OBJECTIVE: (1) Identify and review current policies for the cardiovascular screening of athletes to assess their applicability to the paediatric population and (2) evaluate the quality of these policy documents using the Appraisal of Guidelines for Research & Evaluation II (AGREE II) tool. DESIGN: Systematic review and quality appraisal of policy documents. DATA SOURCES: a systematic search of PubMed, MEDLINE, Scopus, Web of Science, SportDiscus and CINAHL. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: an article was included if it was a policy/position statement/guideline/consensus or recommendation paper relating to athletes and cardiovascular preparticipation screening. RESULTS AND SUMMARY: of the 1630 articles screened, 13 met the inclusion criteria. Relevance to paediatric athletes was found to be high in 3 (23%), moderate in 6 (46%) and low in 4 (31%), and only 2 provide tailored guidance for the athlete aged 12-18 years. A median 5 related citations per policy investigated solely paediatric athletes, with study designs most commonly being retrospective (72%). AGREEII overall quality scores ranged from 25% to 92%, with a median of 75%. The lowest scoring domains were rigour of development; (median 32%) stakeholder involvement (median 47%) and Applicability (median 52%). CONCLUSION: Cardiac screening policies for athletes predominantly focus on adults, with few providing specific recommendations for paediatric athletes. The overall quality of the policies was moderate, with more recent documents scoring higher. Future research is needed in paediatric athletes to inform and develop cardiac screening guidelines, to improve the cardiac care of youth athletes.

Background and Objectives: Drug-related bradyarrhythmia is a well-documented major adverse event among beta-blocker users and a potential cause for hospitalization or additional inter-ventions. Whether beta-blocker use is associated with specific bradyarrhythmia presentations, and how this relates to other predisposing factors, is not well known. We aim to evaluate the association between beta-blocker use and the type of atrioventricular (AV) conduction disorder in patients with symptomatic bradycardia. Materials and Methods: We conducted a retrospective cohort study on 596 patients with a primary diagnosis of symptomatic bradyarrhythmia admitted to a single tertiary referral center. of the cases analyzed, 253 patients were on beta-blocker treatment at presentation and 343 had no bradycardic treatment. We analyzed demographics, clinical and paraclinical parameters in relation to the identified AV conduction disorder. A multivariate regression analysis was performed to explore factors associated with beta-blocker use. Results: of the 596 patients (mean age 73.9 ± 8.8 years, 49.2% male), 261 (43.8%) had a third-degree AV block, 92 (15.4%) had a second-degree AV block, 128 (21.5%) had slow atrial fibrillation, 93 (15.6%) had sick sinus syndrome and 21 (3.5%) had sinus bradycardia/sinus pauses. Beta-blocker use was associated with the female gender (p < 0.001), emergency admission (p. 120 ms (p = 0.02). Slow atrial fibrillation (OR = 4.2, p

Athlete preparticipation screening focuses on preventing sudden cardiac death (SCD) by detecting diseases such as arrhythmogenic ventricular cardiomyopathy (AVC), which affects primarily the right ventricular myocardium. Diagnosis may be obscured by physiological remodeling of the athlete heart. Healthy athletes may meet the 2010 Task Force Criteria right ventricular outflow tract (RVOT) dimension cut-offs, questioning the suitability of the modified Task Force Criteria (mTFC) in adolescent athletes. In this study, 67 male adolescent footballers undergoing preparticipation screening were reviewed. All athletes underwent a screening for resting ECG and echocardiogram according to the English FA protocol, as well as cardiopulmonary exercise testing, stress ECG, and exercise echocardiography. Athletes' right ventricular outflow tract (RVOT) that met the major AVC diagnostic criteria for dilatation were identified. of 67 evaluated athletes, 7 had RVOT dilatation that met the major criteria, all in the long axis parasternal view measurement. All had normal right ventricular systolic function, including normal free-wall longitudinal strain (ranging from - 21.5 to - 32.7%). Left ventricular ejection fraction ranged from 52 to 67%, without evidence of structural changes. Resting ECGs and cardiopulmonary exercise tests were normal in all individuals. In a series of healthy athletes meeting the major AVC diagnostic criteria for RVOT dilatation, none had any other pathological changes on a detailed screening including ECG, exercise testing, and echocardiography. This report highlights that current AVC echocardiographic diagnosis criteria have limitations in this population.

