Double Ventricular Response with Aberrant Conduction Leading to Ventricular Dysfunction.

Double ventricular response (DVR), where a single P wave results in two QRS complexes, is a rare presentation of dual AV node physiology. It has been associated with ventricular dysfunction in the setting of incessant tachycardia. We present the case of an otherwise healthy adolescent who had frequent DVR without tachycardia leading to left ventricular dysfunction. Slow pathway modification led to a significant reduction in ectopy and normalization of ventricular function. This highlights that DVR without tachycardia might lead to ventricular dysfunction in pediatric patients. Slow pathway modification with reduction of ectopy may be sufficient to restore ventricular function.

(F)utility of preoperative pulmonary function testing in pectus excavatum to assess severity.

PURPOSE: The utility of pulmonary function testing (PFT) in pectus excavatum (PE) has been subject to debate. Although some evidence shows improvement from preoperative to postoperative values, the clinical significance is uncertain. A high failure-to-completion rate for operative PFT (48%) was identified in our large institutional cohort. With such a high non-completion rate, we questioned the overall utility of PFT in the preoperative assessment of PE and sought to evaluate if other measures of PE severity or cardiopulmonary function could explain this finding. METHODS: Demographics, clinical findings, and results from cardiac MRI, PFT (spirometry and plethysmography), and cardiopulmonary exercise tests (CPET) were reviewed in 270 patients with PE evaluated preoperatively between 2015 and 2018. Regression modeling was used to measure associations between PFT completion and cardiopulmonary function. RESULTS: There were no differences in demographics, symptoms, connective tissue disorders, or multiple indices of pectus severity and cardiac deformation in PFT completers versus non-completers. While regression analysis revealed higher RVEF, LVEF, and LVEF-Z scores, lower RV-ESV/BSA, LV-ESV/BSA, and LV-ESV/BSA-Z scores, and abnormal breathing reserve in PFT completers vs. non-completers, these findings were not consistent across continuous and binary analyses. CONCLUSIONS: We found that PFT completers were not significantly different from non-completers in most structural and functional measures of pectus deformity and cardiopulmonary function. Inability to complete PFT is not an indicator of pectus severity.

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.

Abdominal CT and MRI Findings of Portal Hypertension in Children and Adults with Fontan Circulation.

Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9-55 years; 32 children) were evaluated (median age, 23 years; IQR, 17-30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12-23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); median follow-up duration was 0.4 year (IQR, 0.1-3.1 years). Compared with patients who had Fontan circulation without deterioration, patients with Fontan deterioration were more likely to have moderate or severe ventricular systolic dysfunction (P < .01), moderate or severe atrioventricular valve regurgitation (P < .01), higher Fontan pressure (P = .01), radiologic evidence of portal hypertension (P < .01), and VAST score of 2 or greater (P < .01). Conclusion Radiologic evidence of portal hypertension at abdominal imaging in children and adults with Fontan circulation was associated with higher venous pressures and an increased risk for Fontan deterioration. These characteristics may be used to identify patients who warrant comprehensive hemodynamic evaluation. © RSNA, 2022.

EKG Abnormalities in a Youth Athlete Following COVID-19: It's Not Always Myocarditis!

COVID-19 associated myocarditis following mild infections is rare while incidental findings may be more common. A young athlete fully recovered from a mild COVID-19 infection presented with inferolateral T-wave inversions and left ventricular hypertrophy on imaging. Exercise testing aided in correctly diagnosing the patient with masked systolic hypertension.

Physical Frailty is Modifiable in Young Cardiac Rehabilitation Patients.

Frailty is a standardized, quantitative metric used to assess multisystem physiologic reserve and vulnerability to poor health outcomes. Cardiac rehabilitation (CR) positively impacts patient outcomes, including frailty, in adult cardiovascular disease (CVD); however, both the frailty paradigm and CR are understudied in pediatric CVD. This retrospective, single-center cohort study aimed to determine baseline composite frailty for pediatric-onset CVD patients and examine its change throughout CR using a proposed frailty assessment tool. Youth with pediatric-onset CVD participating in CR were stratified into five CVD diagnostic groups: post-heart transplant (HTx) (n = 34), post-ventricular assist device (VAD) (n = 12), single ventricle (n = 20) and biventricular (n = 29) congenital heart disease, and cardiomyopathy (n = 25), and frailty was assessed at baseline and every 30 days during CR. Post-HTx and post-VAD groups had significantly higher median frailty scores at baseline (6/10 and 5.75/10, respectively) driven by reduced strength, gait speed, and functional status. All groups except post-VAD displayed a significant absolute reduction in frailty from baseline to 120 days (HTx: - 3.5; VAD: - 3; SV CHD: - 1; BV CHD: - 1; CM: - 1.5), with similar median post-CR scores (1-3/10 in all groups). These improvements did not significantly correlate with number of CR sessions attended. This study established that frailty exhibits discriminatory utility across pediatric-onset CVD groups at baseline and is significantly modifiable over time. Improvements in frailty and other fitness metrics are likely due to a combination of post-operative recovery, post-diagnosis pharmacological and lifestyle changes, and CR. Further study of this frailty tool is needed to explore its prognostic utility.

