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The aim of this pilot study was to explore whether executive functioning (EF) problems of children with severe congenital heart disease (CHD) could be improved through a personalized EF-training program. Eleven preschool-aged children (mean age = 62 months, SD = 6) received a standardized test battery. Eight children were eligible for training and six children participated in the training and posttest. Due to the small sample size, only effect sizes were analyzed. Clinically relevant improvements were found with large effect sizes. Findings indicate that a personalized EF-training program could be effective in improving EF of children with severe CHD. Further research is needed.

Competing Interests: Conflict of interest: none declared.

Aims: To understand the compliance, influencing factors, and action path of family cardiac rehabilitation exercise prescriptions for children after congenital heart disease surgery., Methods and Results: A random sampling method was used to select 200 paediatric patients and their parents from a paediatric hospital in Shanghai. Among them, 57 cases (28.5%) of children's families followed the cardiac rehabilitation exercise prescription. Path analysis showed that peak oxygen uptake exerted a negative impact on the compliance of family cardiac-rehabilitation prescriptions for patients after congenital heart disease surgery through doctor-patient trust, with a standardized path coefficient of -0.246 (P = 0.001). Disease-related knowledge exerted a positive effect on the compliance of family cardiac-rehabilitation prescriptions for children after congenital heart surgery through doctor-patient trust, with a standardized path coefficient of 0.353 (P < 0.001). The dimension of friend support in social support had a direct positive effect on the compliance of family cardiac-rehabilitation prescriptions for children after cardiac surgery, with a standardized path coefficient of 0.641 (P = 0.006)., Conclusion: The compliance of cardiac rehabilitation exercise prescription in children with congenital heart disease is not good and is affected by many factors, and there is a complex path relationship between various factors; the kilogram oxygen consumption of the child, the disease-related knowledge of the caregiver, and social support all play important roles in the compliance of the child's family's health prescription., Registration: ChiCTR2200062022., Competing Interests: Conflict of interest: none declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Heart valve disease patients undergo multiple surgeries to replace structurally degraded valve prostheses, highlighting the need for valve replacements with growth and self-repair capacity. Given allogeneic valve transplantation's promise in meeting these goals by delivering a living valve replacement, we propose a framework for preserving and rehabilitating living valves ex vivo., Competing Interests: Declaration of interests E.M.C., G.V.-N., and D.M.K. are inventors on an international patent application describing methods for the ex vivo storage and preservation of living allogeneic valves., (Copyright © 2024 Elsevier Inc. All rights reserved.)

The 2024 Interamerican Society of Cardiology (SIAC) guidelines on cardiorespiratory rehabilitation (CRR) in pediatric patients with congenital heart disease aim to gather and evaluate all relevant evidence available on the topic to unify criteria and promote the implementation of CRR programs in this population in Latin America and other parts of the world. Currently, there is no unified CRR model for the pediatric population. Consequently, our goal was to create these CRR guidelines adapted to the characteristics of congenital heart disease and the physiology of this population, as well as to the realities of Latin America. These guidelines are designed to serve as a support for health care workers involved in the care of this patient group who wish to implement a CRR program in their workplace. The guidelines include an easily reproducible program model that can be implemented in any center. The members of this Task Force were selected by the SIAC on behalf of health care workers dedicated to the care of pediatric patients with congenital heart disease. To draft the document, the selected experts performed a thorough review of the published evidence., (Copyright © 2024 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Risk stratification is required to set an exercise prescription for cardiac rehabilitation, but an optimal scheme for congenital heart disease (CHD) is unknown. We piloted a system based on hemodynamic rather than anatomic factors: function, oxygen level, rhythm, complex/coronary anatomy, and elevated load (FORCE). Feasibility, efficacy, and safety of the FORCE tool were evaluated. Patients < 22 years old participating in the Cardiac Fitness Program at Boston Children's Hospital between 02/2017 and 12/2021 were retrospectively analyzed. Assigned FORCE levels, anatomy, adverse events, fitness and exercise test data were collected. Of 63 attempts at FORCE classification, 62 (98%) were successfully classified while one with restrictive cardiomyopathy was not. Thirty-nine (62%) were FORCE 1, 16 (25%) were FORCE 2, and seven (11%) were FORCE 3. Almost half of FORCE 1 patients had simple or complex CHD and the majority of FORCE 2 patients had single ventricle CHD. FORCE 3 patients were more likely to have serious arrhythmias or cardiomyopathy than those in FORCE 1 or 2 (p < 0.001). Postural orthostatic tachycardia syndrome patients appeared in FORCE 1 only. No adverse events occurred over 958 total sessions. The total number of fitness sessions/participant was similar across FORCE levels. It was feasible to risk stratify patients with CHD using a clinical FORCE tool. The tool was effective in categorizing patients and simple to use. No adverse events occurred with fitness training over nearly 1000 exercise training sessions. Adding diastolic dysfunction to the original model may add utility., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: The authors declare no conflicts of interest.

Exercise and physical activity (PA) have been shown to be effective, safe and feasible in both healthy children and children with congenital heart disease (CHD). However, implementing exercise training as an intervention is still not routine in children with CHD despite considerable evidence of health benefits and well-being. Understanding how children with CHD can safely participate in exercise can boost participation in PA and subsequently reduce inactivity-related diseases. Home-based exercise intervention, with the use of personal wearable activity trackers, and high-intensity interval training have been beneficial in adults' cardiac rehabilitation programmes. However, these remain underutilised in paediatric care. Therefore, the aims of this narrative review were to synthesise prescribed exercise interventions in children with CHD, identify possible limitation to exercise training prescription and provide an overview on how to best integrate exercise intervention effectively for this population into daily practice., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Aims: Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long-term survival is negatively affected by the absence of sub-pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O 2 peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease-specific reference V̇O 2 peak dataset., Methods and Results: A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O 2 peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter-quartile range) of 3.0 (2.0-5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O 2 peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O 2 peak decline correlated negatively with the trend of body mass index z-score (P = 0.006) and was faster in women than men (P = 0.008)., Conclusions: Total cavopulmonary connection patients have significantly reduced exercise capacity. The age-related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O 2 peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

