Socioeconomic Factors Influencing Pediatric Peak Oxygen Consumption Prediction.

OBJECTIVE: To determine if socioeconomic status (SES) has a greater effect than standard demographic values on predicted peak oxygen consumption (pVO2). STUDY DESIGN: We conducted a single-institution, retrospective analysis of maximal cardiopulmonary exercise test (CPET) data from 2010 to 2020 for healthy patients age <19 years with body mass index (BMI) percentile (BMI%) between 5-95. Data were sorted by self-identified race, BMI%, and adjusted gross income (AGI); AGI served as a surrogate for SES. Mean percent predicted pVO2 (pppVO2) was compared between groups. Linear regression was used to adjust for differences. RESULTS: A total of 541 CPETs met inclusion criteria. Mean pppVO2 was 97% ± 22.6 predicted (P < .01) with 30% below criterion standard for normal (85% predicted). After excluding unknown AGI and race, 418 CPETs remained. Mean pppVO2 was lower for Blacks (n = 36) and Latinx (n = 26) compared with Whites (n = 333, P < .01). Mean pppVO2 declined as AGI decreased (P < .01). The differences in pppVO2 between racial categories remained significant when adjusted for BMI% (Black r = -7.3, P = .035; Latinx r = -15.4, P < .01). These differences both decreased in magnitude and were no longer significant when adjusted for AGI (Black r = -6.0, P = .150; Latinx r = -9.3, P = .06). CONCLUSIONS: Lower SES correlates with lower measured cardiovascular fitness and may confound data interpretation. When using normative reference ranges in clinical decision making, providers should recognize that social determinants of health may influence predicted fitness. Social inequities should be considered when assessing pediatric cardiovascular fitness.

Combined Exercise and Mindset Training During a Pediatric Cardiac Fitness and Rehabilitation Program Benefits Youth with a Range of Congenital Heart Disease.

Pediatric cardiac fitness and rehabilitation programs vary widely in structure and content. The Cardiac Fitness Program (CFP) is built on traditional training pillars of aerobic, strength, and flexibility, and adds a fourth, training a positive mindset. This study assesses whether the systematic and comprehensive framework of the CFP results in broad benefits for a range of patients with congenital heart disease (CHD). Data from participants between 01/2017 and 12/2022 were analyzed. Pre- and post-CFP cardiopulmonary exercise test parameters, strength and flexibility metrics, and mindset survey results were compared overall, and by sex, age, diagnosis, and hemodynamic level. Of 62 participants (median age 15.5 years, range 8 to 23, 50% female), 3% had simple, 37% complex, 24% single ventricle CHD, and 35% arrhythmia, cardiomyopathy, or transplant. Significant improvements were noted in aerobic fitness (mean 9 ± 15% increase in % predicted peak oxygen consumption, p < 0.001). Strength metrics significantly improved (each p < 0.001), as did flexibility (p < 0.001). Patient-reported positive mindset scores did not improve significantly (mean increase 1.8 ± 5.1, p = 0.10, n = 25), whereas parents reported significant improvements (5.9 ± 10.4, p = 0.02, n = 20). Improvements were not significantly different by sex, age, diagnosis, or hemodynamic level. Comprehensive training across four pillars of fitness yields significant improvements in aerobic fitness, strength, flexibility, and parent-reported mindset scores for pediatric CHD patients, regardless of patient characteristics, diagnosis type, or severity of hemodynamic limitation. Further study is warranted into optimal standardization of training and whether a comprehensive approach amplifies individual pillars to create more than the sum of its parts.

Flexibility: A Hidden But Trainable Morbidity in Pediatric Patients with Congenital Heart Disease.