Left Atrial Strain in the Repaired Tetralogy of Fallot Population: Comparisons to Biventricular Function, Native T1 Values, Exercise Parameters and Healthy Controls.

BACKGROUND: Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF. METHODS: Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19-32 years) were compared with 20 age-matched healthy volunteers (age range 19-34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations. RESULTS: Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p < 0.001 for each comparison). LA conduit:reservoir GLS and pump:reservoir GLS had correlations to native LV T1 (ρ = 0.26, p = 0.03 and ρ = - 0.26, p = 0.03, respectively). LA reservoir SR had positive correlation to RV EF (ρ = 0.27, p = 0.03). There were no statistically significant correlations between LA function and exercise capacity. CONCLUSIONS: LA function is altered in adolescent and young adult patients with rTOF indicating worse diastolic function and relates to increasing native LV T1 values. Future studies are indicated to investigate the progression of adverse atrial-ventricular interactions and poor outcomes in this population.

Dynamic exercise changes in venous pressure and liver stiffness in Fontan patients: effects of Treprostinil.

BACKGROUND: Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response. METHODS: In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise. RESULTS: Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times. CONCLUSIONS: Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.

Implementation of a Pediatric Chest Pain Local Consensus Guideline Decreases the Total Tests Performed Without Negatively Affecting the Yield of Abnormal Cardiac Results.

Pediatric chest pain is common and though usually benign often leads to unnecessary diagnostic testing. There is limited evidence as to whether a local consensus guideline can decrease testing frequency without negatively affecting the overall yield. In addition, it is unknown whether the addition of pulmonary function testing to a cardiopulmonary exercise test increases the diagnostic yield in pediatric patients with chest pain. A retrospective chart review was performed on all new pediatric patients who presented with chest pain at our academic center's pediatric cardiology clinic 18 months before and after the implementation of a standard management guideline. Data from the encounter-associated echocardiogram, cardiopulmonary exercise test, and pulmonary function test, when available, were analyzed. There were no significant differences in patient volume or demographic characteristics in the 18 months before (n = 768) and after (n = 778) guideline implementation. There were significant reductions in the number of ordered echocardiograms (n = 131; 17% vs. n = 75; 9.6%, p < 0.001) and cardiopulmonary exercise tests (n = 46; 6% vs. n = 29; 4%, p = 0.04) with no concerning pathology discovered in either group. Associated pulmonary function testing performed prior to with exercise testing discovered abnormalities in 19% of the total patients tested. The implementation of a local consensus guideline for pediatric chest pain results in fewer unnecessary tests ordered. There was no concerning pathology before or after guideline implementation, therefore conclusions regarding the diagnostic yield of these guidelines are unfeasible. The addition of pulmonary function testing to cardiopulmonary exercise tests increases the potential diagnostic yield in these patients.

Abnormal submaximal cardiopulmonary exercise parameters predict impaired peak exercise performance in sickle cell anemia patients.

PURPOSE: Sickle cell anemia (SCA) patients frequently have many comorbidities, including diastolic dysfunction (DD) and exercise intolerance. SCA patients often cannot reach maximal effort on exercise testing; little is known regarding whether submaximal exercise parameters can predict abnormal maximal exercise results in SCA patients and if there are any possible associations with DD. METHODS: A prospective longitudinal study was performed in SCA patients. All patients had a resting cardiac MRI (CMR), cardiopulmonary exercise test (CPET) with cycle ergometry using a ramp protocol, and an echocardiogram. Exercise data were compared with age-, gender-, and size-matched normal controls. RESULTS: Compared with normal controls, the SCA group (n = 19) had lower mean max oxygen consumption (VO2 ; 1378 ± 412 mL/min vs 2237 ± 580, P < 0.01) and workload (117 ± 37.6 watts vs 175 ± 50.5 watts, P = 0.0003). When evaluating the submaximal exercise parameters, there was lower VO2 at the anaerobic threshold (AT; 950 ± 311.7 vs 1460 ± 409.9, P < 0.01) and oxygen uptake efficiency slope (OUES) at AT (1512 ± 426.2 vs 2080 ± 339, P < 0.01). The max VO2 strongly correlated with VO2 at AT (r = 0.9, P < 0.01) and OUES (r = 0.83, P < 0.01) at AT. The VO2 at AT correlated with hematocrit (r = 0.77, P < 0.05). The OUES correlated with left ventricular ejection fraction by CMR (r = 0.55, P = 0.01), hematocrit (r = 0.52, P = 0.02), and lateral E/e' (r = -0.54, P = 0.01). CONCLUSIONS: SCA patients have abnormal submaximal exercise measures compared with controls, which is strongly associated with abnormal maximal exercise results. The degree of submaximal abnormality correlates with DD abnormalities by echocardiography. These data expand the scope of functional cardiovascular abnormalities in SCA.

