represent efficient surrogates for randomised trials. Well-designed observational prospective studies should remain a principle mode of researching congenital cardiac disease.

While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.