ObjectiveThe importance of aerobic fitness (VO2peak) in cystic fibrosis (CF) is well established, and regular exercise testing is recommended. To standardise VO2peak, a ‘percentage of predicted’ (%pred) derived from normative reference values (NRV), as promoted by the 2015 European Cystic Fibrosis Society Exercise Working Group (ECFS EWG), can be reported. However, the NRVs used in CF and their relative frequency is unknown.MethodA scoping review was performed via systematic database searches (PubMed, Embase, Web of Science, SciELO, EBSCO) and forward citation searches for studies that include people with CF and report VO2peakas %pred. Studies were screened using Covidence, and data related to patient demographics, testing modality and reference equations were extracted. Additional analyses were performed on studies published in 2016–2021, following the ECFS EWG statement in 2015.ResultsA total of 170 studies were identified, dating from 1984 to 2022, representing 6831 patients with CF, citing 34 NRV. Most studies (154/170) used cycle ergometry, 15/170 used treadmills, and the remainder used alternative, combination or undeclared modalities. In total, 61/170 failed to declare the NRV used. There were 61 studies published since the ECFS EWG statement, whereby 18/61 used the suggested NRV.ConclusionThere is a wide discrepancy in NRV used in the CF literature base to describe VO2peakas %pred, with few studies using NRV from the ECFS EWG statement. This high variance compromises the interpretation and comparison of studies while leaving them susceptible to misinterpretation and limiting replication. Standardisation and alignment of reporting of VO2peakvalues are urgently needed.

. Background
.
. Peak oxygen consumption (peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. ) is traditionally divided (“ratio‐scaled”) by body mass (BM) for clinical interpretation. Yet, it is unknown whether ratio‐scaling to BM can produce a valid size‐independent expression of peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. in people with a Fontan circulation. Furthermore, people with a Fontan circulation have deficits in lean mass, and it is unexplored whether using different measures of body composition may improve scaling validity. The objective was to assess the validity of different scaling denominators (BM, stature, body surface area, fat‐free mass, lean mass, and appendicular lean mass using ratio and allometric scaling).
.
.
.
. Methods and Results
.
. Eighty‐nine participants (age: 23.3±6.7 years; 53% female) with a Fontan circulation had their cardiorespiratory fitness and body composition measured by cardiopulmonary exercise testing and dual‐energy x‐ray absorptiometry. Ratio and allometric (log‐linear regression) scaling was performed and Pearson correlations assessed scaling validity. Scaling denominators BM (
. r
. =−0.25,
. P
. =0.02), stature (
. r
. =0.46,
. P
. <0.001), and body surface area (0.23,
. P
. =0.03) were significantly correlated with their respective ratio‐scaled expressions of peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. but fat‐free mass, lean mass, or appendicular lean mass were not (
. r
. ≤0.11;
. R
. 2
. =1%). Allometrically expressed peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. resulted in no significant correlation with any scaling denominator (
. r
. =≤0.23;
. R
. 2
. =≤4%).
.
.
.
. Conclusions
.
. The traditional and accepted method of ratio‐scaling to BM is invalid because it fails to create a size‐independent expression of peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. in people with a Fontan circulation. However, ratio‐scaling to measures of body composition (fat‐free mass, lean mass, and appendicular lean mass) and allometric techniques can produce size‐independent expressions of peak
.
.
.
.
. V
. ̇
.
.
. O
. 2
.
.
. $$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$
.
.
. in people with a Fontan circulation.
.
.