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.

Cardiopulmonary fitness assessment on maximal and submaximal exercise testing in patients with Fabry disease.

The cardiopulmonary exercise test (CPET) is a valuable tool to assess a patient's aerobic fitness and cardiac function, including the response to stress. There have been few studies using CPET to evaluate cardiopulmonary exercise capacity in patients with Fabry disease. We performed a retrospective chart review of patients with Fabry disease from 2001 to 2016, compared to age, gender, and size-matched normal controls. A total of 18 patients were evaluated using the Bruce protocol (treadmill) and 11 patients were evaluated with the ramp protocol (cycle ergometer). The Fabry group demonstrated significantly lower heart rate at peak exercise (151.2 ± 22.5 vs. 178.6 ± 16.2, p < .05), max indexed VO2 (23.7 ± 7 vs. 33.9 ± 8.4, p < .05), and peak index oxygen pulse (12.1 ± 3 vs. 15.2 ± 4.2, p < .05). When the groups were further separated into treadmill or cycle ergometry testing only, there remained statistically significant differences in peak indexed oxygen pulse, heart rate at peak exercise, and max indexed VO2 . There was a statistically significant difference between the Fabry patients evaluated by treadmill testing for systolic blood pressure at peak exercise that was not seen in the cycle ergometry group. Additionally, when looking at the patients who had concurrent cardiac MRI (cMRI) with their CPET, there was a positive correlation with max indexed VO2 and right ventricular end-diastolic volume (r = .55, p = .007) and end-systolic volume (r = .59, p = .007). Patients with Fabry disease have impaired cardiopulmonary exercise capacity as measured by CPET. Additionally, in patients with Fabry disease there is a positive correlation with functional capacity and right ventricular volumes on cMRI.

Diastolic dysfunction is associated with exercise impairment in patients with sickle cell anemia.

BACKGROUND: Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood. OBJECTIVE: To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. METHODS AND RESULTS: As part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43 years). Maximum exercise was reached in 18 patients and 17 (94%) had reduced exercise capacity (%predicted VO2 less than 80%). Six patients had DD and none had systolic dysfunction. Patients with DD had lower exercise capacity compared to patients with normal diastolic function (%predicted VO2 48.2 ± 9.1% vs. 61.2 ± 11.7%; P = 0.01). The z-score of left ventricular lateral E/e' ratio, which is a marker of DD, was negatively associated with %predicted VO2 (r = -0.61, P = 0.01). All patients with moderate-to-severe exercise impairment (%predicted VO2  < 60%) had lateral E/e' z-score > 2. In a multivariate analysis, lateral E/e' z-score was independently associated with %predicted VO2 (P = 0.02). All participants had elevated ECV but the degree of elevation was not associated with exercise parameters. CONCLUSION: Left ventricular DD is associated with decreased exercise capacity in SCA. Interventions to prevent or delay DD could improve exercise capacity, quality of life, and long-term outcomes in SCA.

Incorrect ventricular lead placement into the systemic right ventricle of a patient with D-transposition of the great vessels after Mustard procedure.

Incorrect pacemaker lead placement into the systemic ventricle is a complication that has rarely been described in patients with D-transposition status after atrial baffle palliation. We present a case of ventricular lead misplacement in the systemic right ventricle of a patient with D-transposition of the great arteries after Mustard procedure. This case demonstrates the challenges with proper imaging of lead placement in patients with atrial baffles and long-term management of a lead in the systemic ventricle.

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.

Effects of a zero-gravity treadmill on body composition and cardiorespiratory fitness after Achilles surgery in a Masters runner.