In the current era, the majority of children born with congenital heart disease (CHD) will survive well into adulthood because of major advances in surgical techniques, as well as in critical and medical care. However, reoperation and palliative surgical interventions are increasingly common in the adults with CHD. Tools to risk stratify patients effectively and therapies to improve outcomes are required to optimize the management of adult patients with CHD during the preoperative and postoperative periods and beyond. Exercise testing is an invaluable tool to guide risk stratification. In addition, exercise training in patients with CHD may decrease postoperative complications by enhancing physiological reserve and also has an important role in physical rehabilitation. This review aims to provide individualized recommendations on exercise prescription in patients with CHD in the preoperative and postoperative settings. The response to exercise testing and prognostic implications is also discussed., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To improve health-related physical fitness (HRPF) (primary outcome) and health-related quality of life (HRQoL) with a web-based motor intervention program in pediatric patients with congenital heart disease (CHD)., Study Design: Overall, 70 patients (13.0 ± 2.6 years; 34% girls) aged 10-18 years with moderate or complex CHD severity were randomly allocated 1:1 to an intervention or control group. The intervention group trained 3 times per week for 20 minutes in a web-based exercise program over a period of 24 weeks. The control group followed lifestyle per usual. At baseline and follow-up HRPF was assessed via 5 tasks of the FITNESSGRAM and converted to a HRPF z score. HRQoL was assessed with KINDL self-report questionnaire., Results: In total, 61 patients completed the follow-up. There was no change in total HRPF z score (intervention group: 0.14 ± 0.38 vs control group: 0.09 ± 0.38, P = .560) and total HRQoL (intervention group: -1.73 ± 8.33 vs control group: 1.31 ± 7.85, P = .160) after the 24-week web-based exercise intervention. This was true for all subcategories of HRPF and HRQoL. There were no adverse events associated with the web-based exercise intervention., Conclusions: We found that 24 weeks of web-based exercise intervention with an aimed volume of 60 minutes of exercise per week was safe but did not improve HRPF and HRQoL in children with moderate or complex CHD., Trial Registration: Clinicaltrials.gov: NCT03488797., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: The main objective of this study was to ascertain if a structured intervention programme can improve the biophysical health of young children with congenital heart disease (CHD). The primary end point was an increase in measureable physical activity levels following the intervention., Methods: Patients aged 5-10 years with CHD were identified and invited to participate. Participants completed a baseline biophysical assessment, including a formal exercise stress test and daily activity monitoring using an accelerometer. Following randomisation, the intervention group attended a 1 day education session and received an individual written exercise plan to be continued over the 4-month intervention period. The control group continued with their usual level of care. After 4 months, all participants were reassessed in the same manner as at baseline., Results: One hundred and sixty-three participants (mean age 8.4 years) were recruited, 100 of whom were male (61.3%). At baseline, the majority of the children were active with good exercise tolerance. The cyanotic palliated subgroup participants, however, were found to have lower levels of daily activity and significantly limited peak exercise performance compared with the other subgroups. One hundred and fifty-two participants (93.2%) attended for reassessment. Following the intervention, there was a significant improvement in peak exercise capacity in the intervention group. There was also a trend towards increased daily activity levels., Conclusion: Overall physical activity levels are well preserved in the majority of young children with CHD. A structured intervention programme significantly increased peak exercise capacity and improved attitudes towards positive lifestyle changes., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Children and adolescents with congenital heart disease often do not have the opportunity, inclination, or education to participate in safe and effective exercise. The consequences of this behavioral pattern affect not only cardiopulmonary parameters, but also psychosocial factors, especially when lack of participation in peer activities or sports leads to isolation and further sedentary behaviors. Importantly, unlike cardiac rehabilitation programs for adults with atherosclerotic disease, the goal for congenital heart disease patients was less about "rehabilitation" and more about promotion of optimal fitness. We thus developed a comprehensive "Cardiac Fitness Program" at Boston Children's Hospital to promote exercise training, enhanced self-confidence, and motivation for patients with congenital heart disease. Since much of sustained fitness relates to consistency and behavior change, we crafted a progressive, goal-oriented exercise curriculum and augmented it with a self-learning workbook of targeted positive mindset practices to develop self-efficacy, an app for motivation and data collection, and exercise videos to demonstrate mechanics and to reiterate a positive message. We now report our experience including program structure and framework, navigating insurance, curriculum development, and outcome measures. Methods employed and barriers encountered in the initial development and execution of this program are reviewed. Key take-aways and further considerations including virtual and home-based programs are discussed.

Objective: To explore the effects of breast milk feeding and formula milk feeding on infants after cardiac surgery in the cardiac intensive care unit (ICU)., Methods: Infants who underwent cardiac surgery in our ICU were divided into two groups, according to feeding type. Breast milk feeding and formula milk feeding were separately implemented in the two groups, and the remaining treatment regimens were the same. The related clinical data and feeding effects were recorded and compared., Results: The prealbumin (147.3 ± 15.2 versus 121.5 ± 18.3mg/L) and albumin (46.4 ± 4.2 versus 40.5 ± 5.1 g/L) levels in the breast milk feeding group were better than those in the formula milk feeding group (P < .05). Infants in the breast milk feeding group achieved a better total enteral nutrition time (3.0 ± 1.2 versus 5.2 ± 2.1 d), average daily weight gain (19.0 ± 3.4 versus 14.4 ± 2.3 g/kg·d), length of ICU stay (6.0 ± 2.2 versus 8.1 ± 2.9 d) and length of hospital stay (13.9 ± 4.2 versus 17.8 ± 5.6 d) than those in the formula milk feeding group (P < .05). The incidence of complications such as feeding intolerance, anemia, dyspeptic diarrhea, and nosocomial infection was lower in the breast milk feeding group than in the formula milk feeding group (P < 0.05)., Conclusion: Breast milk feeding has a definite nutritional effect on infants after cardiac surgery. It is better than formula milk feeding, making it worthy of popularization and application.