PURPOSE: Cerebral palsy (CP) is the commonest motor disability affecting children. This study reviewed the evidence for virtual reality (VR) intervention compared with conventional physiotherapy in upper limb function of children with CP. METHODS: Searches were undertaken in MEDLINE, EMBASE, PEDro, CENTRAL, Web of Science, CINAHL, ERIC, ICTRP, EU-CTR, ClinicalTrials.gov and EThOS databases. Only randomised-controlled trials (RCTs) were included. Two reviewers independently screened the search results, assessed full-text articles, extracted data and appraised the methodological quality by using the Cochrane collaboration's risk of bias (RoB2) tool. Albatross plots were used to synthesise the data. RESULTS: Seven RCTs, examining motor function in a total of 202 children with CP, included. Four trials used the Quality of Upper Extremity Skills Test (QUEST) as an outcome measure, and three trials used grip strength. These outcome measures were utilised to develop two Albatross plots. Data from the plots showed contradictory findings of the included studies. CONCLUSIONS: the effect of VR in the upper limb rehabilitation of children with CP remains unclear. All included studies used commercial non-immersive VR games. Future high-quality clinical research is needed to explore the extent to which non-immersive and immersive VR is feasible and effective with children and adolescents.IMPLICATIONS FOR REHABILITATIONThe current evidence supporting the use of VR as a rehabilitative tool is weak and uncertain.The current use of VR relies only on commercial non-immersive VR (off-shelf) games, which are not adjustable to meet the demands and goals of therapy programmes.Future research is needed to study the therapeutic feasibility of immersive VR with children and adolescents.

Exercise and physical activity (PA) have been shown to be effective, safe and feasible in both healthy children and children with congenital heart disease (CHD). However, implementing exercise training as an intervention is still not routine in children with CHD despite considerable evidence of health benefits and well-being. Understanding how children with CHD can safely participate in exercise can boost participation in PA and subsequently reduce inactivity-related diseases. Home-based exercise intervention, with the use of personal wearable activity trackers, and high-intensity interval training have been beneficial in adults’ cardiac rehabilitation programmes. However, these remain underutilised in paediatric care. Therefore, the aims of this narrative review were to synthesise prescribed exercise interventions in children with CHD, identify possible limitation to exercise training prescription and provide an overview on how to best integrate exercise intervention effectively for this population into daily practice.

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Included were dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM). A total of 14 cohorts were identified, of which six were in DCM, four in HCM, three in LVNC and one in ACM. The most commonly reported STE measurements were left ventricular longitudinal strain (Sl), circumferential strain (Sc), radial strain (Sr) and rotation/torsion/twist. Sl, Sc and were abnormal in all DCM and LVNC cohorts, but not in all HCM. Apical rotation and twist/torsion were increased in HCM, and decreased in LVNC. Abnormal STE parameters were reported even in cohorts with normal non-STE systolic/diastolic measurements. STE in childhood cardiomyopathies can detect early changes which may not be associated with changes in cardiac function detectable by non-STE methods. Longitudinal and circumferential strain should be introduced in the cardiomyopathy echocardiography protocol, reflecting current practice in adults.