OBJECTIVE: Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A meta-analysis of LMCo cases could fill the knowledge gaps in this topic. METHODS: Electronic databases were searched for all LMCo/PAH studies, abstracts and case reports including pulmonary artery (PA) size. Restricted maximum likelihood meta-analysis was used to evaluate LMCo-associated factors. Specificity, sensitivity and accuracy of PA size thresholds for diagnosis of LMCo were calculated. Treatment options and outcomes were summarized. RESULTS: a total of five case-control cohorts and 64 case reports/series (196 LMCo and 438 controls) were included. LMCo cases had higher PA diameter (Hedge's g 1.46 [1.09; 1.82]), PA/aorta ratio (Hedge's g 1.1 [0.64; 1.55]) and probability of CHD (log odds-ratio 1.22 [0.54; 1.9]) compared to non-LMCo, but not PA pressure or vascular resistance. A 40 mm cut-off for the PA diameter had balanced sensitivity (80.5%), specificity (79%) and accuracy (79.7%) for LMCo diagnosis, while a value of 44 mm had higher accuracy (81.7%), higher specificity (91.5%) but lower sensitivity (71.9%). Pooled mortality after non-conservative treatment (n = 150, predominantly stenting) was 2.7% at up to 22 months of mean follow-up, with 83% survivors having no angina at follow-up. CONCLUSION: PA diameter, PA/aorta ratio and CHD are associated with LMCo, while hemodynamic parameters are not. Data from this study support that a PA diameter cut-off between 40 and 44 mm can offer optimal accuracy for LMCo screening. Preferred treatment was coronary stenting, associated with low mid-term mortality and symptom relief. Diagnosis and management of left main coronary artery compression (LMCo) in patients with pulmonary arterial hypertension (PAH).

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.

Background. Percutaneous coronary intervention (PCI) of unprotected left main coronary artery disease (ULMCAD) have become a feasible and efficient alternative to coronary artery bypass surgery, especially in patients with acute coronary syndrome (ACS). There are limited data regarding early and late outcomes after ULMCAD PCI in patients with ACS and stable angina.The aim of this study was to compare early and four-year clinical outcomes in patients with ULMCAD PCI presenting as ACS or stable angina in a high-volume PCI center.Methods. We conducted a single center retrospective observational study, which included 146 patients with ULMCAD undergoing PCI between 2014 and 2018. Patients were divided in two groups: Group a included patients with stable angina (n = 70, 47.9%) and Group B patients with ACS (n = 76, 52.1%).Results. 30-day mortality was 8.22% overall, lower in Group a (1.43% vs 14.47%, p = 0.02). Mortality and major adverse cardiac events (MACE) rates at 4 years were significantly lower in Group a (9.64% vs 33.25%, p = 0.001, and 24.06% vs 40.11%, p = 0.012, respectively). Target lesion revascularization (TLR) at 4 year did not differ between groups (15% in Group a vs 12.76% in Group B, p = 0.5).Conclusions. In our study patients with ULMCAD and ACS undergoing PCI had higher early and long-term mortality and MACE rates compared to patients with stable angina, with similar TLR rate at 4-year follow-up.

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.