Flexibility is important for range of motion, muscular performance, and injury prevention with exercise. Promoting exercise is important for patients with congenital and pediatric acquired heart disease (CHD), yet there are a paucity of data addressing flexibility in this population. We hypothesized that flexibility was worse in pediatric patients with CHD than the general population but could be improved with directed training. Patients at Boston Children's Hospital who participated in the pediatric Cardiac Fitness Program between 09/2016 and 11/2022 were retrospectively analyzed. Flexibility was assessed via sit-and-reach (SaR) box. Data from baseline and 60 days into the fitness program intervention were compared to age-matched population norms, and changes over time were assessed. Analyses were also stratified by sex and history of sternotomy. Patients with paired baseline and 60-day data were analyzed (n = 46, age 8-23 years old, 52% male). The mean SaR at baseline for CHD patients was 24.3 cm, significantly lower than the population norm (p = 0.002). The mean for male (n = 24, 21.2 cm) and female (n = 22, 27.2 cm) CHD patients was significantly lower than their respective population norms (p = 0.017 and p = 0.026, respectively). After the fitness intervention, flexibility in CHD patients significantly improved to normal, including patients with a history of sternotomy. Flexibility was significantly lower in CHD patients than the general population, but normalized with training. Further research is warranted to investigate associations of flexibility with other measures of fitness, cardiovascular status, and quality of life, as well as benefits gained with training.

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.

Disproportionate Negative Career Impact of the COVID-19 Pandemic on Female Pediatric Cardiologists in the Northeast United States.

The COVID-19 pandemic has had a dramatic impact on practicing physicians, with effects in clinical practice, academic pursuits, research endeavors, and personal lives. Women in medicine have been uniquely impacted. We examined the impact of the pandemic on the careers of pediatric cardiologists in the Northeast with an anonymous online survey. Participants reported demographic data, information on work hours, administrative burden, career satisfaction, academic productivity, and burnout. We approached 490 cardiologists and received 127 completed surveys (response rate 26%; 49% female). Among all respondents, 72% reported increased burnout, 43% reported decreased career satisfaction, and 57% reported decreased academic productivity. In multivariable ordinal regression analysis, when compared to male physicians, females were 2.4 times more likely to report decreased overall career satisfaction (p = 0.027), 2.6 times more likely to report decreased academic productivity (p = 0.028), and 2.6 times more likely to report increased feelings of burnout "to a large degree" (p = 0.022). Among all respondents, decreased career satisfaction was independently associated with increased household responsibility (OR = 4.4, p = 0.001). Increased administrative burden was independently associated with decreased academic productivity (OR = 2.6, p = 0.038). Open-ended responses highlighted loss of community due to remote work and blurring of the boundaries between work and home. Conversely, respondents appreciated flexibility to work remotely. In conclusion, the majority of pediatric cardiologists in the Northeast experienced negative career impacts due to the COVID-19 pandemic. Important gender differences emerged, with female physicians disproportionately reporting increased burnout, decreased career satisfaction, and decreased academic productivity.

Activity During the COVID-19 Pandemic in Children with Cardiac Rhythm Management Devices.

Decreased physical activity is associated with cardiovascular, metabolic and mental health disease. While decreases in physical activity during the COVID-19 pandemic have been described in the general population, there is a paucity of data regarding children with underlying cardiovascular disease. We hypothesized there would be a decrease in physical activity at the onset of the COVID-19 pandemic. Performed a single-center, retrospective cohort study of children aged < 19 years with cardiac rhythm management devices. Patients were included if they had device-measured physical activity data from > 80% of dates from February 3, 2020 through June 30, 2020. Patients with significant neurologic/neuromuscular disease were excluded. We identified 144 patients with a median age of 15.4 years. 47% were female. 34% had congenital heart disease, 20% had cardiomyopathy, 19% had an inherited arrhythmia syndrome and 5% had atrioventricular block without congenital heart disease. 47% of patients had an implantable loop recorder, 29% had a permanent pacemaker and 24% had an implantable cardioverter-defibrillator. We observed a significant decrease in device-measured physical activity from baseline (February 3-March 9), with up to a 21% decrease in physical activity during mid-March through early May. Activity levels returned to pre-pandemic levels in June. Physical activity sharply declined in children with cardiac rhythm management devices at the onset of the COVID-19 pandemic. These data highlight the importance of finding strategies to maintain physical activity during the current pandemic and future public health crises.

