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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.

Purpose-Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Two dimensional-speckle tracking echocardiography (2D-STE) has recently emerged as a non-invasive functional biomarker for early detection of DMD-related cardiomyopathy. This study aimed to determine, in DMD children, the existence of a left ventricle (LV) dyssynchrony using 2D-STE analysis.Methods-This prospective controlled study enrolled 25 boys with DMD (mean age 11.0±3.5 years) with normal LV ejection fraction and 50 age-matched controls. Three measures were performed to assess LV mechanical dyssynchrony: the opposing-wall delays (longitudinal and radial analyses), the modified Yu index, and the time-to-peak delays of each segment. Feasibility and reproducibility of 2D-STE dyssynchrony were evaluated. Results-All three mechanical dyssynchrony criteria were significantly higher in the DMD group than in healthy subjects: (1) opposing-wall delays in basal inferoseptal to basal anterolateral segments (61.4±45.3 msec vs. 18.3±50.4 msec, Prespectively) and in mid inferoseptal to mid anterolateral segments (58.6±35.3 msec vs. 42.4±36.4 msec, Prespectively), (2) modified Yu index (33.3±10.1 msec vs. 28.5±8.1 msec, P, respectively), and (3) most of time-to-peak values, especially in basal and mid anterolateral segments. Feasibility was excellent and reliability was moderate to excellent, with ICC values ranging from 0.49 to 0.97.Conclusion-Detection of LV mechanical dyssynchrony using 2D-STE analysis is an easily and reproducible method in pediatrics. The existence of an early LV mechanical dyssynchrony visualized using 2D-STE analysis in children with DMD before the onset of cardiomyopathy represents a perspective for future pediatric drug trials in the DMD-related cardiomyopathy prevention. Clinical Trial Registration-Clinicaltrials.gov NCT02418338. Post-hoc study, registered on April 16, 2015.

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. Aims To compare the accuracy of the existing pregnancy cardiovascular risk scores in prediction of maternal complications during pregnancy in CHD patients. Method and results From 2007 to 2018, all pregnant women with a CHD who delivered birth after 20 weeks of gestation were identified. The discriminating power and the accuracy of the five existing pregnancy cardiovascular risk scores [CARPREG, CARPREG II, HARRIS, ZAHARA risk scores, and modified WHO (mWHO)] were evaluated. Out of 104 pregnancies in 65 CHD patients, 29% experienced cardiovascular complications during pregnancy or post-partum. For the five scores, the observed rate of cardiovascular events was higher than the expected risk. The values of area under the ROC curve were 0.75 (0.62–0.88) for mWHO, 0.65 (0.53–0.77) for CARPREG II, 0.60 (0.40–0.80) for HARRIS, 0.59 (0.47–0.72) for ZAHARA, and 0.58 (0.43–0.73) for CARPREG. Conclusion The modified WHO classification appeared to better predict cardiovascular outcome in pregnant women with CHD than the four other existing risk scores. Clinical Trial Registration: Clinicaltrials.gov: NCT04221048.

Backgrounds Systemic right ventricle (RV) is a rare and complex form of congenital heart disease (CHD) with a prognosis related to RV dysfunction and impaired physical capacity. Routine follow-up relies on echocardiography, however the prognostic value of echocardiography parameters remains under debate. Real-life patient follow-up involves different ultrasound systems. We aimed to evaluate echocardiography parameters’ reliability in systemic RV, in terms of reproducibility, using vendor-independent software, and in terms prediction of physical capacity impairment. Methods Adult patients with D-transposition of the great artery (d-TGA) who underwent atrial switch or with congenitally-corrected TGA (cc-TGA) were included in this multicentre prospective study. Current echocardiography parameters were analysed using TomTec-Arena™ software. Intraclass correlation coefficients (ICC) assessed inter- and intraobserver reliability. Associations between the most reproducible echocardiography parameters and exercise capacity (peak VO2, VE/VCO2 slope) were explored. Results A total of 47 patients were included in the study (87% d-TGA, median age 36.4 ± 8 years). Conventional and 2D strain echocardiography parameters indicated the existence of a RV dysfunction (TAPSE = 12.8 ± 3.1 mm; RV free wall longitudinal 2D strain = −13.6 ± 3.9%). Good reproducibility (ICC > 0.75) for both intra and interobserver variability was observed in 8 RV echocardiography parameters. Only the TAPSE was significantly associated with peak VO2 (r = 0.4, P = 0.02). Conclusions In this prospective study mimicking real-life echocardiography follow-up of systemic RV, TAPSE, RV free wall longitudinal 2D strain and peak systolic S wave, were the most reproducible echocardiography parameters. However, only the TAPSE was associated with peak VO2.