Background: Congenital heart disease (ConHD) affects approximately 1% of all live births. People with ConHD are living longer due to improved medical intervention and are at risk of developing non-communicable diseases. Cardiorespiratory fitness (CRF) is reduced in people with ConHD, who deteriorate faster compared to healthy people. CRF is known to be prognostic of future mortality and morbidity: it is therefore important to assess the evidence base on physical activity interventions in this population to inform decision making., Objectives: To assess the effectiveness and safety of all types of physical activity interventions versus standard care in individuals with congenital heart disease., Search Methods: We undertook a systematic search on 23 September 2019 of the following databases: CENTRAL, MEDLINE, Embase, CINAHL, AMED, BIOSIS Citation Index, Web of Science Core Collection, LILACS and DARE. We also searched ClinicalTrials.gov and we reviewed the reference lists of relevant systematic reviews., Selection Criteria: We included randomised controlled trials (RCT) that compared any type of physical activity intervention against a 'no physical activity' (usual care) control. We included all individuals with a diagnosis of congenital heart disease, regardless of age or previous medical interventions.  DATA COLLECTION AND ANALYSIS: Two review authors (CAW and CW) independently screened all the identified references for inclusion. We retrieved and read all full papers; and we contacted study authors if we needed any further information. The same two independent reviewers who extracted the data then processed the included papers, assessed their risk of bias using RoB 2 and assessed the certainty of the evidence using the GRADE approach. The primary outcomes were: maximal cardiorespiratory fitness (CRF) assessed by peak oxygen consumption; health-related quality of life (HRQoL) determined by a validated questionnaire; and device-worn 'objective' measures of physical activity., Main Results: We included 15 RCTs with 924 participants in the review. The median intervention length/follow-up length was 12 weeks (12 to 26 interquartile range (IQR)). There were five RCTs of children and adolescents (n = 500) and 10 adult RCTs (n = 424). We identified three types of intervention: physical activity promotion; exercise training; and inspiratory muscle training. We assessed the risk of bias of results for CRF as either being of some concern (n = 12) or at a high risk of bias (n = 2), due to a failure to blind intervention staff. One study did not report this outcome. Using the GRADE method, we assessed the certainty of evidence as moderate to very low across measured outcomes. When we pooled all types of interventions (physical activity promotion, exercise training and inspiratory muscle training), compared to a 'no exercise' control CRF may slightly increase, with a mean difference (MD) of 1.89 mL/kg -1 /min -1 (95% CI -0.22 to 3.99; n = 732; moderate-certainty evidence). The evidence is very uncertain about the effect of physical activity and exercise interventions on HRQoL. There was a standardised mean difference (SMD) of 0.76 (95% CI -0.13 to 1.65; n = 163; very low certainty evidence) in HRQoL. However, we could pool only three studies in a meta-analysis, due to different ways of reporting. Only one study out of eight showed a positive effect on HRQoL. There may be a small improvement in mean daily physical activity (PA) (SMD 0.38, 95% CI -0.15 to 0.92; n = 328; low-certainty evidence), which equates to approximately an additional 10 minutes of physical activity daily (95% CI -2.50 to 22.20). Physical activity and exercise interventions likely result in an increase in submaximal cardiorespiratory fitness (MD 2.05, 95% CI 0.05 to 4.05; n = 179; moderate-certainty evidence). Physical activity and exercise interventions likely increase muscular strength (MD 17.13, 95% CI 3.45 to 30.81; n = 18; moderate-certainty evidence). Eleven studies (n = 501) reported on the outcome of adverse events (73% of total studies). Of the 11 studies, six studies reported zero adverse events. Five studies reported a total of 11 adverse events; 36% of adverse events were cardiac related (n = 4); there were, however, no serious adverse events related to the interventions or reported fatalities (moderate-certainty evidence). No studies reported hospital admissions., Authors' Conclusions: This review summarises the latest evidence on CRF, HRQoL and PA. Although there were only small improvements in CRF and PA, and small to no improvements in HRQoL, there were no reported serious adverse events related to the interventions. Although these data are promising, there is currently insufficient evidence to definitively determine the impact of physical activity interventions in ConHD. Further high-quality randomised controlled trials are therefore needed, utilising a longer duration of follow-up., (Copyright © 2020 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

Low levels of habitual physical activity in children and adolescents with congenital heart disease (CHD) leads to various negative health outcomes, yet review of the literature indicates that many physicians, caregivers, and patients restrict physical activity owing to safety concerns. There is no evidence supporting absolute restriction of physical activity in pediatric patients with CHD; in fact, physically active lifestyles are as important for pediatric patients living with CHD as for the general population. To encourage long-term maintenance of physically active lifestyles, physical activity counselling and exercise prescription should be started early in childhood and be a core component of every patient encounter. Physical activity counselling should include clear messaging from physicians about recommended physical activities-not just restrictions-and a personalised written exercise prescription for the patient and family. Regular follow-up is essential to ensure adherence to recommendations, monitor patient responses to the increased physical activity level, and long-term surveillance. This review discusses the importance of a physically active lifestyle in children and adolescents with and without surgically repaired CHD and hypertrophic cardiomyopathy, excluding those with electrical abnormalities and channelopathies. It outlines gaps in knowledge regarding best practices for physical activity promotion in these patient populations, and provides recommendations on how to include physical activity promotion and exercise prescription in clinical practice based on existing literature. An important role for clinicians is identified, because the type of messaging they provide regarding physical activity will have a major impact on patients' and families' decisions to adopt an active lifestyle., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Children with congenital heart disease (CHD) are at risk for both COVID-19 and secondary cardiovascular outcomes. Their increased cardiovascular risk may be mitigated through physical activity, but public health measures implemented for COVID-19 can make physical activity challenging. We objectively measured the impact of the COVID-19 pandemic on physical activity, continuously measured by Fitbit step counts, in children with CHD. Step counts were markedly lower in late March and early April 2020, compared with 2019 and early March 2020. It is vital to understand how precautions for COVID-19 will affect the health of children with CHD, especially if they persist long term., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Critical surgical and medical advances have shifted the focus of congenital heart disease (CHD) patients from survival to achievement of a greater health-related quality of life (HRQoL). HRQoL is influenced, amongst other factors, by aerobic capacity and respiratory muscle strength, both of which are reduced in CHD patients. This study evaluates the influence of a cardiopulmonary rehabilitation program (CPRP) on respiratory muscle strength and functional capacity. Fifteen CHD patients, ages 12 to 16, with reduced aerobic capacity in cardiopulmonary exercise testing (CPET) were enrolled in a CPRP involving strength and aerobic training for three months. Measurements for comparison were obtained at the start, end, and six months after the CPRP. A significant improvement of inspiratory muscle strength was evidenced (maximum inspiratory pressure 21 cm H 2 O, 23%, p < 0.01). The six-minute walking test showed a statistically and clinically significant rise in walked distance (48 m, p < 0.01) and a reduction in muscle fatigue (1.7 out of 10 points, p = 0.017). These results suggest CPRP could potentially improve respiratory muscle function and functional capacity, with lasting results, in children with congenital heart disease, but additional clinical trials must be conducted to confirm this finding.