Better preoperative exercise function is associated with shorter hospital stay after paediatric pulmonary valve replacement or conduit revision.

Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored. METHODS: Single centre retrospective cohort study of patients aged 8-18 years who underwent surgical pulmonary valve replacement. The primary predictor was preoperative percent predicted peak oxygen consumption, and primary outcome was total hospital length of stay. Clinical, imaging, and cardiopulmonary exercise test data were reviewed and compared to total hospital length of stay. Cox proportional hazards regression was used to examine the association between total hospital length of stay and percent predicted peak oxygen consumption. RESULTS: Three-hundred and seventy patients undergoing pulmonary valve replacement/conduit change between 2003 and 2017 at Boston Children's Hospital were identified. Ninety had preoperative cardiopulmonary exercise tests within 6 months of surgery. Exclusion for inadequate exercise data (n = 3) and imaging data (n = 1) left 86 patients for review. Patients with percent predicted peak oxygen consumption ≥ 70% (n = 46, 53%) had shorter total hospital length of stay (4.4 days) than the 40 with percent predicted peak oxygen consumption <70% (5.4 days, p = 0.007). Median percent predicted peak oxygen consumption increased over sequential surgical eras (p < 0.001), but total hospital length of stay did not correlate with surgical era, preoperative left ventricular function, or preoperative right ventricular dilation. CONCLUSION: Children undergoing surgical pulmonary valve replacement with better preoperative exercise capacity had shorter total hospital length of stay. Exercise capacity is a potentially modifiable factor prior to and after pulmonary valve replacement. Until more patients systematically undergo cardiopulmonary exercise tests, the full impact of optimisation of exercise capacity will not be known.

Neurodevelopmental and psychosocial interventions for individuals with CHD: a research agenda and recommendations from the Cardiac Neurodevelopmental Outcome Collaborative.

In 2018, the Neurodevelopmental and Psychosocial Interventions Working Group of the Cardiac Neurodevelopmental Outcome Collaborative convened through support from an R13 grant from the National Heart, Lung, and Blood Institute to survey the state of neurodevelopmental and psychosocial intervention research in CHD and to propose a slate of critical questions and investigations required to improve outcomes for this growing population of survivors and their families. Prior research, although limited, suggests that individualised developmental care interventions delivered early in life are beneficial for improving a range of outcomes including feeding, motor and cognitive development, and physiological regulation. Interventions to address self-regulatory, cognitive, and social-emotional challenges have shown promise in other medical populations, yet their applicability and effectiveness for use in individuals with CHD have not been examined. To move this field of research forward, we must strive to better understand the impact of neurodevelopmental and psychosocial intervention within the CHD population including adapting existing interventions for individuals with CHD. We must examine the ways in which dedicated cardiac neurodevelopmental follow-up programmes bolster resilience and support children and families through the myriad transitions inherent to the experience of living with CHD. And, we must ensure that interventions are person-/family-centred, inclusive of individuals from diverse cultural backgrounds as well as those with genetic/medical comorbidities, and proactive in their efforts to include individuals who are at highest risk but who may be traditionally less likely to participate in intervention trials.

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.

Identifying elements for a comprehensive paediatric cardiac rehabilitation programme.

INTRODUCTION: The aim of this study was to identify relevant content among four important domains for the development and structure of a paediatric cardiac rehabilitation curriculum for young patients with congenital heart disease using a consensus approach. METHODS: A three-round e-Delphi study among congenital heart disease and paediatric exercise physiology experts was conducted. Round 1, experts provided opinions in a closed- and open-ended electronic questionnaire to identify specific elements necessary for inclusion in a paediatric cardiac rehabilitation programme. Round 2, experts were asked to re-rate the same items after feedback and summary data were provided from round 1. Round 3, the same experts were asked to re-rate items that did not reach consensus from round 2. RESULTS: Forty-seven experts were contacted via e-mail to participate on the Delphi panel, 37 consented, 35 completed round 1, 29 completed round 2, and 28 completed the final round. After round 2, consensus was reached in 55 of 60 (92%) questionnaire items across four domains: exercise training, education, outcome metrics, and self-confidence. CONCLUSION: This study established consensus towards programme structure, exercise training principles, educational content, patient outcome measures, and self-confidence promotion. By identifying the key components within each domain, there is potential to benchmark recommended standards and practice guidelines for the development of a paediatric cardiac rehabilitation curriculum to be used and tested by exercise physiologists, paediatric and adult congenital cardiologists, and other healthcare team members for optimising the health and wellness of paediatric patients with congenital heart disease.

