Abstract

Congenital heart disease (ConHD) affectsapproximately 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 deterioratefaster compared to healthy people. CRF is known to be prognostic of future mortality and morbidity: itisthereforeimportant to assess the evidence base on physical activity interventions in this population to inform decision making. To assess the effectiveness and safety of all types of physical activity interventions versusstandard carein individuals with congenital heart disease. We undertook asystematicsearch on23September 2019 of the following databases: CENTRAL,MEDLINE, Embase,CINAHL, AMED, BIOSIS Citation Index,Web of Science Core Collection, LILACS andDARE. We also searched ClinicalTrials.gov and wereviewed the reference lists of relevant systematic reviews. We includedrandomised controlled trials(RCT) that compared any type of physical activity intervention against a 'no physical activity' (usual care) control. We included all individualswith a diagnosis ofcongenital 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 whoextracted the data thenprocessed the included papers, assessed their risk of bias using RoB 2and assessed the certainty of the evidence using the GRADE approach.The primary outcomes were: maximal cardiorespiratory fitness (CRF) assessed bypeak oxygen consumption; health-related quality of life (HRQoL) determined by a validated questionnaire; and device-worn 'objective' measures of physical activity. We included 15RCTswith 924participantsin 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 ata high risk of bias (n=2), due to afailure to blindintervention 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 'noexercise' controlCRF may slightly increase, with amean difference (MD) of 1.89 mL/kg-1/min-1 (95% CI -0.22 to3.99; 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 threestudies 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 to0.92; n = 328; low-certainty evidence), which equates toapproximately an additional 10 minutes of physical activity daily (95% CI -2.50 to22.20). 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, sixstudies reported zeroadverse events. Fivestudies reported a total of 11 adverse events; 36% of adverse events were cardiac related(n= 4); there were, however, no serious adverse eventsrelated to the interventions or reportedfatalities (moderate-certainty evidence).No studies reported hospital admissions. This review summarises the latest evidence on CRF, HRQoL and PA. Although there were only small improvements in CRF and PA, and small to noimprovements in HRQoL, there were no reported seriousadverse events related to the interventions. Although these data are promising, there is currentlyinsufficient evidence to definitively determine the impact of physical activityinterventions in ConHD. Further high-quality randomised controlled trials are therefore needed, utilisinga longer duration of follow-up.

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