A strict and rational approach to Fallot's tetralogy with pulmonary atresia and major aortopulmonary collateral arteries allows to achieve optimal results. Rehabilitative and unifocalization strategies do not constitute separate philosophies; instead the surgical strategy should be tailored to each individual patient. Based on our previous experience, the ability to achieve definitive intracardiac repair is the real determinant of both improved survival and adequate systolic right ventricular performance on mid-term follow-up.

Purpose: Cardiac rehabilitation (CR) effectively decreases morbidity and mortality in adults after cardiovascular events. Cardiac rehabilitation has been underutilized for patients with congenital heart disease (CHD). The primary objective was to evaluate the inclusion of adolescents and adults with CHD in a CR program by analyzing data from our single-center CR program. The secondary objectives were to evaluate the efficacy and safety of CR as well as referral barriers., Methods: This was a retrospective study of patients aged ≥15 yr who were referred to regional CR centers. Data on efficacy and safety were collected., Results: Over a 4-yr period, 36 patients were referred to 23 regional centers: 23 patients completed CR, 12 are currently enrolled or in the referral process, and 1 died before initiation. The median age was 22 yr (range: 15-55). The primary indication was post-surgical (61%), followed by chronic heart failure (30%), and post-transplant (9%). After CR, metabolic equivalent tasks increased by 1.6 (P < .001), maximal heart rate increased by 13 beats/min (P = .026), exercise time increased by 1.35 min (P = .047), and treadmill speed increased by 0.7 mph (P = .007). There were no serious adverse events. All patients who completed CR remain alive at a median follow-up of 17 mo (range: 5-45). Common barriers to CR included accessibility, social circumstances, and cost for phase III CR., Conclusion: In our cohort, CR was effective and safe for adolescents and adults with CHD.

Cardiac rehabilitation is an important part of daily routine for cardiac disorder patients in adults. However, pediatric rehabilitation is an emerging field, and is totally different and new field in case of pediatric patients. The main reason of variability is the Pediatric patients differ from adult patients in several ways. The main difference is they are dependent on their parents for meeting their needs, including for transportation and following of rehabilitation initiatives. Furthermore, rehabilitation initiatives are often connected to large urban university hospitals and unavailable to children whose parents cannot bring them for exercise training on a regular basis. The present review article is focused on these aspects of rehabilitation during congenital heart disease.

Objectives: To examine the strengths and opportunities for improvement of current home care education practices to inform the development of the Home Care for Heart Health intervention, and to develop a web-based intervention for parents and clinicians with complimentary print materials that could provide the right education at the right time to foster a safer transition from hospital to home., Methods: An inter-professional focus group of parents, clinicians, and designers was formed to co-create a home care education intervention for parents of children with congenital heart disease (CHD) and their care team. We used the Integrated New Product Development process model created by Jonathon Cagan and Craig Vogel at Carnegie Mellon University to develop the intervention. This process model is a way of thinking that combines horizontal and inter-disciplinary teams, stakeholder-centric focus, and a system of qualitative discovery and development evolving towards quantitative methods of refinement., Results: Our team developed the Home Care for Heart Health intervention. The evidenced-based intervention includes a quick reference guide for parents of children with CHD, an accompanying app, family-friendly pathways, and clinician education., Conclusion: Using an inter-professional approach, our team of clinicians, parents, and design experts were able to co-create a clinician-parent home care education intervention with broad application and lifelong relevance to the Congenital Heart Disease Community., Practice Implications: Our intervention has the potential to be used as a model for other home care education interventions for parents of children with chronic illnesses.

Objective: To study physical activity and sleep in Fontan patients and healthy controls before and after an endurance training program, and after 1 year., Method: Fontan patients (n = 30) and healthy controls (n = 25) wore accelerometers for seven consecutive days and nights during a school week before and after a 12-week endurance training program and after 1 year., Results: Patients had similar sleep duration and sleep efficiency as healthy controls. Latency to sleep onset in minutes was longer for patients than controls (22.4 (4.3-55.3) minutes versus 14.8 (8.6-29.4) minutes, p < 0.01). More time in moderate-to-vigorous activity daytime was correlated with increased sleep time (p < 0.05; r2 = 0.20), improved sleep efficiency (p < 0.01; r2 = 0.24) and less time as wake after sleep onset (p < 0.05; r2 = 0.21) for patients but not controls. Sleep variables did not change after the exercise intervention for patients or controls. After 1 year, patients had decreased total sleep time, decreased sleep efficiency, increased accelerometer counts during sleep and more time as wake after sleep onset during sleep time, but not controls., Conclusions: Fontan patients have prolonged latency to sleep onset compared with controls. More time in physical activities was correlated with better sleep quality for the patients. Also, subjects with low sleep efficiency and long latency to sleep onset may benefit most from physical exercise. These patients should be encouraged to engage in individually designed physical exercise as this could improve sleep quality.