Positive mindset and exercise capacity in school-aged children and adolescents with congenital heart disease.

Introduction: Patients with congenital heart disease (CHD) have variable degrees of peak oxygen consumption (VO2) that can be improved with supervised fitness training. The ability to exercise is affected by anatomy, hemodynamics, and motivation. Motivation is in part related to mindset, or personal attitudes and beliefs, and a more positive mindset around exercise has been associated with better outcomes. It is unknown whether variations in measured peak VO2 in patients with CHD are related to having a positive mindset. Methods: Patient's ages 8-17 years with CHD were administered quality of life and physical activity questionnaires at the time of their routine cardiopulmonary exercise test. Those with severe hemodynamic burden were excluded. Patients were grouped based on disease classification. Mindset was evaluated via validated questionnaires including a PROMIS Meaning and Purpose (MaP) survey and an Anxiety survey. Pearson correlation coefficients were calculated to estimate the magnitude of the association between percent predicted peak oxygen consumption (pppVO2) and questionnaire scores overall and within CHD subgroups. Results: Eighty-five patients participated; median age was 14.7 years, 53% were female, 66% had complex CHD, 20% had simple CHD, and 14% had single ventricle heart disease. Mean MaP scores were significantly lower in all CHD groups compared to population norms (p < 0.001). As a group, MaP scores were positively associated with the amount of reported physical activity (p = 0.017). In patients with simple CHD, MaP scores were positively associated with pppVO2 (p = 0.015). The association was even stronger for MaP:Anxiety, with worse ratios associated with lower pppVO2 (p = 0.005). Patients with complex and single ventricle CHD did not show a similar association. Conclusions: Patients with CHD, regardless of severity, had lower meaning and purpose scores than the general population, and these scores were associated with amount of reported physical activity. In the simple CHD subset, having a more positive mindset was associated with higher peak VO2 and a more negative mindset with lower peak VO2. This relationship was not seen with more significant CHD. While underlying CHD diagnoses are not modifiable, mindset and peak VO2 are, and consideration should be given to measuring both as each may be a target for intervention.

Right ventricular contraction patterns in healthy children using three-dimensional echocardiography.

Background: The right ventricle (RV) has complex geometry and function, with motion along three separate axes-longitudinal, radial, and anteroposterior. Quantitative assessment of RV function by two-dimension echocardiography (2DE) has been limited as a consequence of this complexity, whereas newer three dimensional (3D) analysis offers the potential for more comprehensive assessment of the contributors to RV function. The aims of this study were to quantify the longitudinal, radial and anteroposterior components of global RV function using 3D echocardiography in a cohort of healthy children and to examine maturational changes in these parameters. Methods: Three-dimensional contours of the RV were generated from a cohort of healthy pediatric patients with structurally normal hearts at two centers. Traditional 2D and 3D echo characteristics were recorded. Using offline analysis of 3D datasets, RV motion was decomposed into three components, and ejection fractions (EF) were calculated (longitudinal-LEF; radial-REF; and anteroposterior-AEF). The individual decomposed EF values were indexed against the global RVEF. Strain values were calculated as well. Results: Data from 166 subjects were included in the analysis; median age was 13.5 years (range 0 to 17.4 years). Overall, AEF was greater than REF and LEF (29.2 ± 6.2% vs. 25.1 ± 7.2% and 25.7 ± 6.0%, respectively; p < 0.001). This remained true when indexed to overall EF (49.8 ± 8.7% vs. 43.3 ± 11.6% and 44.4 ± 10%, respectively; p < 0.001). Age-related differences were present for global RVEF, REF, and all components of RV strain. Conclusions: In healthy children, anteroposterior shortening is the dominant component of RV contraction. Evaluation of 3D parameters of the RV in children is feasible and enhances the overall understanding of RV function, which may allow improvements in recognition of dysfunction and assessment of treatment effects in the future.

