Facility-Based and Virtual Cardiac Rehabilitation in Young Patients with Heart Disease During the COVID-19 Era.

Cardiac rehabilitation (CR) is an important tool for improving fitness and quality of life in those with heart disease (HD). Few pediatric centers use CR to care for these patients, and virtual CR is rarely used. In addition, it is unclear how the COVID-19 era has changed CR outcomes. This study assessed fitness improvements in young HD patients participating in both facility-based and virtual CR during the COVID-19 pandemic. This retrospective single-center cohort study included new patients who completed CR from March 2020 through July 2022. CR outcomes included physical, performance, and psychosocial measures. Comparison between serial testing was performed with a paired t test with P < 0.05 was considered significant. Data are reported as mean ± standard deviation. There were 47 patients (19 ± 7.3 years old; 49% male) who completed CR. Improvements were seen in peak oxygen consumption (VO2, 62.3 ± 16.1 v 71 ± 18.2% of predicted, p = 0.0007), 6-min walk (6 MW) distance (401 ± 163.8 v 480.7 ± 119.2 m, p = < 0.0001), sit to stand (16.2 ± 4.9 v 22.1 ± 6.6 repetitions; p = < 0.0001), Patient Health Questionnaire-9 (PHQ-9) (5.9 ± 4.3 v 4.4 ± 4.2; p = 0.002), and Physical Component Score (39.9 ± 10.1 v 44.9 ± 8.8; p = 0.002). Facility-based CR enrollees were less likely to complete CR than virtual patients (60%, 33/55 v 80%, 12/15; p = 0.005). Increases in peak VO2 (60 ± 15.3 v 70.2 ± 17.8% of predicted; p = 0.002) were seen among those that completed facility-based CR; this was not observed in the virtual group. Both groups demonstrated improvement in 6 MW distance, sit-to-stand repetitions, and sit-and-reach distance. Completion of a CR program resulted in fitness improvements during the COVID-19 era regardless of location, although peak VO2 improved more for the in-person group.

Pulmonary effects on exercise testing in tetralogy of Fallot patients repaired with a transannular patch.

BACKGROUND: A transannular patch is often used in the contemporary surgical repair of tetralogy of Fallot. This can lead to significant pulmonary insufficiency and increased right ventricular volumes and ultimately pulmonary valve replacement. Cardiopulmonary exercise testing is used to assess exercise capacity in tetralogy of Fallot patients before pulmonary valve replacement. There is only few published literatures on how lung function affects functional capacity in tetralogy of Fallot patients repaired with a transannular patch. METHODS: A retrospective chart review was done from 2015 to 2017 on patients with tetralogy of Fallot who underwent maximal effort cardiopulmonary exercise testing with cycle ergometry and with concurrent pulmonary function testing. Tetralogy of Fallot patients repaired with a transannular patch without pulmonary valve replacement were compared with age, gender, and size-matched normal controls. RESULTS: In the tetralogy of Fallot group, 24 out of 57 patients underwent primary repair with a transannular patch. When compared to the normal controls, they demonstrated abnormal predicted forced expiratory volume in one second (79 ± 23.1% versus 90.7 ± 14.1%, p<0.05), predicted maximal voluntary ventilation (74 ± 18% versus 90.5 ± 16.2%, p<0.05) while having low-normal predicted forced vital capacity (80.5 ± 17.2% versus 90.2 ± 12.4%, p<0.05) and normal breathing reserve percentage (50.3 ± 11.3% versus 47.5 ± 17.3%, p = 0.52). Cardiopulmonary exercise testing abnormalities included significantly lower percent predicted oxygen consumption (63.2 ± 12.2% versus 87 ± 12.1%, p<0.05), maximal heart rate (171.8 ± 18.9 versus 184.6 ± 13.6, p<0.05), and percent predicted maximum workload (61.7 ± 15.9% versus 88.3 ± 21.5%, p<0.05). CONCLUSIONS: Tetralogy of Fallot patients repaired with a transannular patch can have abnormal pulmonary function testing with poor exercise capacity in addition to chronotropic incompetence and impaired muscular power.