Background: Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods., Methods: The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2 max ) < 80% and/or a ventilatory anaerobic threshold (VAT) < 55% of predicted VO2 max , will be eligible. Patients will be randomised into 2 groups (12-week cardiac rehabilitation program vs. controls). The primary outcome is the change in the PedsQL quality of life score between baseline and 12-month follow-up. A total of 130 patients are required to observe a significant increase of 7 ± 13.5 points in the PedsQL, with a power of 80% and an alpha risk of 5%. The secondary outcomes are: VO2 max , VAT, stroke volume, clinical outcomes, physical and psychological status, safety and acceptability., Conclusion: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD., Trial Registration: Clinicaltrials.gov (NCT03690518)., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Inconsistencies have been reported in health-related quality of life (HRQOL) in postoperative congenital heart disease (CHD). Despite the need for lifelong care due for residual symptoms, only a few studies have explored cardiac-related HRQOL but none in lower middle-income countries (LMIC). This study therefore addresses the gap by exploring HRQOL and its associated predictors in postoperative CHD in Pakistan., Outcome Measures: General and cardiac-related HRQOL, associated predictors., Methods: This cross-sectional study recruited patients with CHD and age-matched healthy siblings as controls (n=129 each) at a single centre in Pakistan. Patients and their siblings completed HQROL surveys (PedsQL 4.0 Generic Core, PedsQL Cognitive Functioning). Patients only completed PedsQL 3.0 Cardiac module. Generalised linear models identified predictors., Results: The sample mean age was 8.84±3.87 years and 70% were below the poverty line for an LMIC. The majority (68%) had their first surgery after 1 year of age and were interviewed at a mean 4.08±1.91 years postoperatively.Patients with CHD had lower HRQOL in all domains compared with their age-matched siblings, with the biggest differences for total HRQOL (effect size, d=-1.35). Patients with complex CHD had lower HRQOL compared with simple to moderate CHDs in cardiac-related HRQOL. The lowest scores were for treatment problems (effect size, d=-0.91). HRQOL was worse for patients who were on cardiac medications, had complex CHD, longer cardiopulmonary bypass time, re-operations and were female., Conclusions: HRQOL issues persist in postoperative patients with CHD in LMIC, Pakistan. Solutions are needed to address poor HRQOL and lifelong concerns of patients and their parents., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The respiratory benefits of muscle strength are well-known in heart-healthy populations, but recommendations and research often focus instead on aerobic fitness (peak oxygen uptake) or total activity. Independent benefits of strength thus may be underestimated, especially in congenital heart disease where perceived dangers of certain types of exercise may outweigh perceived benefits. To assess whether it is plausible that pulmonary benefits of strength in heart-healthy populations also apply in congenital heart disease, we simultaneously correlated these patients' lung function with fitness, strength, and cardiac diagnosis., Methods: Lung function (forced expiratory volume in one second percentage predicted (FEV1%pred)) was modeled as function of handgrip strength, congenital heart disease diagnosis, peak oxygen uptake and the interactions of handgrip with sex and diagnosis in 538 Germans (58% male, ages 6-82 years) in linear models corrected for age, sex, height and weight. Congenital heart disease diagnoses were: complex cyanotic; Fallot/Truncus arteriosus communis (common arterial trunk) (TAC); shunts; transposition of the great arteries (TGA); left heart; and other/none., Results: Each kg of handgrip was associated with 0.74% higher FEV1%pred ( p < 0.001) and handgrip explained almost 10% of variance in FEV1%pred. While some groups had higher FEV1%pred than others ( p for global null <0.0001), all experienced similar associations with strength ( p for interaction with handgrip >0.10 for both sex and diagnosis.) Correction for peak oxygen uptake eliminated the association with congenital heart disease, but not handgrip., Conclusion: Strength was associated with better lung function in all ages even after correction for peak oxygen uptake, regardless of sex and congenital heart disease. This suggests that strength may be at least as important for lung function as aerobic fitness. Heart-safe strength training may improve pulmonary function in congenital heart disease.

Aim: The preoperative poor nutrition of children with congenital heart disease (CHD) impacts the postoperative rehabilitation process of pediatric CHD cases. The factors of these children's preoperative poor nutrition, excluding the disease, have been underreported. The aim was to investigate the preoperative nutritional status of children with CHD who required a simple surgical repair and to analyze the maternal characteristics that are associated with poor nutrition in these sick children., Methods: This was a cross-sectional survey. The weight and height of the children were measured, maternal data were collected via a questionnaire and a univariate analysis and multivariate logistic regression were used to analyze the association between maternal factors and the preoperative poor nutrition of the children with CHD., Results: A total of 119 children with simple CHD were recruited to the study. The prevalence of poor nutrition was higher in the children with CHD ("cases") than in the healthy children ("controls"). An increased risk of poor nutrition was associated with lower mothers' perception, education level, understanding of the disease, and higher anxiety., Conclusions: Paying attention to maternal anxiety, depression, and knowledge and providing interventions for the mothers of children with CHD are important in order to promote the nutritional status of these children., (© 2018 Japan Academy of Nursing Science.)

Objective: Exercise training is recommended for its possible favorable effects on Fontan pathophysiology. This study aimed to elucidate the impact of pediatric exercise capacity trajectory, which may mimic the effect of exercise training, on late adult Fontan pathophysiology., Methods: Since 1990, 97 Fontan patients had consecutively undergone two serial cardiopulmonary exercise tests (CPX1 and CPX2) during childhood (ages 8 ± 2 and 14 ± 2 years) and one during adulthood (CPX3; age 23 ± 5 years). The changes in peak oxygen uptake (PVO 2 : % of normal value) from CPX1 to CPX2 (1-dPVO 2 ) and from CPX2 to CPX3 (2-dPVO 2 ) were calculated, and then the patients were divided into four subgroups according the 1-dPVO 2 and 2-dPVO 2 ., Results: In their adulthood, when compared with groups with negative 1-dPVO 2 , the central venous pressure, plasma brain natriuretic peptide level, and renal resistive index were lower, whereas liver synthetic function, body fat-free percentage, and PVO 2 were higher in those with positive 1-dPVO 2 (p < 0.05-0.0001). However, these favorable associations of 2-d-PVO 2 with adult Fontan pathophysiology were not observed, except for the PVO 2 . After CPX3, 13 unexpected events occurred, and the risk was 76% lower in the groups having positive 1-dPVO 2 than in those with negative 1-dPVO 2 (hazard ratio, 0.24; 95% confidence interval, 0.09-0.62; p = 0.0035)., Conclusions: A positive exercise capacity trajectory during childhood predicts better adult Fontan pathophysiology, including better prognosis. Thus, prescription of exercise could be a promising strategy in the management of pediatric Fontan patients., (Copyright © 2018 Elsevier B.V. All rights reserved.)

The great advances in the medical and surgical management of congenital heart diseases have allowed many children to reach adulthood with often a good hemodynamic result. Nevertheless most of these adults have a limitation of their functional capacity. This limitation is more or less important, penalizes them in their daily life and alters their quality of life. The origin of this limitation is generally multifactorial. It is linked, of course, to the severity of the heart disease and the quality of the operative result. But there is very often a physical deconditioning. It can be secondary to the heart disease but is often secondary to a lack of physical activity. It is the parents, sometimes overprotective, but frequently the doctors who imposed, often wrongly, this restriction. It is essential to take this dimension into account in view of the important benefits expected for health and quality of life. Cardiac rehabilitation is a privileged tool for providing advice in a suitable environment. This requires close collaboration between cardiac rehabilitators and congenital cardiologists to offer appropriate care. We bring here some reflections and the basic elements to guide the re-training of these patients., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Objective: We aimed to study the efficiency and safety of once-a-week outpatient rehabilitation followed by home program with tele-monitoring in patients with complex cyanotic congenital heart disease., Design: Prospective nonrandomized study., Method: Patients who have been diagnosed either Eisenmenger's syndrome or inoperable complex cyanotic heart disease and able to attend 12-week cardiac rehabilitation program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once-a-week in the first 6 weeks followed by home-based exercise program (bicycle and walking) with a target at 40%-70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO 2 ), oxygen pulse (O 2 pulse), ventilatory equivalent for carbon dioxide (VE/CO 2 at anaerobic threshold), constant work-rate endurance time (CWRET) at 75% of peak VO 2 , and 6-minute walk distance (6MWD) were compared between baseline and after training by paired t test., Result: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respectively). The mean difference of 6MWD change in the home-program group was significantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training., Conclusion: Once-a-week outpatient hospital-based exercise program followed by supervised home-based exercise program showed a significant benefit in improvement of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes., (© 2018 Wiley Periodicals, Inc.)