ehealth technology in cardiac exercise therapeutics for pediatric patients with congenital and acquired heart conditions: a summary of evidence and future directions.

Many children and adolescents with congenital and acquired heart disease (CHD) are physically inactive and participate in an insufficient amount of moderate-to-vigorous intensity exercise. Although physical activity (PA) and exercise interventions are effective at improving short- and long-term physiological and psychosocial outcomes in youth with CHD, several barriers including resource limitations, financial costs, and knowledge inhibit widespread implementation and dissemination of these beneficial programs. New and developing eHealth, mHealth, and remote monitoring technologies offer a potentially transformative and cost-effective solution to increase access to PA and exercise programs for youth with CHD, yet little has been written on this topic. In this review, a cardiac exercise therapeutics (CET) model is presented as a systematic approach to PA and exercise, with assessment and testing guiding three sequential PA and exercise intervention approaches of progressive intensity and resource requirements: (1) PA and exercise promotion within a clinical setting; (2) unsupervised exercise prescription; and (3) medically supervised fitness training intervention (i.e., cardiac rehabilitation). Using the CET model, the goal of this review is to summarize the current evidence describing the application of novel technologies within CET in populations of children and adolescents with CHD and introduce potential future applications of these technologies with an emphasis on improving equity and access to patients in low-resource settings and underserved communities.

Core components of a rehabilitation program in pediatric cardiac disease.

There is increasing effort in both the inpatient and outpatient setting to improve care, function, and quality of life for children with congenital heart disease, and to decrease complications. As the mortality rates of surgical procedures for congenital heart disease decrease, improvement in perioperative morbidity and quality of life have become key metrics of quality of care. Quality of life and function in patients with congenital heart disease can be affected by multiple factors: the underlying heart condition, cardiac surgery, complications, and medical treatment. Some of the functional areas affected are motor abilities, exercise capacity, feeding, speech, cognition, and psychosocial adjustment. Rehabilitation interventions aim to enhance and restore functional ability and quality of life for those with physical impairments or disabilities. Interventions such as exercise training have been extensively evaluated in adults with acquired heart disease, and rehabilitation interventions for pediatric patients with congenital heart disease have similar potential to improve perioperative morbidity and quality of life. However, literature regarding the pediatric population is limited. We have gathered a multidisciplinary team of experts from major institutions to create evidence- and practice-based guidelines for pediatric cardiac rehabilitation programs in both inpatient and outpatient settings. To improve the quality of life of pediatric patients with congenital heart disease, we propose the use of individualized multidisciplinary rehabilitation programs that include: medical management; neuropsychology; nursing care; rehabilitation equipment; physical, occupational, speech, and feeding therapies; and exercise training.

Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation.

INTRODUCTION: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures. METHODS: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs. RESULTS: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice. CONCLUSION: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients.

Regional Implementation of a Pediatric Cardiology Syncope Algorithm Using Standardized Clinical Assessment and Management Plans (SCAMPS) Methodology.