OBJECTIVES: Many adults with repaired tetralogy of Fallot will require a pulmonary valve replacement (PVR), but there is no consensus on the best timing. In this study, we aim to evaluate the impact of age at PVR on outcomes. METHODS: This is a national multicentre retrospective study including all patients >15 years of age with repaired tetralogy of Fallot who underwent their first PVR between 2000 and 2013. The optimal age cut-off was identified using Cox regression and classification and regression tree analysis. RESULTS: a total of 707 patients were included, median age 26 (15-72) years. The mortality rate at 10 years after PVR was 4.2%, and the second PVR rate of 6.8%. Age at PVR of 35 years was identified as the optimal cut-off in relation to late mortality. Patients above 35 years of age had a 5.6 fold risk of death at 10 years compared with those with PVR under 35 years (10.4% vs 1.3%, P < 0.001), more concomitant tricuspid valve repair/replacement (15.1% vs 5.7%, P < 0.001) and surgical arrhythmia treatment (18.4% vs 5.9%, P < 0.001). In those under 50 years, there was an 8.7 fold risk of late death compared with the general population, higher for those with PVR after 35 than those with PVR below 35 years (hazard ratio 9.9 vs 7.4). CONCLUSIONS: Patients above 35 years of age with repaired tetralogy of Fallot have significantly worse mortality after PVR, compared with younger patients and a higher burden of mortality relative to the general population. This suggests that there are still cases where the timing of initial PVR is not optimal, warranting a re-evaluation of criteria for intervention.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: 'In [patients undergoing Fontan palliation] does [fenestration] affect [early and late postoperative outcomes]?' Altogether 509 papers were found using the reported search, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Current data suggest that the use of fenestration has advantages in the immediate postoperative course, with fewer complications such as pleural effusions, shorter hospitalization and decreased early Fontan failure, but comparable long-term outcomes to a non-fenestrated approach. Fenestration should be used in high-risk patients or based on the haemodynamic parametersmeasured before weaning from cardiopulmonary bypass. Routine use may potentially lead to additional late fenestration closure procedures in some patients, without improving long-term outcomes.

In the originally published version of this article the Funding section is incomplete. The complete Funding section is reproduced below and has also been corrected in the online version of the full article. Funding Dr Dorobantu was supported by a Medical Research Council (MRC) doctoral studentship [grant number MR/N0137941/1 for the GW4 BIOMED DTP, awarded to the Universities of Bath, Bristol, Cardiff and Exeter from the MRC/UKRI]. The study was supported by the NIHR through the Biomedical Research Centre at University Hospitals Bristol NHS Foundation Trust, the University of Bristol, and the British Heart Foundation and used data provided by the National Institute for Cardiovascular Outcomes Research, as part of the National Congenital Heart Disease Audit (NCHDA). The NCHDA is commissioned by the Healthcare Quality Improvement Partnership (HQIP) as part of the National Clinical Audit and Patient Outcomes Programme (NCAPOP) and within the National Health Service, UK. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health and Social Care.

OBJECTIVES: Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making. METHODS: This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease. RESULTS: a total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day-65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients. CONCLUSIONS: AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes.

Aim: the chemotherapy protocol for acute lymphoblastic leukemia (ALL) uses low doses of anthracyclines (AC), generally associated with subclinical cardiotoxicity. The aim of our study was to evaluate the serum biomarkers and echocardiography parameters in children with ALL treated with AC in order to determine the most useful element for early detection of cardiotoxicity.Material and methods: in this prospective study, troponin I (TnI) and heart-type fatty acid binding protein (HFABP) were assessed five times during the first year after the onset of ALL. Serial Tissue Doppler Imaging and conventional cardiac echography were performed by two pediatric cardiologists (intraclass correlation coefficient over 0.85 for all measurements) in three periods during the study protocol.Results: We evaluated 48 children with ALL. TnI increased during therapy, without returning to baseline values one year after diagnosis. HFABP did not show significant changes during the study protocol. Left ventricle outflow tract time-velocity integral and peak systolic septal mitral annulus velocity decreased during chemotherapy and returned to baseline levels at one year after diagnosis, while peak systolic tricuspid annulus velocity and excursion, maintained a descending tendency. Early filling transmitral flow velocity and E/A ratio were also transiently influenced by chemotherapy.Conclusions: the study showed signs of transient cardiotoxicity in the left ventricle and diastolic parameters after chemotherapy, compared to right ventricle parameters which maintained low values even one year after diagnosis. TnI proved to be directly proportional to chemotherapy doses but HFABP was not useful in this setting