Background: Exercise training has been shown to increase exercise capacity in survivors of Fontan surgery. The geographic distribution of the Fontan population has been a barrier to hospital-based exercise training programmes. The objective of this study was to establish whether a home exercise training programme could achieve similar improvements to a hospital programme., Methods: Adolescents with a Fontan circulation aged 12-19 years were prospectively recruited in a hospital or home exercise training programme. Patients underwent cardiopulmonary exercise testing and completed the Paediatric Quality of Life Inventory at initial assessment and after completion of an 8-week programme. Both groups performed two 1-hour training sessions per week. Patients in the home training programme had their first session in the hospital, and then progressed independently with one phone consult per week and one home visit by a physiotherapist., Results: In total, 17 patients, with a mean age of 15±3 years, completed the training programme (six hospital). Characteristics and baseline performance of patients were similar in both groups. Oxygen consumption at anaerobic threshold increased from 19.3±3.8 to 21.6±6.0 ml/kg/minute (p=0.02) and peak oxygen pulse increased from 8.8±2.5 to 9.5±2.7 ml/beat (p=0.049). Total quality of life scale improved from 68 to 74% (p=0.01) and psychosocial health improved from 67 to 74% (p=0.02). No patient experienced training-related complications., Conclusions: Exercise training is beneficial and most likely safe after Fontan, resulting in improved exercise capacity and self-reported quality of life. Home exercise training programmes are probably as effective as hospital programmes. Home exercise training programmes should be integrated in the follow-up care of patients undergoing Fontan surgery.

Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To assess a motivational interviewing (MI) intervention to improve moderate-to-vigorous physical activity (MVPA) in adolescents with congenital heart disease., Methods: Intervention participants received one-on-one telephone-based adapted MI sessions over 3 months. Outcomes were acceptability, change mechanisms (stage of change and self-efficacy), and limited efficacy (physical activity, fitness, and quality of life)., Results: Thirty-six participants were randomized. Intervention participants completed 4.2 ± 1.2/6 MI sessions, with no improvements in the high self-efficacy or stage of change observed. Participants accumulated 47.24 ± 16.36 minutes of MVPA/day, and had comparable outcomes to peers without heart disease (except for functional capacity). There was no significant difference in change in any outcome by group., Conclusions: The intervention was acceptable, but effectiveness could not be determined due to the nature and size of sample., Clinical Relevance: Pediatric cardiac rehabilitation remains the sole effective intervention to increase MVPA in this population.

Owing to significant medical advances, it is now estimated that more than 90% of persons born with congenital heart disease (CHD) will reach adulthood. Medically appropriate physical activity represents an opportunity to improve physical functioning as well as quality of life and psychosocial outcomes. By reviewing published CHD research and clinical recommendations, herein we first summarize how adults with CHD are known to be less physically active and have reduced exercise capacity compared with healthy peers. Cardiopulmonary exercise testing is important for routine clinical management and before the onset of an exercise program. Physiological anomalies are common in adults with CHD, although very few necessitate activity restrictions, and positive results from exercise training have been demonstrated. In recent decades, the focus has thus shifted from restriction of exercise to promotion of exercise. Adults with CHD also face unique psychosocial challenges associated with living with a chronic cardiac condition, many of which may influence exercise behaviours. However, much less is known about participation of adults with CHD in cardiac rehabilitation (CR) programs, which differ from exercise training in their comprehensive, interdisciplinary approach to management of chronic disease and that might be uniquely poised to meet the physical and psychosocial needs of adults with CHD. Initial CR outcomes have been positive and with no reported adverse events. This review summarizes the unique physical and psychosocial considerations that may guide the provision of CR to adults with CHD., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Due to medical advances, growing numbers of adolescents with congenital heart disease (CHD) survive into adulthood and transferring from paediatric to adult healthcare. This transfer is significant step in a young person's life, and this study examines the views of Irish healthcare professionals' on how best to manage this transition., Methods: Purposeful sampling was used to invite participation by healthcare professionals (HCPs) from a variety of disciplines whose caseloads include adolescents and young adults with CHD. Fourteen professionals participated in semistructured interviews regarding their experiences of the transition process and their recommendations. Data were collected during Spring 2016 and analysed using thematic analysis., Results: Results indicated that the current approach to transition and transfer could be improved. Professionals identified barriers hindering the transition process such as cultural and attitudinal differences between HCPs dealing with child and adult patients, inadequate preparation and education of patients about their condition, parental reluctance to transfer, and concern about parents' role in on-going treatment. Measures such as better support and education for both the patients and their parents were recommended, in order to facilitate a smoother transition process for all parties involved. Additionally, HCPs identified the need for better collaboration and communication, both between paediatric and adult healthcare professionals and between hospitals, to ensure greater continuity of care for patients., Conclusions: Action is required in order to improve the current transition process. Measures need to be taken to address the barriers that currently prevent a smooth transition process for young adult CHD patients. Professionals recommended the implementation of a structured transition clinic to deal with the wide variety of needs of transitioning adolescent patients and their families. Recommendations for future research are also made., (© 2018 John Wiley & Sons Ltd.)

The absence of a subpulmonary ventricle in the Fontan circulation results in non-pulsatile pulmonary blood flow. Lower limb exercise in this setting can generate pulsatile pulmonary blood flow.