Pulmonary responses following cardiac rehabilitation and the relationship with functional outcomes in children and young adults with heart disease.

Introduction: Patients with congenital heart disease (CHD) often have pulmonary abnormalities and exercise intolerance following cardiac surgery. Cardiac rehabilitation (CR) improves exercise capacity in patients with CHD, but minimal study has been performed to see if resting and dynamic pulmonary performance improves following CR in those with prior cardiac surgery. Methods: This was a retrospective cohort study of all patients who completed ≥12 weeks of CR from 2018 through 2022. Demographic, cardiopulmonary exercise test (CPET), spirometry, 6-minute walk, functional strength measures, and outcomes data were collected. Data are presented as median[IQR]. A Student's t-test was used for comparisons between groups and serial measurements were measured with a paired t-test. A p < 0.05 was considered significant. Results: There were a total of 37 patients [age 16.7 (14.2-20.1) years; 46% male] included. Patients with prior surgery (n = 26) were more likely to have abnormal spirometry data than those without heart disease (n = 11) (forced vital capacity [FVC] 76.7 [69.1-84.3]% vs. 96.4 [88.1-104.7]%, p = 0.002), but neither group experienced a significant change in spirometry. On CPET, peak oxygen consumption increased but there was no change in other pulmonary measures during exercise. Percent predicted FVC correlated with hand grip strength (r = 0.57, p = 0.0003) and percent predicted oxygen consumption (r = 0.43, p = 0.009). The number of prior sternotomies showed negative associations with both percent predicted FVC (r = -0.43, p = 0.04) and FEV1 (r = -0.47, p = 0.02). Discussion: Youth and young adults with a prior history of cardiac surgery have resting and dynamic pulmonary abnormalities that do not improve following CR. Multiple sternotomies are associated with worse pulmonary function.

Stable fitness during COVID-19: Results of serial testing in a cohort of youth with heart disease.

Background: Little is known about how sport and school restrictions early during the novel coronavirus 2019 (COVID-19) pandemic impacted exercise performance and body composition in youth with heart disease (HD). Methods: A retrospective chart review was performed on all patients with HD who had serial exercise testing and body composition via bioimpedance analysis performed within 12 months before and during the COVID-19 pandemic. Formal activity restriction was noted as present or absent. Analysis was performed with a paired t-test. Results: There were 33 patients (mean age 15.3 ± 3.4 years; 46% male) with serial testing completed (18 electrophysiologic diagnosis, 15 congenital HD). There was an increase in skeletal muscle mass (SMM) (24.1 ± 9.2-25.9 ± 9.1 kg, p < 0.0001), weight (58.7 ± 21.5-63.9 ± 22 kg, p < 0.0001), and body fat percentage (22.7 ± 9.4-24.7 ± 10.4%, p = 0.04). The results were similar when stratified by age <18 years old (n = 27) or by sex (male 16, female 17), consistent with typical pubertal changes in this predominantly adolescent population. Absolute peak VO2 increased, but this was due to somatic growth and aging as evidenced by no change in % of predicted peak VO2. There remained no difference in predicted peak VO2 when excluding patients with pre-existing activity restrictions (n = 12). Review of similar serial testing in 65 patients in the 3 years before the pandemic demonstrated equivalent findings. Conclusions: The COVID-19 pandemic and related lifestyle changes do not appear to have had substantial negative impacts on aerobic fitness or body composition in children and young adults with HD.

The Adaptation of Pediatric Exercise Testing Programs to the Coronavirus/COVID-19 Pandemic.