ObjectivesIn young and middle-aged adults, there are three current options for aortic valve replacement (AVR), namely mechanical AVR (mechAVR), tissue AVR (biological AVR) and the Ross operation, with no clear guidance on the best option. We aim to compare the clinical effectiveness and cost-effectiveness of the Ross procedure with conventional AVR in young and middle-aged adults.MethodsThis is a systematic literature review and meta-analysis of AVR options. Markov multistate model was adopted to compare cost-effectiveness. Lifetime costs, quality-adjusted life years (QALYs), net monetary benefit (NMB), population expected value of perfect information (EVPI) and expected value of partial perfect information were estimated.ResultsWe identified 48 cohorts with a total number of 12 975 patients (mean age 44.5 years, mean follow-up 7.1 years). Mortality, bleeding and thromboembolic events over the follow-up period were lowest after the Ross operation, compared with mechAVR and biological AVR (p&lt;0.001). Aortic reoperation rates were lower after Ross compared with biological AVR, but slightly higher when compared with mechAVR (p&lt;0.001). At a willingness-to-pay threshold of £20effective. At a willingness-to-pay threshold of £20, 000 per QALY000 per QALY, the Ross procedure is more cost-effective compared the Ross procedure is more cost-effective compared withwith conventional AVR, with a lifetime incremental NMB of £60 conventional AVR, with a lifetime incremental NMB of £60 952 (952 (££3030 236236 to to ££7979 464). Incremental costs were £12464). Incremental costs were £12 323 (323 (££61086108 to to ££1515 972) and incremental QALYs 3.66 (1.81972) and incremental QALYs 3.66 (1.81 to to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost--effective.At a willingness-to-pay threshold of £20 000 per QALY, the Ross procedure is more cost-effective compared with conventional AVR, with a lifetime incremental NMB of £60 952 (£30 236 to £79 464). Incremental costs were £12 323 (£6108 to £15 972) and incremental QALYs 3.66 (1.81 to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost-effective.ConclusionsIn young and middle-aged adults with aortic valve disease, the Ross procedure may confer greater quality of life and be more cost-effective than conventional AVR. A high-quality randomised trial could be warranted and cost-effective.

ObjectiveThere are conflicting data on choosing balloon aortic valvoplasty (BAV) or surgical aortic valvotomy (SAV) in neonates and infants requiring intervention for aortic valve stenosis. We aim to report the outcome of both techniques based on results from the UK national registry.MethodsThis is a retrospective study, including all patients under 1 year undergoing BAV/SAV between 2000 and 2012. A modulated renewal approach was used to examine the effect of reinterventions on outcomes.ResultsA total of 647 patients (488 BAV, 159 SAV, 292 neonates) undergoing 888 aortic valve procedures were included, with a median age of 40 days. Unadjusted survival at 10 years was 90.6% after initial BAV and 84.9% after initial SAV. Unadjusted aortic valve replacement (AVR) rate at 10 years was 78% after initial BAV and 80.3% after initial SAV. Initial BAV and SAV had comparable outcomes at 10 years when adjusted by covariates (p&gt;0.4). AVR rates were higher after BAV and SAV reinterventions compared with initial valvoplasty without reinterventions (reference BAV, HR=3 and 3.8, respectively, p&lt;0.001). Neonates accounted for 29/35 of early deaths after the initial procedure, without significant differences between BAV and SAV, with all late outcomes being worse compared with infants (p&lt;0.005).ConclusionsIn a group of consecutive neonates and infants, BAV and SAV had comparable survival and freedom from reintervention as initial procedures and when performed as reinterventions. These findings support a treatment choice based on patient characteristics and centre expertise, and further research into the best patient profile for each choice.

ObjectivesTreatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study.MethodsThis a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated.ResultsPatients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P&lt;0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09).ConclusionsWe found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.