Storage, preservation, and rehabilitation of living heart valves to treat congenital heart disease.

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.

2024 SIAC guidelines on cardiorespiratory rehabilitation in pediatric patients with congenital heart disease.

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.

FORCE Risk Stratification Tool for Pediatric Cardiac Rehabilitation and Fitness Programs.

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.

Habilitación de funciones frontales básicas en cardiópatas congénitos a través de LEGO® Education / Basic frontal functions qualification in congenital heart disease with LEGO® Education

Las cardiopatías congénitas se consideran una de las anomalías que alteran la irrigación y el intercambio de oxigenación adecuado a las principales venas y arterias. Esto puede generar consecuencias en el desarrollo neurológico que se puede traducir en retraso psicomotor, déficits de aprendizaje, dificultades académicas y problemas de integración social. Para mejorar los trastornos cognitivos, se propone la habilitación cognitiva basada en los principios de mecánica y robótica de LEGO® Education. El objetivo de este estudio fue medir el efecto de un programa de intervención, basado en el uso de ensamblado y programación robótica con LEGO® Education, sobre las funciones frontales básicas como primera aproximación a un modelo propuesto en pacientes cardiópatas congénitos que han sido sometidos a cirugía cardiovascular. Se trató de un estudio de serie de casos, en el que finalizaron el tratamiento una niña y dos niños con cardiopatías congénitas con RACHS 2 y 3. Se aplicaron sub-escalas BANFE-2 y el cuestionario neuropsicológico de daño frontal antes y después del tratamiento; así como una escala para medir el nivel de ejecución por intervención, durante las ocho sesiones. Los resultados muestran en la escala BANFE2, cambios en las medias de las funciones frontales básicas, de daño leve-moderado y normal a normal alto, principalmente en memoria de trabajo y fluidez verbal. En esta primera aproximación, el método LEGO® Education mostró ser una buena herramienta para la habilitación neuropsicológica de estos pacientes.

Congenital heart diseases are considered to be an anomaly which alter the irrigation and the adequate exchange of oxygenation to the main veins and arteries. They can have neurodevelopmental consequences that could translate into psychomotor retardation, learning deficits, academic difficulties, and social integration problems. Cognitive empowerment based on the mechanics and robotics principles of LEGO® Education is proposed to improve cognitive disorders. In this study, the objective was to measure the effect of an intervention program, based on the use of assembly and robotic programming with LEGO® Education, upon basic frontal functions as a first approach to a proposed model in congenital heart disease patients who have undergone cardiovascular surgery. This was a case-series study, in which a girl and two boys with congenital heart disease with RACHS 2 and 3, completed the treatment. BANFE-2 subscales and the neuropsychological questionnaire of frontal damage were applied before and after the treatment; as well as a scale to measure the level of performance per intervention, through all the eight sessions. The BANFE-2 scale showed changes in the means of frontal functions, from mild-moderate damage and normal to high normal, mainly in working memory and verbal fluency. In this first approach, LEGO® Education method proved to be a useful tool for the neuropsychological empowerment of these patients.

Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study.

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 (V̇O2 peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease-specific reference V̇O2 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 V̇O2 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 V̇O2 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. V̇O2 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 V̇O2 peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

Exercise training in paediatric congenital heart disease: fit for purpose?

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.

Exercise prescription improves exercise tolerance in young children with CHD: a randomised clinical trial.

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.

E-Health Exercise Intervention for Pediatric Patients with Congenital Heart Disease: A Randomized Controlled Trial.

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.

Exercise Testing and Training in Adults With Congenital Heart Disease: A Surgical Perspective.

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.

A Comparative Study on Breast Milk Feeding and Formula Milk Feeding in Infants With Congenital Heart Disease After Surgery: A Retrospective Study.

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.

Physical activity interventions for people with congenital heart disease.

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.

Establishing a Comprehensive Pediatric Cardiac Fitness and Rehabilitation Program for Congenital Heart Disease.

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.

Physical Activity Promotion in Pediatric Congenital Heart Disease: Are We Running Late?

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.

Seguridad y beneficio de la rehabilitación cardiopulmonar en cardiopatías congénitas complejas / Safety and clinical benefit of cardiopulmonary rehabilitation in complex congenital heart disease

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Cardiopulmonary Rehabilitation Improves Respiratory Muscle Function and Functional Capacity in Children with Congenital Heart Disease. A Prospective Cohort Study.

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 H2O, 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.

Reduced Physical Activity During COVID-19 Pandemic in Children With Congenital Heart Disease.

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.

Cardiac Rehabilitation for Adults and Adolescents With Congenital Heart Disease: EXTENDING BEYOND THE TYPICAL PATIENT POPULATION.

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.