BACKGROUND: Pediatric syncope is common. Cardiac causes are rarely found. We describe and assess a pragmatic approach to these patients first seen by a pediatric cardiologist in the New England region, using Standardized Clinical Assessment and Management Plans (SCAMPs). METHODS AND RESULTS: Ambulatory patients aged 7 to 21 years initially seen for syncope at participating New England Congenital Cardiology Association practices over a 2.5-year period were evaluated using a SCAMP. Findings were iteratively analyzed and the care pathway was revised. The vast majority (85%) of the 1254 patients had typical syncope. A minority had exercise-related or more problematic symptoms. Guideline-defined testing identified one patient with cardiac syncope. Syncope Severity Scores correlated well between physician and patient perceived symptoms. Orthostatic vital signs were of limited use. Largely incidental findings were seen in 10% of ECGs and 11% of echocardiograms. The 10% returning for follow-up, by design, reported more significant symptoms, but did not have newly recognized cardiac disease. Iterative analysis helped refine the approach. CONCLUSIONS: SCAMP methodology confirmed that the vast majority of children referred to the outpatient pediatric cardiology setting had typical low-severity neurally mediated syncope that could be effectively evaluated in a single visit using minimal resources. A simple scoring system can help triage patients into treatment categories. Prespecified criteria permitted the effective diagnosis of the single patient with a clear cardiac etiology. Patients with higher syncope scores still have a very low risk of cardiac disease, but may warrant attention.

Relationship between Exercise Parameters and Noninvasive Indices of Right Ventricular Function in Patients with Biventricular Circulation and Systemic Right Ventricle.

OBJECTIVES: In patients with systemic right ventricles (RVs) in a biventricular circulation, exercise capacity and RV function often deteriorate over time and echocardiographic assessment of systemic RV function is difficult. The purpose of this study was to examine the relationship between exercise capacity and RV function and to determine which noninvasive imaging parameters correlate most closely with exercise capacity. DESIGN: Patients with a systemic RV (D-loop transposition of the great arteries [TGA] after atrial switch procedure or physiologically "corrected" TGA) who underwent cardiopulmonary exercise testing (CPX) and noninvasive imaging (cardiac magnetic resonance [CMR] and echocardiography [echo]) within 1 year of CPX were identified. Regression analysis was used to evaluate the relationship between exercise variables and noninvasive indices of ventricular function. RESULTS: We identified 92 patients with 149 encounters (mean age 31.0 years, 61% men, 70% D-loop TGA) meeting inclusion criteria. Statistically significant correlations between % predicted peak oxygen uptake (%pVO2 ) and RV ejection fraction (EF) (r = 0.29, P = .0007), indexed RV end-systolic volume (r = -0.25, P = .002), and Tei index (r = -0.22, P = .03) were found. In patients without additional hemodynamically significant lesions, the correlations between %pVO2 and RV EF (r = 0.37, P = .0007) and the Tei index (r = -0.28, P = .03) strengthened and a correlation emerged between %pVO2 and dP/dtic (r = 0.31, P = .007). On multivariable analysis, Tei index was the only statistically significant correlate of %pVO2 (P = .04). CONCLUSIONS: In patients with systemic RVs in a biventricular circulation, CMR-derived RVEF and echo-derived Tei index correlate with %pVO2 . On multivariable analysis, the Tei index was the strongest predictor of peak %pVO2 response.

Regional implementation of a pediatric cardiology chest pain guideline using SCAMPs methodology.

BACKGROUND AND OBJECTIVES: Chest pain is a complaint for which children are frequently evaluated. Cardiac causes are rarely found despite expenditure of considerable time and resources. We describe validation throughout New England of a clinical guideline for cost-effective evaluation of pediatric patients first seen by a cardiologist for chest pain using a unique methodology termed the Standardized Clinical Assessment and Management Plans (SCAMPs). METHODS: A total of 1016 ambulatory patients, ages 7 to 21 years initially seen for chest pain at Boston Children's Hospital (BCH) or the New England Congenital Cardiology Association (NECCA) practices, were evaluated by using a SCAMPs chest pain guideline. Findings were analyzed for diagnostic elements, patterns of care, and compliance with the guideline. Results from the NECCA practices were compared with those of Boston Children's Hospital, a regional core academic center. RESULTS: Two patients had chest pain due to a cardiac etiology, 1 with pericarditis and 1 with an anomalous coronary artery origin. Testing performed outside of guideline recommendations demonstrated only incidental findings. Patients returning for persistent symptoms did not have cardiac disease. The pattern of care for the NECCA practices and BCH differed minimally. CONCLUSIONS: By using SCAMPs methodology, we have demonstrated that chest pain in children is rarely caused by heart disease and can be evaluated in the ambulatory setting efficiently and effectively using minimal resources. The methodology can be implemented regionally across a wide range of clinical practice settings and its approach can overcome a number of barriers that often limit clinical practice guideline implementation.