Background: Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD)., Methods: We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O 2 saturation ≥92%, and had peak O 2 consumption ([Formula: see text]) < 80% predicted. We assessed exercise capacity, physical activity, quality of life, self-reported health status, and other variables at baseline and after 12 weeks. The prespecified primary end point was change in [Formula: see text]., Results: We analyzed data on 28 participants (aged 41.1 ± 12.1 years, 50% male), 13 randomized to CR and 15 to SOC. [Formula: see text] averaged 16.8 ± 3.8 mL/kg/min, peak work rate = 95 ± 28 W, and median Minnesota Living with Heart Failure Questionnaire (MLHFQ) score = 27 (interquartile range: 11-44). Cardiac rehabilitation participants were older (48 ± 9 years vs 36 ± 12 years; P = .01), but there were no significant between-group differences in other variables. There were no adverse events related to CR. [Formula: see text] increased in the CR group compared with SOC (+2.2 mL/kg/min, 95% confidence interval: 0.7-3.7; P = .002, age-adjusted +2.7 mL/kg/min; P = .004); there was a nonsignificant improvement in work rate (+8.1 W; P = .13). Among the 25 participants with baseline MLHFQ > 5, there was a clinically important >5-point improvement in 72.7% and 28.6% of CR and SOC participants, respectively ( P = .047). Cardiac rehabilitation was also associated with improved self-assessment of overall health ( P < .04)., Conclusions: Cardiac rehabilitation is safe and is associated with improvement in aerobic capacity and self-reported health status compared with SOC in adults with CHD.

Objective: The beneficial effects of exercise training in acquired heart failure and coronary artery disease are well known and have been implemented in current treatment guidelines. Knowledge on appropriate exercise training regimes for adults with congenital heart disease is limited, thus further studies are needed. The aim of this study was to examine the effect of home-based interval exercise training on maximal endurance capacity and peak exercise capacity., Design: Randomized controlled trial., Methods: Twenty-six adults with complex congenital heart disease were recruited from specialized units for adult congenital heart disease. Patients were randomized to either an intervention group-12 weeks of home-based interval exercise training on a cycle ergometer (n = 16), or a control group (n = 10). The latter was instructed to maintain their habitual physical activities. An incremental cardiopulmonary exercise test and a constant work rate cardiopulmonary exercise test at 75% of peak workload were performed preintervention and postintervention., Results: Twenty-three patients completed the protocol and were followed (intervention n = 13, control n = 10). Postintervention exercise time at constant work rate cardiopulmonary exercise test increased in the intervention group compared to controls (median[range] 12[-4 to 52]min vs 0[-4 to 5]min, P = .001). At incremental cardiopulmonary exercise test, peak VO 2 increased 15% within the intervention group (P = .019) compared to 2% within the control group (P = .8). However, in comparison between the groups no difference was found (285[-200 to 535] ml/min vs 17[-380 to 306] ml/min, P = .10). In addition, peak workload at incremental cardiopulmonary exercise test increased in the intervention group compared to controls (20[-10 to 70]W vs 0[-20 to 15]W, P = .003)., Conclusion: Home-based interval exercise training increased endurance capacity and peak exercise capacity in adults with complex congenital heart disease. Aerobic endurance might be more relevant than peak oxygen uptake with regard to daily activities, and therefore a more clinically relevant measure to evaluate., (© 2017 Wiley Periodicals, Inc.)

A 32-year-old man born with double inlet left ventricle (DILV) and other significant cardiac abnormalities underwent surgical palliation at 1 day, 2 years and 20 years, before receiving a donor heart at 29 years. To our knowledge, there are no case reports or cohort studies of the effect of exercise training on exercise capacity and peak oxygen uptake (VO 2 peak) following heart transplantation (HTx) for individuals born with DILV. The patient accessed our clinical exercise physiology service for assessment, advice and support for exercise training over a 7-year period spanning pre-HTx and post-HTx. An individualised exercise plan, together with careful assessment and monitoring, and the patient's own motivation have contributed to him achieving an outstanding post-HTx doubling of VO 2 peak and exercise capacity., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objective: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease., Design, Setting, and Patients: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively., Intervention and Outcome Measures: Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer., Results: Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P = .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P = .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted., Conclusions: Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes., (© 2017 Wiley Periodicals, Inc.)

Objective: Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease., Design: Retrospective review of a prospective registry., Setting: United Network for Organ Sharing registry of transplant recipients in the United States., Patients: Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015., Interventions: None., Outcome Measures: Employment status reported by transplant centers at required follow-up intervals up to 5 y posttransplant., Results: Among 470 patients included in the analysis (mean follow-up: 5 ± 3 y), 127 (27%) worked after transplant, 69 (15%) died before beginning or returning to work, and 274 (58%) survived until censoring, but did not participate in paid work. Multivariable competing-risks regression analysis examined characteristics associated with posttransplant employment, accounting for mortality as a competing outcome. In descriptive and multivariable analysis, pretransplant work participation was associated with a greater likelihood of posttransplant employment, while the use of Medicaid insurance at the time of transplant was associated with a significantly lower likelihood of working after transplant (subhazard ratio compared to private insurance: 0.55; 95% confidence interval: 0.32, 0.95; P = .032)., Conclusions: Employment was rare after heart transplantation for congenital heart disease, and was significantly less common than in the broader population of adults with congenital heart disease. Differences in return to work were primarily related to pretransplant employment and the use of public insurance, rather than clinical characteristics., (© 2017 Wiley Periodicals, Inc.)

Aim: This historical cohort study describes the use of educational and rehabilitation services in adolescents born preterm or with a congenital heart defect (CHD)., Method: Parents of 76 young people (mean age 15y 8mo [SD 1y 8mo]) with CHD and 125 born ≤29 weeks gestational age (mean age 16y [SD 2y 5mo]) completed a demographics questionnaire including educational and rehabilitation resource utilization within the previous 6 months. Rehabilitation services included occupational therapy, physical therapy, speech language pathology, psychology. Developmental (Leiter Brief IQ, Movement-ABC, Strengths and Difficulties Questionnaire) and functional (Vineland) status of the young people was assessed. Pearson χ 2 tests were used to perform simple pairwise comparisons of categorical outcomes across the two groups (CHD, preterm). Univariate logistic regression was used to examine predictors of service utilization., Results: Developmental profiles of the two groups (CHD/preterm) were similar (29.9%/30% IQ<80; 43.5%/50.0% motor difficulties; 23.7%/22.9% behavior problems). One-third received educational supports or attended segregated schools. Only 16% (preterm) and 26.7% (CHD) were receiving rehabilitation services. Services were provided predominantly in the school setting, typically weekly. Few received occupational therapy or physical therapy (1.3-7.6%) despite functional limitations. Leiter Brief IQ<70 was associated with receiving educational supports (CHD: OR 5.53, 95% CI 1.29-23.68; preterm: OR 14.63, 3.10-69.08) and rehabilitation services (CHD: OR 4.46, 1.06-18.88; preterm: OR 5.11, 1.41-18.49). Young people with motor deficits were more likely to require educational (CHD: OR 5.72, 1.99-16.42; preterm: OR 3.11, 1.43-6.77) and rehabilitation services (preterm: OR 3.97, 1.21-13.03)., Interpretation: Although young people with impairments were more likely to receive educational and rehabilitation services, many may not be adequately supported, particularly by rehabilitation specialists. Rehabilitation services at this important transition phase could be beneficial in optimizing adaptive functioning in the home, school, and community., (© 2017 Mac Keith Press.)