Background: Recent advances in the field of congenital heart disease (CHD) led to an improved prognosis of the patients and in consequence the growth of a new population: the grown up with congenital heart disease. Until recently, more than 50% of these patients were lost to follow up because of the lack of specialized structures. The critical moment is the transition between paediatric and adult unit. Therapeutic education is crucial to solve this issue by helping patient to become independent and responsible. The TRANSITION randomized trial aims to assess the impact of a transition education program on the quality of life (QoL) of adolescents and young adults with CHD. Methods: multicentre, randomised, controlled, parallel arm study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (Education program vs. no intervention). The primary outcome is the change in self-reported QoL between baseline and 12-month follow-up. A total of 100 patients in each group is required to observe a significant increase of the overall QoL score of 7±13.5 points (on 100) with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VO2max,VE/VCO2 slope), level of knowledge, physical and psychological status.Discussion: As the current research is opening on patient related outcomes, and as the level of proof in therapeutic education still low, we sought to assess the efficacy of a therapeutic education program on the QoL of CHD patients with a randomized trial.Trial registration: This study was approved by the National Ethics Committee (South-Mediterranean IV 2016-A01681-50) and was registered on Clinicaltrials.gov (NCT03005626) on 29th December 2016

BackgroundRecent advances in the field of congenital heart disease (CHD) led to an improved prognosis of the patients and in consequence the growth of a new population: the grown up with congenital heart disease. Until recently, more than 50% of these patients were lost to follow up because of the lack of specialized structures. The critical moment is the transition between paediatric and adult unit. Therapeutic education is crucial to solve this issue by helping patients to become independent and responsible. The TRANSITION-CHD randomized trial aims to assess the impact of a transition education program on health-related quality of life (HRQoL) of adolescents and young adults with CHD.MethodsMulticentre, randomised, controlled, parallel arm study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (education program vs. no intervention). The primary outcome is the change in self-reported HRQoL between baseline and 12-month follow-up. A total of 100 patients in each group is required to observe a significant increase of the overall HRQoL score of 7 ± 13.5 points (on 100) with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (peak VO2, VAT, VE/VCO2 slope), level of knowledge of the disease using the Leuven knowledge questionnaire for CHD, physical and psychological status.DiscussionAs the current research is opening on patient related outcomes, and as the level of proof in therapeutic education is still low, we sought to assess the efficacy of a therapeutic education program on HRQoL of CHD patients with a randomized trial.Trial registrationThis study was approved by the National Ethics Committee (South-Mediterranean IV 2016-A01681-50) and was registered on Clinicaltrials.gov (NCT03005626).