OBJECTIVE: Response to the coronavirus/COVID-19 pandemic has resulted in several initiatives that directly impact hospital operations. There has been minimal information on how COVID-19 has affected exercise testing in pediatric patients. DESIGN: A web-based survey was designed and sent to pediatric exercise testing laboratories in the United States and Canada. Questions were designed to understand the initial and ongoing adaptations made by pediatric exercise testing laboratories in response to COVID-19. Results were analyzed as frequency data. RESULTS: There were responses from 42% (35/85) of programs, with 68% (23/34) of laboratories discontinuing all exercise testing. Of the 23 programs that discontinued testing, 15 (65%) are actively working on triage plans to reopen the exercise laboratory. Personal protective equipment use include gloves (96%; 25/26), surgical masks (88%; 23/26), N-95 masks (54%; 14/26), face shields (69%; 18/26), and gowns (62%; 16/26). Approximately 47% (15/32) of programs that typically acquire metabolic measurements reported either ceasing or modifying metabolic measurements during COVID-19. Additionally, 62% (16/26) of the programs that previously obtained pulmonary function testing reported either ceasing or modifying pulmonary function testing. Almost 60% of respondents expressed a desire for additional guidance on exercise laboratory management during COVID-19. CONCLUSIONS: Pediatric exercise testing laboratories largely closed during the early pandemic, with many of these programs either now open or working on a plan to open. Despite this, there remains heterogeneity in how to minimize exposure risks to patients and staff. Standardization of exercise testing guidelines during the COVID-19 pandemic may help reduce some of these differences.

Skeletal Muscle Mass Is Linked to Cardiorespiratory Fitness in Youth.

INTRODUCTION: Cardiorespiratory fitness (CRF) measured by oxygen consumption (V˙O2) during exercise is an important marker of health. The traditional method of indexing V˙O2 to total body mass is suboptimal because skeletal muscle mass (SMM), rather than fat and extracellular fluid, is the main contributor to CRF. The traditional estimating equations for peak V˙O2 in youth do not account for this. Bioelectric impedance analysis (BIA) is a noninvasive method to accurately measure body composition. The objectives of this study were to 1) examine the relationship of body composition indices and peak V˙O2 in healthy children, adolescents, and young adults, and 2) derive an optimized estimating equation incorporating BIA and compare its performance with traditional estimating equations. METHODS: A retrospective, cross-sectional, single-center study of patients <21 yr old referred for exercise testing who did not have underlying cardiovascular disease. All patients underwent BIA immediately before exercise testing. Univariable and multivariable linear regression models were constructed and tested for model performance. RESULTS: A total of 165 young healthy people (mean age 14 yr, 48% male) were studied. There was a strong and linear relationship between peak V˙O2 and SMM (R = 0.79). The sex difference in SMM explained the most variability in CRF between boys and girls. A generalized equation using SMM (peak V˙O2 = 302 - (23.7 × age) - (50.3 × [female = 1, male = 0]) + (81.8 × SMM)) had superior performance (R = 0.80) compared with estimating equations currently used in clinical practice (R = 0.67). CONCLUSIONS: SMM is a stronger correlate of CRF than is total body mass in youth and may be a better scaling variable to estimate expected peak V˙O2.

Body Composition and Exercise Performance in Youth With a Fontan Circulation: A Bio-Impedance Based Study.