Zero-gravity treadmills allow alterations in training load. Data are lacking on the utilization of this strategy to allow injured Masters-level athletes to return to activity and regain their fitness. A 6-week training program was designed for a 39-year-old male runner recovering from Achilles surgery using a zero-gravity treadmill. Three training sessions per week were performed with gradually increasing loads. Cardiopulmonary exercise testing and bioelectrical impedance analysis were performed before and after program completion. Following the training program, the athlete was able to return to full weight-bearing running. On cardiopulmonary exercise testing, there were improvements in peak oxygen consumption (42.9 vs 47.3 mL/min/kg; 118.6% vs 130.5% of predicted). On bioelectrical impedance analysis, there were small improvements in total weight, skeletal muscle mass, and adiposity felt to be within the standard of error for bioelectrical impedance analysis. In conclusion, load-altering exercise may be helpful for the Masters-level athlete recovering from Achilles tendon surgery.

Cardiac Tamponade Related to a Large Pericardial Mass in a Female with Juvenile Idiopathic Arthritis.

We report the case of a young female with juvenile idiopathic arthritis presenting with cardiac tamponade secondary to an unusual pericardial mass. Pericardial masses are typically incidental findings. In rare circumstances they can cause compressive physiology warranting urgent intervention. She required surgical excision which revealed a pericardial cyst encapsulating a chronic solidified hematoma. Though certain inflammatory disorders are associated with myopericarditis, to our knowledge this is the first reported case of a pericardial mass in a well-controlled young patient. We theorize her immunosuppressant therapy resulted in hemorrhage into a pre-existing pericardial cyst, suggesting the need for further follow-up in those on adalimumab therapy.

Long-term exercise and pulmonary function outcomes in a contemporary cohort of children with congenital diaphragmatic hernia.

OBJECTIVE: Congenital diaphragmatic hernia (CDH) survivors are at risk for long-term exercise impairment and pulmonary morbidity, but the generalizability of prior reported cohorts are limited by reduced disease severity and older surgical eras. We assessed the mid-childhood exercise and pulmonary function outcomes in a contemporary cohort of CDH survivors. STUDY DESIGN: In this retrospective cohort study, we identified 36 consecutive pediatric CDH survivors who underwent cardiopulmonary exercise testing (CPET) and spirometry from 2014 to 2021. Inferential statistics compared survivors with age-, sex-, and size-matched healthy controls; univariate analyses identified factors associated with abnormal testing. RESULTS: Maximal effort CPET and resting spirometry were completed by 27/36 (75%) and 31/36 (86%) subjects, respectively (median age: 8.1 years [interquartile range: 7.5, 10]; 16 females [44%]). Abnormal CPET (percent predicted [pp] peak VO2 < 80%) was more common in the CDH cohort (12 vs. 1, p < 0.001) and associated with longer neonatal intensive care unit (NICU) stay (p = 0.02) and oxygen therapy at discharge (p = 0.03). Exercise impairment was mild (pp peak VO2 70%-80%), moderate (60%-70%), and severe (<60%) in 6, 4, and 2 survivors, respectively. Abnormal spirometry was more common in the CDH cohort (21 vs. 3, p < 0.001; obstructive [n = 15], restrictive [n = 6]) and associated with decreased gestational age (p = 0.046), longer mechanical ventilation in the NICU (p = 0.02), and orthopedic abnormalities (p = 0.03). CONCLUSION(S): Exercise capacity was normal or mildly impaired in most CDH survivors, however, approximately two-thirds demonstrated abnormal spirometry. Impaired exercise capacity and lung function were associated with severity of lung disease postnatally. These data support cardiopulmonary follow-up throughout childhood.

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 low incidence of clinically significant heart disease in school-age children following COVID-19. / Baja incidencia de cardiopatía clínicamente significativa en niños en edad escolar tras padecer COVID-19.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3% of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings.

No es clara la carga de morbimortalidad de la lesión cardíaca clínicamente evidente secundaria a la enfermedad por coronavirus de 2019 (COVID-19) en los niños en edad escolar. A lo largo de 12 meses, en un importante hospital pediátrico académico en la región del medio oeste de Estados Unidos, hubo 1481 casos de COVID-19 sin hospitalización en niños en edad escolar por lo demás sanos, en quienes se hicieron 195 pruebas cardíacas. Si bien aparecieron hallazgos fortuitos, no se descubrió ninguna patología cardíaca relacionada con la COVID-19. Además, ~3 % de los niños solamente tuvieron síntomas cardíacos agudos que requirieron una evaluación por el área de cardiología pediátrica. Los niños que no fueron hospitalizados por COVID-19 tienen un riesgo muy bajo de desarrollar daño cardíaco clínicamente significativo y son más propensos a presentar hallazgos fortuitos.