BACKGROUND: Congenital heart disease (ConHD) affects approximately 1% of all live births. People with ConHD are living longer due to improved medical intervention and are at risk of developing non-communicable diseases. Cardiorespiratory fitness (CRF) is reduced in people with ConHD, who deteriorate faster compared to healthy people. CRF is known to be prognostic of future mortality and morbidity: it is therefore important to assess the evidence base on physical activity interventions in this population to inform decision making. OBJECTIVES: to assess the effectiveness and safety of all types of physical activity interventions versus standard care in individuals with congenital heart disease. SEARCH METHODS: We undertook a systematic search on 23 September 2019 of the following databases: CENTRAL, MEDLINE, Embase, CINAHL, AMED, BIOSIS Citation Index, Web of Science Core Collection, LILACS and DARE. We also searched ClinicalTrials.gov and we reviewed the reference lists of relevant systematic reviews. SELECTION CRITERIA: We included randomised controlled trials (RCT) that compared any type of physical activity intervention against a 'no physical activity' (usual care) control. We included all individuals with a diagnosis of congenital heart disease, regardless of age or previous medical interventions.  DATA COLLECTION AND ANALYSIS: Two review authors (CAW and CW) independently screened all the identified references for inclusion. We retrieved and read all full papers; and we contacted study authors if we needed any further information. The same two independent reviewers who extracted the data then processed the included papers, assessed their risk of bias using RoB 2 and assessed the certainty of the evidence using the GRADE approach. The primary outcomes were: maximal cardiorespiratory fitness (CRF) assessed by peak oxygen consumption; health-related quality of life (HRQoL) determined by a validated questionnaire; and device-worn 'objective' measures of physical activity. MAIN RESULTS: We included 15 RCTs with 924 participants in the review. The median intervention length/follow-up length was 12 weeks (12 to 26 interquartile range (IQR)). There were five RCTs of children and adolescents (n = 500) and 10 adult RCTs (n = 424). We identified three types of intervention: physical activity promotion; exercise training; and inspiratory muscle training. We assessed the risk of bias of results for CRF as either being of some concern (n = 12) or at a high risk of bias (n = 2), due to a failure to blind intervention staff. One study did not report this outcome. Using the GRADE method, we assessed the certainty of evidence as moderate to very low across measured outcomes. When we pooled all types of interventions (physical activity promotion, exercise training and inspiratory muscle training), compared to a 'no exercise' control CRF may slightly increase, with a mean difference (MD) of 1.89 mL/kg-1/min-1 (95% CI -0.22 to 3.99; n = 732; moderate-certainty evidence). The evidence is very uncertain about the effect of physical activity and exercise interventions on HRQoL. There was a standardised mean difference (SMD) of 0.76 (95% CI -0.13 to 1.65; n = 163; very low certainty evidence) in HRQoL. However, we could pool only three studies in a meta-analysis, due to different ways of reporting. Only one study out of eight showed a positive effect on HRQoL. There may be a small improvement in mean daily physical activity (PA) (SMD 0.38, 95% CI -0.15 to 0.92; n = 328; low-certainty evidence), which equates to approximately an additional 10 minutes of physical activity daily (95% CI -2.50 to 22.20). Physical activity and exercise interventions likely result in an increase in submaximal cardiorespiratory fitness (MD 2.05, 95% CI 0.05 to 4.05; n = 179; moderate-certainty evidence). Physical activity and exercise interventions likely increase muscular strength (MD 17.13, 95% CI 3.45 to 30.81; n = 18; moderate-certainty evidence). Eleven studies (n = 501) reported on the outcome of adverse events (73% of total studies). of the 11 studies, six studies reported zero adverse events. Five studies reported a total of 11 adverse events; 36% of adverse events were cardiac related (n = 4); there were, however, no serious adverse events related to the interventions or reported fatalities (moderate-certainty evidence). No studies reported hospital admissions. AUTHORS' CONCLUSIONS: This review summarises the latest evidence on CRF, HRQoL and PA. Although there were only small improvements in CRF and PA, and small to no improvements in HRQoL, there were no reported serious adverse events related to the interventions. Although these data are promising, there is currently insufficient evidence to definitively determine the impact of physical activity interventions in ConHD. Further high-quality randomised controlled trials are therefore needed, utilising a longer duration of follow-up.

Abstract
Background
Numerous studies have measured the prognostic associations between cardiorespiratory fitness and patient outcomes in congenital heart disease, but no systematic review has assessed these associations for all types of congenital heart disease. It is therefore a timely opportunity to syntheses all available data using a systematic review methodology. The aim of this study is to detail the protocol for a systematic review and meta-analysis.

Objectives
Within this paper we have developed a protocol for a prognostic factors systematic review and meta-analysis, to assess the role of cardiopulmonary exercise testing/cardiorespiratory fitness, in the prognosis of mortality and morbidity in congenital heart disease.

Methods
We have outlined, in detail, the process for this systematic review using the latest accepted methodological guidelines for prognostic factors research, such as the PICOTS system, CHARMS-PF data extraction, QUIPS risk of bias assessments and the prognostic GRADE guidelines (see list of abbreviations).

Conclusion
The implications of this review will aid future treatments, interventions and individual patient risk prediction. The publication of this protocol aims to improve scientific rigour by ensuring transparency in the systematic review and meta-analysis process.

This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: to assess the effectiveness and safety of physical activity promotion and exercise training interventions in individuals with congenital heart disease.