Subjective fitness relates to performance and can be improved by exercise in children and young adults with heart disease.

Introduction: The Duke Activity Status Index is used to assess an individual patient's perception of their fitness abilities. It has been validated and shown to predict actual fitness in adults but has been studied less in the paediatric population, specifically those with heart disease. This study aims to assess if the Duke Activity Status Index is associated with measured markers of physical fitness in adolescents and young adults with heart disease.

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.

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.

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.

Isosorbide DiNitrate Effect on Hemodynamic Profile, Liver Stiffness, and Exercise Tolerance in Fontan Circulation (The NEET Clinical Trial).

After Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation.

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 bioimpedance analysis performed within 12 months before and during the COVID-19 pandemic. Formal activity restriction was noted as present or absent.

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.

Pediatric youth who have obesity have high rates of adult criminal behavior and low rates of homeownership.

Objectives: The social outcomes in adulthood for pediatric patients with obesity are not well-described. This study investigated lifetime criminal behavior and homeownership in youth with obesity.

Methods: Retrospective data on all children enrolled in the weight management program from 1999 to 2009 and who completed exercise testing were collected.

Arrhythmia Burden and Heart Rate Response During Exercise in Anderson-Fabry Disease.

Patients with Anderson-Fabry disease (AFD) have an elevated incidence of resting arrhythmias and ischemic heart disease, but their exercise arrhythmia burden and ischemic changes are not well understood. In addition, little research has been done on heart rate recovery in these patients. We retrospectively reviewed charts of patients with AFD who underwent maximal effort cardiopulmonary exercise testing (CPET) (n=44; 38.

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.

Practices and Procedures in Clinical Pediatric Exercise Laboratories in North America.

Unlabelled: Interinstitutional differences in clinical pediatric exercise laboratory (CPEL) practices may affect patient care and efficacy of multicenter research.

Purpose: To describe current practices/procedures in CPELs and explore differences in CPELs employing exercise physiologists to those that do not.

Methods: A 40-item survey was distributed to CPELs in North America focusing on (1) staffing; (2) exercise stress testing (EST) volumes, reporting, and interpretation; and (3) EST procedures/protocols.

Abnormal Maximal and Submaximal Cardiopulmonary Exercise Capacity in Pediatric Stem Cell Transplant Recipients Despite Normal Standard Echocardiographic Parameters: A Pilot Study.

Left ventricular systolic dysfunction is a known complication of stem cell transplantation (SCT). There has been minimal research to determine whether subclinical cardiac dysfunction exists in SCT patients using tools other than standard echocardiography, such as maximal and submaximal effort cardiopulmonary exercise testing (CPET) and vascular function studies. The objective of this study was to determine the rate of subclinical cardiac dysfunction in patients with normal ejection fraction after SCT, identified by abnormal values by CPET, tissue-Doppler imaging, and arterial stiffness measurements and to further describe submaximal exercise test measures 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.

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.

Peripheral venous pressure changes during exercise are associated with adverse Fontan outcomes.

Objective: Elevated central venous pressure (CVP) plays an important role in the development of adverse Fontan outcomes. Peripheral venous pressure (PVP) has been validated as a surrogate for CVP in Fontan patients. We hypothesised that PVP in response to exercise will be associated with a greater prevalence of Fontan morbidity.

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.

Probenecid Improves Cardiac Function in Subjects with a Fontan Circulation and Augments Cardiomyocyte Calcium Homeostasis.

Subjects with functionally univentricular circulation who have completed staged single ventricle palliation, with the final stage culminating in the Fontan procedure, are often living into adulthood. However, high morbidity and mortality remain prevalent in these patients, as diastolic and systolic dysfunction of the single systemic ventricle are linked to Fontan circulatory failure. We presently investigated the effects of probenecid in post-Fontan patients.

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.

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.

The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity.

Background: Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan).

Methods: We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children's Hospital Medical Center from 2013 to 2018.

Functional Capacity Is Affected by Younger Age of Repair in Tetralogy of Fallot Patients But Not by Era of Repair.

Background: Cardiopulmonary exercise testing is widely used to assess functional capacity in patients with tetralogy of Fallot after surgical repair. Little is known regarding the impact of age at the time of primary complete repair and surgical era effect on exercise capacity.

Methods: A retrospective, single-center chart review was done from 1976 to 2016 on pediatric patients with tetralogy of Fallot who underwent cardiopulmonary exercise testing with cycle ergometry utilizing the James protocol and were then sorted by repair before/after 1990 and by age of complete repair before/after one year.

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.

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.

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.