Patients with repaired Tetralogy of Fallot (rToF) typically report having preserved subjective exercise tolerance. Chronic pulmonary regurgitation (PR) with varying degrees of right ventricular (RV) dilation as assessed by cardiac magnetic resonance imaging (MRI) is prevalent in rToF and may contribute to clinical compromise. Cardiopulmonary exercise testing (CPET) provides an objective assessment of functional capacity, and the International Physical Activity Questionnaire (IPAQ) can provide additional data on physical activity (PA) achieved. Our aim was to assess the association between CPET values, IPAQ measures, and MRI parameters. All rToF patients who had both an MRI and CPET performed within one year between March 2019 and June 2021 were selected. Clinical data were extracted from electronic records (including demographic, surgical history, New York Heart Association (NYHA) functional class, QRS duration, arrhythmia, MRI parameters, and CPET data). PA level, based on the IPAQ, was assessed at the time of CPET. Eighty-four patients (22.8 ± 8.4 years) showed a reduction in exercise capacity (median peak VO₂ 30 mL/kg/min (range 25–33); median percent predicted peak VO₂ 68% (range 61–78)). Peak VO₂, correlated with biventricular stroke volumes (RVSV: β = 6.11 (95%CI, 2.38 to 9.85), p = 0.002; LVSV: β = 15.69 (95% CI 10.16 to 21.21), p < 0.0001) and LVEDVi (β = 8.74 (95%CI, 0.66 to 16.83), p = 0.04) on multivariate analysis adjusted for age, gender, and PA level. Other parameters which correlated with stroke volumes included oxygen uptake efficiency slope (OUES) (RVSV: β = 6.88 (95%CI, 1.93 to 11.84), p = 0.008; LVSV: β = 17.86 (95% CI 10.31 to 25.42), p < 0.0001) and peak O₂ pulse (RVSV: β = 0.03 (95%CI, 0.01 to 0.05), p = 0.007; LVSV: β = 0.08 (95% CI 0.05 to 0.11), p < 0.0001). On multivariate analysis adjusted for age and gender, PA level correlated significantly with peak VO₂/kg (β = 0.02, 95% CI 0.003 to 0.04; p = 0.019). We observed a reduction in objective exercise tolerance in rToF patients. Biventricular stroke volumes and LVEDVi were associated with peak VO₂ irrespective of RV size. OUES and peak O₂ pulse were also associated with biventricular stroke volumes. While PA level was associated with peak VO₂, the incremental value of this parameter should be the focus of future studies.

Background: Our aim was to comprehensively review published evidence on the association between having a congenital heart disease (CHD) compared with not, on educational attainment (i.e. not obtaining a university degree, completing secondary education, or completing any vocational training vs. obtaining/completing) in adults. Method: Studies were eligible if they reported the rate, odds, or proportion of level of educational attainment in adults by whether or not they had a CHD. Result: Out of 1537 articles screened, we identified 11 (N = 104,585 participants, 10,487 with CHD), 10 (N = 167,470 participants, 11,820 with CHD), and 8 (N = 150,813 participants, 9817 with CHD) studies reporting information on university education, secondary education, and vocational training, respectively in both CHD and non-CHD participants. Compared to their non-CHD peers, CHD patients were more likely not to obtain a university degree (OR = 1.38, 95% CI [1.16, 1.65]), complete secondary education (OR = 1.33, 95% CI [1.09, 1.61]) or vocational training (OR = 1.11, 95% CI [0.98, 1.26]). For all three outcomes there was evidence of between study heterogeneity, with geographical area contributing to this heterogeneity. Conclusion: This systematic review identified all available published data on educational attainment in CHD patients. Despite broad inclusion criteria we identified relatively few studies that included a comparison group from the same population, and amongst those that did, few adjusted for key confounders. Pooled analyses suggest evidence of lower levels of educational attainment in patients with CHD when compared to non-CHD peers. The extent to which this may be explained by confounding factors, such as parental education, or mediated by treatments is not possible to discern from the current research literature.

Background: Advances in the management of congenital heart disease (CHD) patients have enabled improvement in long-term survival even for those with serious defects. Research priorities (for patients, families and clinicians) have shifted from a focus on how to improve survival to exploring long-term outcomes in patients with CHD. A comprehensive appraisal of available evidence could inform best practice to maximize health and well-being, and identify research gaps to direct further research toward patient and clinical need. We aimed to critically appraise all available published systematic reviews of health and well-being outcomes in adult patients with CHD.Methods: We conducted an umbrella review, including any systematic reviews that assessed the association of having vs. not having CHD with any long-term health (physical or mental), social (e.g., education, occupation) or well-being [e.g., quality of life (QoL)] outcome in adulthood (≥18-years).Results: Out of 1330 articles screened, we identified five systematic reviews of associations of CHD with adult outcomes. All but one (which studied QoL) explored health outcomes: one cardiovascular, two mental, and one mortality after transplant. CHD patients had a higher risk of stroke, coronary heart disease and heart failure, with the pooled relative risk (RR) for any outcome of 3.12 (95% CI: 3.01 to 3.24), with substantial heterogeneity (I² = 99%) explained by the outcome being studied (stronger association for heart failure) and geography (stronger in Europe compared with other regions). CHD patients had a higher risk of anxiety (OR = 2.58 (1.45 to 4.59)], and higher mean scores for depression/anxiety symptoms (difference in means = −0.11 SD (–0.28 to 0.06), I² = 94%)]. Compared with patients having a cardiac transplant for other (non-CHD) diseases, CHD patients had higher short-term mortality (RR at 30-days post-transplant = 2.18 [1.62 to 2.93)], with moderate heterogeneity (I² = 41%) explained by previous surgery (higher mortality with prior Fontan/Glenn operation). All domains of QoL were lower in patients with Fontan’s circulation than non-CHD adults.Conclusion: Adults with CHD have poorer cardiovascular, mental health and QoL outcomes, and higher short-term mortality after transplant. The paucity of systematic reviews, in particular for outcomes such as education, occupation and lifestyles, highlights the need for this to be made a priority by funders and researchers.Systematic Review Registration: [www.crd.york.ac.uk/prospero], identifier [CRD42020175034].