Background Adults with a Fontan circulation tend to have myopenia and elevated adiposity when measured by dual energy x-ray absorptiometry. Bioelectrical impedance analysis is an alternative validated approach to assess body composition. We used bioelectrical impedance analysis to compare body composition between pediatric patients with a Fontan circulation and control individuals without heart disease. Methods and Results A retrospective chart review identified all patients aged <22 years with a Fontan circulation who presented for cardiopulmonary exercise testing and bioelectrical impedance analysis from April 2019 to January 2020. Data were compared with control subjects tested during the same period. We studied 47 patients with a Fontan circulation (53% boys; 15±3.1 years) and 165 controls (48% boys; 14.4±2.5 years). Fontan status was associated with shorter height, but similar age, sex, and overall body mass. Patients with Fontan had lower lean body mass (-12.0±22%, Z-score -0.5±1, P=0.005), skeletal muscle mass (-13.6±1.4%; Z-score, -0.5±1; P=0.004), skeletal muscle indexed to height (-10.3±13.3%; Z-score, -0.5±1; P=0.005), and higher percent body fat (+13.8±18.6%; Z-score, 0.4±1.2; P=0.03). Greater skeletal muscle mass was associated with higher peak oxygen consumption (r2=0.52, P<0.0001) and oxygen pulse (r2=0.68, P<0.0001). Patients who had suffered a late complication (ie, heart transplant referral or evidence of extracardiac organ dysfunction) of the Fontan operation (13 of 47, 27.7%) had lower skeletal muscle mass (P=0.048) and higher body fat percentage (P=0.003). Conclusions The Fontan circulation is associated with marked myopenia and increased adiposity. Higher muscle mass was associated with better exercise capacity. Fontan complications are associated with lower muscle mass and increased adiposity.

Decreased maximal aerobic capacity in pediatric chronic kidney disease.

Adult and pediatric patients with ESRD have impaired maximum oxygen consumption (VO(2) max), a reflection of the cardiopulmonary system's ability to meet increased metabolic demands. We sought to determine factors associated with decreased VO(2) max in pediatric patients with different stages of CKD. VO(2) max was measured using a standardized exercise testing protocol in patients with stage 2 to 4 chronic kidney disease (CKD) (n = 46), in renal transplant recipients (n = 22), in patients treated with maintenance hemodialysis (n = 12), and in age-matched healthy controls (n = 33). VO(2) max was similar between children with stage 2 CKD and controls, whereas lower VO(2) max was observed among children with stage 3 to 4 CKD, those treated with hemodialysis, and transplant recipients. In univariate analysis, VO(2) max was significantly associated with body mass index, resting heart rate, C-reactive protein, serum triglycerides, serum creatinine, and measures of diastolic function; no significant associations with left ventricular structure or systolic function were identified. In multivariate regression analysis, patient category versus control and the presence of diastolic dysfunction were independent predictors of lower VO(2) max. These results suggest that aerobic capacity is decreased in the early stages of CKD in children and that lower VO(2) max can be predicted by the presence of diastolic dysfunction, even if systolic function is normal.

Cardiopulmonary Aerobic Fitness Assessment During Maximal and Submaximal Exercise Testing in Pediatric Oncology Patients After Chemotherapy.

PURPOSE: The cardiopulmonary exercise test (CPET) is a valuable tool to assess cardiopulmonary exercise capacity in pediatric oncology patients after chemotherapy. In addition, few studies on the utility of submaximal testing have been performed, which could be important as some patients are unable to complete a maximal effort test secondary to deconditioning by both disease and treatment. MATERIALS AND METHODS: We performed a retrospective chart review of pediatric cancer patients exposed to chemotherapy from 1992 to 2013 who underwent CPET with cycle ergometry (n=27). The study patients were compared with age-matched, sex-matched, and size-matched normal controls. The submaximal measure recorded was the oxygen consumption (VO2)@respiratory exchange ratio (RER) 1.0 during a maximal effort test. RESULTS: The chemotherapy group demonstrated significantly lower exercise time (9.2±3.6 vs. 11.4±3.8; P=0.008), total work capacity (4914.4±3290.3 vs. 7664.4±4289.5; P=0.004), systolic blood pressure at peak exercise (162.9±23.2 vs. 177.3±23.8; P=0.01), indexed peak VO2 (33.9±6.9 vs. 40.0±6.3; P=0.001), and indexed peak oxygen pulse (6.1±1.3 vs. 7.0±1.5; P=0.004)). For the submaximal outcome measured, 11/27 of the chemotherapy patients had VO2@RER 1.0 values <-2 SD from the mean compared with 0/27 control patients. CONCLUSIONS: Pediatric patients exposed to chemotherapy have impaired cardiopulmonary exercise capacity. The VO2@RER 1.0 in chemotherapy patients suggests that this may be a reliable submaximal measure in this population. IMPLICATIONS FOR CANCER SURVIVORS: This study demonstrates that the CPET can be used in pediatric cancer survivors with prior exposure to chemotherapy to demonstrate impaired cardiopulmonary exercise tolerance, which is demonstrated on submaximal and maximal effort testing.

