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Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group.We aimed to analyze outcomes of patients with SRV implanted with a CRT device.Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade.A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS).In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle.

Background Management of pregnancy and risk stratification in women with congenital heart diseases (CHD) are challenging especially due to physiological haemodynamic modifications that inevitably occur during pregnancy. Objective To compare the accuracy of the main published scores including CARPREG II score in prediction of maternal complications during pregnancy in CHD patients. Method We included all pregnant women with CHD who delivered their babies after the 20th gestational week in our institution between 2007 and 2018 until 6 months postpartum. Pregnancy scores (CARPREG, CARPREG II, Harris, ZAHARA risk scores and modified WHO (mWHO) risk classification) were applied retrospectively. Results Of 121 pregnancies in 65 CHD patients, 30% had cardiovascular complications. The c-statistic was 0.577 (95% confidence interval (CI): 0.428, 0.727; P = 0.19) for CAPREG score, 0.593 (95% CI: 0.469, 0.717; P = 0.11) for the ZAHARA score, 0.601 (95% CI: 0.402, 0.799; P = 0.15) for Harris score, 0.646 (95% CI: 0.525, 0.767; P Fig. 1 ). Conclusion Even if the new CARPREG II score seems to be better than most of the previously used scores as predictor of adverse maternal cardiovascular events mWHO appears to be more accurate in CHD patients.

International audience; Advances in heart surgery over the past 30 years have significantly improved the prognosis of congenital heart diseases (CHD). Therefore, the epidemiology of CHD has changed dramatically with a shift of mortality from pediatrics to adulthood and an increased prevalence of complex CHD. Today, caregivers and patients focus their interests to new perspectives: improving the quality of life, practicing sports, improving psychosocial care. Cardiac rehabilitation is completely integrated in these new therapeutic strategies. The starting point is the cardiopulmonary exercise test (CPET), with the measurement of oxygen uptake, or "VO2". CPET is now recommended in the follow-up of the adults with CHD. Maximum oxygen uptake correlates to the quality of life of children and adults with CHD. The principles of the rehabilitation in patients with heart failure may usually be applied to CHD patients. Some studies in complex CHD showed improvement of VO2 and quality of life after rehabilitation, without any adverse events. However few physicians have the experience in rehabilitation among CHD patients, especially children. Randomized trials on cardiac rehabilitation in adult and pediatric CHD patients are essential to increase the level of evidence and lead to specific guidelines in this population.

Background In the past decade, the adult population with a congenital heart disease (CHD) has overpassed in numbers the paediatric population. Adolescents with CHD frequently interrupt their follow-up soon after transfer to adult cardiology. In 2014, three tertiary care CHD referral centres developed a common regional structured education transition program dedicated to adolescents and young adults with CHD. This study aimed to determine the factors that influenced the inclusion in this transition program. Methods Prospective controlled multicentre study carried out from November 2015 to January 2017 in southern France. Patients with any CHD aged 13 to 25 y were offered to participate in the program. During their annual check-up, they filled-out the same questionnaires. A multiple linear regression identified the explanatory factors for participation in the program. Results A total of 123 patients (mean age 19.6 ± 3.4 y) were included in the study, with 57 participants (group A) and 66 non-participants (group B). Both groups presented similar socio-demographic and quality of life characteristics, a low level of physical activity with muscular deconditioning and a high exposure to risk behaviours (71% patients with ≥ 1 risk factor). Patients with complex CHDs (OR:4.1, P = 0.03), patients with a low level of knowledge of their disease (OR:0.3, P = 0.02), patients with risk behaviours (piercing, OR:5.53, P = 0.01; alcohol, OR:3.12, P = 0.06), and adolescents Conclusions Many factors influencing the participation of adolescents and young adults with a CHD in a transition education program are controllable.

Background Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction, occurring in the second decade. The prevention of cardiomyopathy stands as one of the most challenging clinical research issues in children with DMD. Speckle tracking echocardiography (STE) is emerging as a functional biomarker to consider in the early detection of DMD-related cardiomyopathy. Methods Prospective controlled study in DMD children with normal or mildly altered left ventricle ejection fraction (LVEF ≥ 45%), compared with healthy age- and gender-matched controls. Left ventricle (LV) and right ventricle (RV) conventional echocardiography was performed, along with STE analyses in LV longitudinal, radial, and circumferential and RV free wall longitudinal displacements. The effect of age on STE evolution in DMD children was evaluated. Results The study included 108 boys, of which 36 who had DMD (mean age 11 ± 3.8 y) and 72 were age- and gender-matched control subjects. Conventional echocardiographic measures were normal in both groups and for both ventricles. LVEF ranged from 45% to 76% (mean 63% ± 6%) in the DMD group and from 55% to 76% (mean 64% ± 5%) in the control group. Global LV strain mean measures were significantly lower in the DMD group for the longitudinal (−16.8% ± 3.9% vs. −20.6% ± 2.6%, P Fig. 1 ). The LV longitudinal strain decrease with age in DMD children was 0.34% per year more marked than that of the controls ( Fig. 2 ). The LV inferolateral and anterolateral basal segments were specifically impaired. RV function with conventional and ST echocardiography was normal and not significantly different between DMD and controls. Conclusions The existence of an altered LV strain despite normal or subnormal LVEF in children with DMD represents an important perspective for future drug trials in DMD-related cardiomyopathy prevention. Clinical Trial Registration Clinicaltrials.gov NCT02418338 .