Sustained effects of cardiac rehabilitation in children with serious congenital heart disease.

OBJECTIVE: Past studies have documented the acute benefits of cardiac rehabilitation in children with congenital heart disease. It is not known whether these benefits persist. PATIENTS AND METHODS: Fifteen patients, ages 8 to 17 years, with complex congenital heart disease, whose exercise function immediately after a 12-week cardiac rehabilitation program was superior to that present on a precardiac rehabilitation exercise test, were restudied 6.9 +/- 1.6 months after completion of the cardiac rehabilitation program (approximately 1 year after the precardiac rehabilitation study). Changes in exercise function relative to baseline, precardiac rehabilitation exercise tests were also compared with changes observed in a group of 18 control subjects, with similar diagnoses, who also had 2 exercise tests separated by a year but did not undergo cardiac rehabilitation. RESULTS: The cardiac rehabilitation patients' exercise function did not change significantly over the 6.9-month period after the completion of the cardiac rehabilitation program; percentage of predicted peak oxygen consumption and peak work rate remained significantly superior to baseline, precardiac rehabilitation values. These changes were also associated with improvements in self-esteem, behavior, and emotional state. In contrast, among the control subjects, small, but statistically insignificant declines in peak oxygen consumption and peak work rate were observed on the final exercise test compared with values obtained at baseline, 1 year earlier. The improvements realized by the cardiac rehabilitation patients differed significantly from the concurrent changes observed among the control subjects and appeared to be a result of an increase in the oxygen pulse at peak exercise; significant changes in peak heart rate were not observed. CONCLUSIONS: In patients with congenital heart disease, cardiac rehabilitation produces significant, sustained improvements in exercise function, behavior, self-esteem, and emotional state.

Impact of cardiac rehabilitation on the exercise function of children with serious congenital heart disease.

OBJECTIVES: The exercise capacity of children with congenital heart disease (CHD) is often depressed. This depression is thought to be attributable to (1) residual hemodynamic defects and (2) deconditioning secondary to physical inactivity. We hypothesized that this latter component would be ameliorated by a formal cardiac rehabilitation program designed specifically for children. The objective of this study was to characterize the effect of a cardiac rehabilitation program on the exercise performance of children with CHD and to define the physiologic mechanisms that might account for any improvements that are observed. METHODS: Nineteen patients with CHD who were referred for exercise testing and found to have a peak oxygen consumption (VO2) and/or peak work rate <80% of predicted were enrolled in the study. Sixteen patients (11 Fontan patients, 5 with other CHD) completed the program and had postrehabilitation exercise tests, results of which were compared with the prerehabilitation studies. RESULTS: Improvements were found in 15 of 16 patients. Peak VO2 rose from 26.4 +/- 9.1 to 30.7 +/- 9.2 mL/kg per min; peak work rate from 93 +/- 32 to 106 +/- 34 W, and the ventilatory anaerobic threshold from 14.2 +/- 4.8 to 17.4 +/- 4.5 mL/kg per min. The peak heart rate and peak respiratory exchange ratio did not change, suggesting that the improvements were not attributable merely to an increased effort. In contrast, the peak oxygen pulse rose significantly, from 7.6 +/- 2.8 to 9.7 +/- 4.1 mL/beat, an improvement that can be attributed only to an increase in stroke volume and/or oxygen extraction at peak exercise. No patient experienced rehabilitation-related complications. CONCLUSION: Cardiac rehabilitation can improve the exercise performance of children with CHD. This improvement is mediated by an increase in stroke volume and/or oxygen extraction during exercise. Routine use of formal cardiac rehabilitation may greatly reduce the morbidity of complex CHD.