Development of exercise-induced arm-leg blood pressure gradient and abnormal arterial compliance in patients with repaired coarctation of the aorta.

Often, the lack of systemic arterial hypertension and the lack of a resting arm-leg blood pressure gradient are used to assess the adequacy of the anatomic result after intervention for coarctation of the aorta (CoA). Some patients with no arm-leg gradient at rest may develop a gradient with exercise, leading caregivers to question the success of the repair. It is not clear what the prevalence is of patients who have undergone a successful intervention for CoA and have no arm-leg gradient at rest but develop a significant gradient with exercise and which factors may predict the development of an arm-leg gradient with exercise. This study evaluates the prevalence and predictors of an exercise-induced arm-leg gradient in subjects who have undergone an apparently successful intervention for CoA.

Cardiovascular physiology during supine cycle ergometry and dobutamine stress.

PURPOSE: This study compared cardiac hemodynamics during supine cycle ergometry and dobutamine stress. METHODS: Thirty-two healthy volunteers (19 female, 13 male, 23.5 +/- 3.5 yr old) completed respective tests on separate days and in random order. Heart rate, blood pressure, and cardiac output were recorded at baseline and peak stress. Echocardiographic measures included left ventricular end-diastolic dimension, fractional shortening, heart rate corrected velocity of circumferential fiber shortening, end-systolic wall stress, and the difference between measured and predicted fiber shortening for measured wall stress. RESULTS: Compared with peak exercise, dobutamine infusion resulted in lower cardiac output (12 +/- 2 vs 16 +/- 4 l x min(-1), P < 0.0001), heart rates (163 +/- 7 vs 175 +/- 12 beats x min(-1), P < 0.0001), and systolic blood pressure (160 +/- 22 vs 185 +/- 20 mm Hg, P < or = 0.0001). Echocardiography demonstrated smaller left ventricular end-diastolic dimension (4.2 +/- 0.7 vs 4.5 +/- 0.7 cm, P = 0.013), higher fractional shortening (0.55 +/- 0.07 vs 0.50 +/- 0.06%, P < 0.001), higher VCFc (2.07 +/- 0.36 vs 1.54 +/- 0.20 circs x s(-1), P < 0.001) higher VCFdiff (0.94 +/- 0.35 vs 0.48 +/- 0.20 circs x s(-1), P < 0.001), and lower end-systolic wall stress (25 +/- 11 vs 42 +/- 16 g x cm(-2), P < 0.001). The stress-velocity relationship during dobutamine demonstrated higher y-intercept and steeper slope, indicating greater load-independent contractility. CONCLUSION: The cardiovascular adaptation to exercise and dobutamine stress differ significantly. Cardiac output during peak exercise is greater than during peak dobutamine secondary to increased heart rate and stroke volume. Despite a greater increase in contractility and decrease in afterload, a smaller increase in cardiac output during dobutamine stress may be secondary to limited ventricular preload.

Pediatric Exercise Stress Laryngoscopy following Laryngotracheoplasty: A Comparative Review.