Objective We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) to that of age- and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2max) in this population. Methods We included in a cross-sectional multicentre study a total of 798 children (496 CHD and 302 controls) who underwent a complete cardiopulmonary exercise test (CPET). The association of clinical characteristics with VO2max was studied using a multivariate analysis. The study was approved by the South Mediterranean IV Ethics Committee (2009-A00423-54) and registered on ClinicalTrials.gov ( NCT01202916 ). Informed consent was obtained from all parents. Results Mean VO2max in the CHD group and control represented 93 ± 20% and 107 ± 17% of predicted values, respectively. VO2max was significantly lower in the CHD group, overall (37.8 ± 0.3 vs. 42.6 ± 0.4 mL/kg/min, P Table 1 ). The mean VO2max decline per year was significantly higher in CHD than in the controls overall (−0.84 ± 0.10 vs −0.19 ± 0.14 mL/kg/min/year, P Fig. 1 ). VO2max was associated with body mass index, ventilatory anaerobic threshold, female gender, restrictive ventilatory disorder, right ventricle systolic hypertension, tricuspid regurgitation, the number of cardiac catheter or surgery procedures, and the presence of a genetic anomaly. Conclusions This comparative CPET study provided, for the first time, relevant values of VO2max and their clinical determinants in the largest reported cohort of CHD children compared to a control group. Although the magnitude of the difference was not large, VO2max among children with CHD was significantly lower than in normal children. We suggest performing CPET in routine follow-up of these patients.

Purpose We aimed to compare, in a large cohort of normal children, ST analyses for right and left ventricles (RV, LV), between the QLAB 10.0 (Philips Healthcare) and the EchoPac 110.1.1 (General Electric Healthcare) ultrasound software. Methods Normal children aged 0 to 18 years referred to the outpatient pediatric cardiology consultation were screened for this prospective cross-sectional study (ClinicalTrials.gov: NCT02056925 ). First echocardiography was performed after randomization on one of both ultrasound systems. Second echocardiography was performed with equal randomization upon 4 levels of variability: same ultrasound (intra-ultrasound variability), different ultrasound (inter-ultrasound variability), same sonographer (intra-observer variability) and different sonographer (inter-observer variability). Post-data ST analyses were performed twice by the same investigator (intra-analyser variability) and by a second investigator (inter-analyser variability). Results Hundred and fifty-six children were included. Mean age was 7.6 ± 5 years [1 month–16.8 years]. There was no significant difference between both ultrasound systems in terms of conventional echocardiography variables. We observed a high proportion of missing ST data for the RV (19 to 78%). Inter-ultrasound system correlation was poor for both global longitudinal ( Table 1 ) and circumferential LV strain, with an ICC of 0.34 (95% CI 0.06 to 0.57) and 0.12 (95% CI −0.18 to −0.40), respectively. We observed a very poor inter-observer reliability in both global LV longitudinal strain and global LV circumferential strain with the two software. Inter-analyzer variability was better especially for the global LV circumferential strain using Philips software (ICC 0.78 (95% CI 0.52 to 0.91). Conclusion This study pointed out a serious limitation in the use of ST analysis in pediatrics with the two main ultrasound software, despite the EACVI/ASE Industry Task Force Standardization Initiatives for Deformation Imaging.

Backgroundpeak oxygen uptake (VO2) correlates with health related quality of life (QoL) in adults with heart failure. Cardio-pulmonary exercise test (CPET) is recommended in the follow-up of adults with congenital heart diseases (CHD). Few data is available as regards correlation between CPET and QoL among children with CHD.Methods and results202 CHD children aged 8 to 18 performed a CPET (treadmill n=96, cycle-ergometer n=106). CHD severity was stratified into 4 groups. All children and parents filled out the Kidscreen QoL questionnaire. Peak VO2, anaerobic threshold (AT), oxygen pulse followed a downward significant trend with increasing CHD severity and conversely for VE/VCO2 slope. Self-reported and parent-reported physical well-being QoL scores correlated with peak VO2 (respectively r=0.27, p

Objectifs Les scores de QDV sont correles a la VO2max chez l’adulte insuffisant cardiaque. Une telle correlation n’a pas ete etablie en cardiopediatrie. Materiels et methodes Deux cent deux enfants belges et francais porteurs d’une cardiopathie congenitale (CC) âges de 8 a 18 ans ont effectue une VO2 (tapis roulant n = 96, cycle ergometre n = 106). La severite de la CC a ete stratifiee en 4 groupes. Tous les enfants et les parents ont rempli le Kidscreen, questionnaire generique de QdV. Les parents ont signe un consentement eclaire (ClinicalTrials.gov # NCT01202916 ). Resultats VO2max, seuil ventilatoire, pente VE/VCO2 et pouls d’oxygene etaient correles a la severite de la CC. Le bien-etre physique etait correle a VO2max (enfants : r = 0,27, p Conclusions VO2max et seuil ventilatoire sont correles a la qualite de vie des enfants porteurs de CC dans la dimension physique. Dans un essai clinique cardiopediatrique, nous suggerons d’utiliser les scores physiques estimes par les parents comme critere de jugement.