Objective: The aim of this study was to evaluate the safety and feasibility of a passive range of motion exercise programme for infants with CHD. Study design This non-randomised pilot study enrolled 20 neonates following Stage I palliation for single-ventricle physiology. Trained physical therapists administered standardised 15-20-minute passive range of motion protocol, for up to 21 days or until hospital discharge. Safety assessments included vital signs measured before, during, and after the exercise as well as adverse events recorded through the pre-Stage II follow-up. Feasibility was determined by the percent of days that >75% of the passive range of motion protocol was completed., Results: A total of 20 infants were enrolled (70% males) for the present study. The median age at enrolment was 8 days (with a range from 5 to 23), with a median start of intervention at postoperative day 4 (with a range from 2 to 12). The median hospital length of stay following surgery was 15 days (with a range from 9 to 131), with an average of 13.4 (with a range from 3 to 21) in-hospital days per patient. Completion of >75% of the protocol was achieved on 88% of eligible days. Of 11 adverse events reported in six patients, 10 were expected with one determined to be possibly related to the study intervention. There were no clinically significant changes in vital signs. At pre-Stage II follow-up, weight-for-age z-score (-0.84±1.20) and length-for-age z-score (-0.83±1.31) were higher compared with historical controls from two earlier trials., Conclusion: A passive range of motion exercise programme is safe and feasible in infants with single-ventricle physiology. Larger studies are needed to determine the optimal duration of passive range of motion and its effect on somatic growth.

More than 90% of adolescents and young adults with chronic medical conditions will survive into adulthood. Transitioning from pediatric to adult health care services for these individuals has often times been associated with deterioration of their health and Quality of Life. Separation from their pediatric provider and lack of preparedness of the adult health care system has been identified as major barriers in preventing the successful transition of these individuals. The purpose of this review is to summarize the available data related to transitioning adolescents and young adults (AYA) with special health care needs into the adult health care system., (Copyright © 2017 Mosby, Inc. All rights reserved.)

Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5-8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group (n = 14) and a non-PLE/PB group (n = 92). Prevalence of PLE was 9.4% (n = 10) and of PB 3.8% (n = 4). At follow-up, five patients (4.7%) died of PLE or PB. Median age at death was 6.2 years (IQR 10.5, 95% CI 5.3-23.4). We observed no significant group differences in gender distribution (p = 0.73), ventricular morphology (p = 0.87), surgical technique (p = 0.64), conduit fenestration (p = 0.34), age at Fontan operation (p = 0.54), and need for diuretics (p = 0.56). Hypoplastic left heart syndrome was more frequent in the PLE/PB group 50 vs. 22.8% (p = 0.03) OR 3.4 (95% CI 1.1-10.8). The modified Glenn procedure was performed at a median age of 4 months (IQR 4.0) in the PLE/PB group versus 8 months (IQR 8.0) in the non-PLE/PB group (p = 0.01). The early Glenn procedure and hypoplastic left heart syndrome may be associated with the development of PLE and PB.

Background: Physical activity and exercise have important health benefits for children and adolescents with CHD. The objective of this study was to survey the provision of advice and recommendations in United Kingdom paediatric CHD clinics., Methods: A three-page questionnaire was sent out to paediatric cardiac consultants in the United Kingdom, paediatric consultants with expertise in cardiology, and nursing staff (Paediatricians with Expertise in Cardiology Special Interest Group), as well as all members of the British Congenital Cardiovascular Association. The aim of this questionnaire was to determine the extent and scope of current information provision and to assess the importance that clinicians place on this advice., Results: There were 68 responses in total, and the data showed that, of these, 24 (36%) clinicians had never provided paediatric CHD patients with written advice about exercise. Only 27 (39%) clinicians provided physical activity advice at every appointment. Lack of time during consultation (n=39, 56.9%), lack of training (n=38, 55.2%), and uncertainty about appropriate recommendations (n=38, 55.2%) were identified as the main factors preventing clinicians from providing patients with advice about physical activity., Conclusion: Although healthcare providers consider physical activity to be very important, the provision of clear, specific advice and recommendations is underutilised; therefore, more education and provision of resources to support the promotion of exercise need to be provided to clinicians and their support teams.

Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries, and new issues are emerging: the evaluation of their capacity to cope with physical activity and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool, because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, and/or pulmonary hypertension), and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak [Formula: see text]o 2 , an early anaerobic threshold, a blunted heart rate response, a reduced increase of Vt, and an increased [Formula: see text]e/[Formula: see text]co 2 . All these measures suggest common pathophysiological abnormalities: (1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs, or muscles; (2) chronotropic incompetence secondary to cardiac autonomic dysfunction or β-blockers and antiarrhythmic therapy; and (3) ventilatory inefficiency caused by left-heart failure with pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease, or cachexia. Most of these variables also have prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting lifestyle and therapeutic interventions.

Advances in heart surgery over the past 30 years have significantly improved the prognosis of congenital heart diseases (CHD). Therefore, the epidemiology of CHD has changed dramatically with a shift of mortality from pediatrics to adulthood and an increased prevalence of complex CHD. Today, caregivers and patients focus their interests to new perspectives: improving the quality of life, practicing sports, improving psychosocial care. Cardiac rehabilitation is completely integrated in these new therapeutic strategies. The starting point is the cardiopulmonary exercise test (CPET), with the measurement of oxygen uptake, or "VO2". CPET is now recommended in the follow-up of the adults with CHD. Maximum oxygen uptake correlates to the quality of life of children and adults with CHD. The principles of the rehabilitation in patients with heart failure may usually be applied to CHD patients. Some studies in complex CHD showed improvement of VO2 and quality of life after rehabilitation, without any adverse events. However few physicians have the experience in rehabilitation among CHD patients, especially children. Randomized trials on cardiac rehabilitation in adult and pediatric CHD patients are essential to increase the level of evidence and lead to specific guidelines in this population., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)