OBJECTIVE: Exercise-induced airway obstruction in pediatric patients is a unique phenomenon with multiple potential etiologies. An accurate diagnosis can be challenging to establish in pediatric patients because they are frequently asymptomatic at rest. Exercise stress laryngoscopy (ESL) is a modality by which pediatric patients can be evaluated under physiologic conditions that produce their symptoms. The purpose of this study was to demonstrate (1) the diagnostic effectiveness of pediatric ESL and (2) the ability of ESL to guide treatment for "normal" and post-airway reconstruction patients with exercise intolerance. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: Patients undergoing ESL for exercise intolerance were reviewed. Demographics, surgical history, examination findings, and management recommendations were extracted. RESULTS: Thirty-seven patients (average age, 13.5 years; range, 5-21 years) were included. There were 14 male and 23 female patients. Airway abnormalities became evident in 56% of patients. Of these, 24% had focal supraglottic collapse, 43% had evidence of paradoxical vocal fold motion, 24% had combined supraglottic and glottic dysfunction, and 9% had distal airway abnormalities. Overall, 18 patients had changes in management after ESL. Twelve patients in this review had histories of laryngotracheoplasty with equivocal findings on operative bronchoscopy. Of these patients, 10 (83%) received focal diagnoses after ESL. CONCLUSION: ESL is a contemporary modality by which complex patients with undiagnosed exercise intolerance can be effectively evaluated. ESL can be an important tool used to guide treatment in pediatric patients with exercise-induced dyspnea after airway reconstruction.

Exercise capacity improves after transcatheter closure of the Fontan fenestration in children.

OBJECTIVES: This study evaluated the aerobic capacity, exercise capacity, and arterial oxygen saturation (O(2)Sat) in children before and after transcatheter Fontan fenestration closure. DESIGN: Observational study comparing exercise parameters and hemodynamics before and after transcatheter fenestration closure in Fontan patients. OUTCOME MEASURES: Working capacity, exercise duration, oxygen consumption (VO(2)), and arterial O(2)Sat were evaluated during aerobic exercise. RESULTS: Twenty patients (mean age 11.4 years) underwent standardized exercise testing before and after fenestration closure. Twelve patients underwent cycle ergometry testing (mean age 14.8 years) (group 1), and eight younger patients (mean age 6.4 years) underwent Bruce treadmill testing (group 2). The same exercise protocol was used in each patient before and after fenestration closure (interval between tests: 118 +/- 142 days). Immediately following fenestration closure at cardiac catheterization, cardiac index decreased (3.0 to 2.1 L/minute/m(2)) and Fontan pressure increased (11 +/- 2 to 12 +/- 2 mm Hg) with an increased arterial saturation (92 to 96%) (P < .001). The total group demonstrated no significant change in pre- and postclosure maximal heart rates (164 +/- 21 and 169 +/- 19 bpm). Rest and exercise O(2)Sat increased (89 and 82 to 95 and 92%) (P < .0001). Exercise duration increased (7.7 +/- 1.9 to 9.2 +/- 2.4 minutes) (P < .0005). Maximal VO(2), indexed maximal VO(2), and total working capacity in kilopond-meters (kpm) increased (1.2 +/- 0.5, 27 +/- 7 and 2466 +/- 1012 to 1.3 +/- 0.4 L/minute, 31 +/- 9 mL/kg/minute and 2869 +/- 1051 kpm, respectively) (P < .005). CONCLUSION: In children with a univentricular heart after Fontan palliation, transcatheter fenestration closure improves exercise arterial O(2)Sat and aerobic capacity despite a restricted resting cardiac output documented by catheterization immediately after the closure procedure.

Exercise evaluation of upper- versus lower-extremity blood pressure gradients in pediatric and young-adult participants.

The aim of this study was to provide a normal reference for arm-leg blood pressure gradients in normal pediatric and young-adult patients before and after exercise. We assessed 216 normal participants by physical or echocardiographic exam, maximally tested using the James Cycle Protocol, with arm and leg blood pressures taken pre- and postexercise. Arm-leg gradients significantly increased from -5 mmHg at rest to 4, 2, and 1 mmHg 1, 3, and 4 min postexercise (p < .05). There was a small, statistically significant increase in arm-leg blood pressure with exercise, which is probably clinically insignificant. These data serve as a normal reference.