Background Following the repair of TOF patients may be left with pulmonary regurgitation and a dilated right ventricle (RV), which in turn can lead to ventricular arrhythmias and sudden death. A prolonged QRS is a predictor of ventricular arrhythmias. However, whether subsequent pulmonary valve replacement (PVR) can reverse QRS‐prolongation is controversial. We hypothesized that changes in QRS duration following PVR are determined by preoperative QRS‐duration and RV volumes Methods A retrospective single‐center cohort study was conducted on 142 post‐TOF repair patients (mean age 25 ± 13 years) who underwent PVR between 1995 and 2019. Information on QRS duration and RV volumes measured by cardiac MRI (available in 83 patients) were collected. A linear mixed model was used to investigate the association between the preoperative QRS duration and RV volumes and the postoperative QRS duration. Results The QRS‐duration following PVR continued to increase in all subjects with a prolonged preoperative QRS‐duration(>160 ms, rate of increase of 0.87 msec ± 0.33 per year; p = .01), markedly raised RV end‐diastolic volume (RVEDV; ≥166 ml/m2, rate of increase of 2.0 msec ± 0.37 per year; p < .01) or RV end‐systolic volume (RVESV; ≥89 ml/m², rate of increase of 1.25 msec ± 0.43 per year; p = .01). In contrast, in patients with preoperative QRS‐duration <160 msec (p = .16), RVEDV <166 ml/m² (p = .14), or RVESV < 89 ml/m2 (p = .37), the QRS‐duration did not change significantly when compared to preoperative values. Conclusions In subjects with shorter QRS and smaller RV volumes, QRS duration did not show further prolongation following PVR. While markedly prolonged QRS and increased RV volumes were associated with a small but constant increase in QRS duration despite PVR.

Objective Independent temporal external validation of the improving partial risk adjustment in surgery model (PRAIS-2) to predict 30-day mortality in patients undergoing paediatric cardiac surgery. Design Retrospective analysis of prospectively collected data. Setting Paediatric cardiac surgery. Intervention PRAIS-2 validation was carried out using a two temporally different single centre (Bristol, UK) cohorts: Cohort 1 surgery undertaken from April 2004 to March 2009 and Cohort 2 from April 2015 to July 2019. For each subject PRAIS-2 score was calculated according to the original formula. Participants A total of 1352 (2004-2009) and 1197 (2015-2019) paediatric cardiac surgical procedures were included in the Cohort 1 and Cohort 2, respectively (median age at the procedure 6.3 and 7.1 months). Primary and secondary outcome measures PRAIS-2 performance was assessed in terms of discrimination by means of ROC (receiver operating characteristic) curve analysis and calibration by using the calibration belt method. Results PRAIS-2 score showed excellent discrimination for both cohorts (AUC 0.72 (95%CI: 0.65 to 0.80) and 0.88 (95%CI: 0.82 to 0.93), respectively). While PRAIS-2 was only marginally calibrated in Cohort 1, with a tendency to underestimate risk in lowrisk and overestimate risk in high risk procedures (P-value = 0.033), validation in Cohort 2 showed good calibration with the 95% confidence belt containing the bisector for predicted mortality (P-value = 0.143). We also observed good prediction accuracy in the non-elective procedures (N = 483;AUC 0.78 (95%CI 0.68 to 0.87); Calibration belt containing the bisector (P-value=0.589). Conclusions In a single centre UK-based cohort, PRAIS-2 showed excellent discrimination and calibration in predicting 30-day mortality in paediatric cardiac surgery including in those undergoing non-elective procedures. Our results support a wider adoption of PRAIS-2 score in the clinical practice.

Background: To determine the association between having a CHD compared with not, on educational attainment in adults. A systematic review and meta-analysisMethod: Studies were eligible if they reported the rate/odds/proportion of level of educational attainment in adults by history of CHD. Result: Out of 1537 articles screened, we identified 11 (N = 104585 participants, 10487 with CHD), 10(N = 167470 participants, 11820 with CHD), and 8 (N = 150813 participants, 9817 with CHD) studies reporting information on university education, secondary education, and vocational training, respectively in both CHD and non-CHD participants. Compared to their non-CHD peers, CHD patients were more likely not to obtain a university degree (OR=1.38, 95% CI [1.16, 1.65]), complete secondary education (OR=1.33, 95 % CI [1.09, 1.61]) or vocational training (OR= 1.11, 95%CI [0.98, 1.26]). For all three outcomes there was evidence of between study heterogeneity, with geographical area contributing to this heterogeneity. Conclusion: This systematic review identified all available published data on educational attainment in CHD patients. Despite broad inclusion criteria we identified relatively few studies that included a comparison group from the same population, and amongst those that did, few adjusted for key confounders. Pooled analyses suggest evidence of lower levels of educational attainment in patients with CHD when compared to non-CHD peers. The extent to which this may be explained by confounding factors, such as parental education, or mediated by treatments is not possible to discern from the current research literature.