Highlights • Exercise capacity in patients with CHD is lower than in the general population. • Non-invasive relaxation therapy may be effective in patients with dyspnoea. • Evidence based-medicine on relaxation therapy in the CHD population is poor. • This trial will assess the impact of Sophrology on exercise capacity in CHD patients., Background Recent advances in the field of congenital heart disease (CHD) have significantly improved the overall prognosis. Now more attention is being given to health-related quality of life (HRQoL) and promotion of physical activity. Non-invasive relaxation therapy may be effective in cardiac patients concerned with exercise-induced dyspnoea. The SOPHROCARE randomised trial aims to assess the impact of Caycedian Sophrology on cardiopulmonary fitness in adolescents and young adults with CHD. Methods The SOPHROCARE trial is a nationwide, multicentre, randomised, controlled study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (8 Sophrology group sessions vs. no intervention). The primary outcome is the change in percent predicted maximum oxygen uptake (VO2max) between baseline and 12-month follow-up. A total of 94 patients in each group is required to observe a significant increase of 10% in VO2max with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VE/VCO2 slope, ventilatory anaerobic threshold, oxygen pulse, respiratory response to hypercapnia), health-related quality of life score (PedsQL), physical and psychological status. Conclusion After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. We sought to assess in the SOPHROCARE trial, if a Sophrology program, could improve exercise capacity and quality of life in youth with CHD. Trial registration Clinicaltrials.gov (NCT03999320).

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.

Aims The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality. Methods and results This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P

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.

Speckle tracking echocardiography (STE) has become a useful tool in cardiology but remains scarcely developed in pediatrics. We aimed to evaluate the feasibility of STE analyses in healthy children and compare reliability of STE for left and right ventricles (LV, RV) between the EchoPAC (GE Healthcare) and the QLAB (Philips Healthcare) software systems. Healthy children were screened for this prospective cross-sectional study. Analyses were performed upon five levels of variability: intra/inter-ultrasound system, intra/inter-sonographer and intra/inter-analyzer. The feasibility was measured, and the tracking quality informed. The study included 156 healthy children. Mean age was 7.6 ± 5 years [1 month-16.8 years]. Conventional echocardiography variables were similar in both ultrasound systems. For both software brands, the tracking quality was excellent in the LV longitudinal and circumferential displacements, but more limited in the RV free wall longitudinal strain. Inter-ultrasound system correlation was poor for global longitudinal and circumferential LV strain (ICC of 0.34 [IC95% 0.06-0.57]) and 0.12 [IC95% - 0.18 to - 0.40], respectively). We observed poor inter-sonographer reliability for both global LV longitudinal strain and global LV circumferential strain with the two software systems. Inter-analyzer variability was good especially for the global LV circumferential strain using Philips software (ICC of 0.78 [IC95% 0.52-0.91]). In pediatrics, the Philips/GE inter-vendor level of variability in STE analysis is mainly due to inter ultrasound systems and inter sonographers' differences. These results need to be taken into account when using STE analysis in the follow-up of cardiac children. Clinicaltrials.gov: NCT02056925.

BACKGROUND: Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Speckle-tracking echocardiographic (STE) imaging is emerging as a noninvasive functional biomarker to consider in the early detection of DMD-related cardiomyopathy. However, STE analysis has not been assessed in a prospectively controlled study, especially in presymptomatic children with DMD, and no study has used STE analysis in all three displacements (longitudinal, radial, and circumferential) and for both ventricles. METHODS: This prospective controlled study enrolled 108 boys, 36 of whom had DMD (mean age, 11 ± 3.8 years) and 72 of whom were age-matched control subjects in a 1:2 case-control design. Conventional echocardiographic variables were collected for the left and right ventricles. STE analyses were performed in the longitudinal, radial, and circumferential displacements for the left ventricle and in the free wall longitudinal displacement for the right ventricle. The effect of age on the evolution of two-dimensional strain in children with DMD was studied by adding an interaction term, DMD × age, in the models. RESULTS: Conventional echocardiographic measures were normal in both groups. Left ventricular (LV) ejection fraction 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 worse in the DMD group for the longitudinal (-16.8 ± 3.9% vs -20.6 ± 2.6%, P < .0001), radial (22.7 ± 11.3% vs 31.7 ± 14%, P = .002), and circumferential (-16.5 ± 3.8% vs -20.3 ± 3.1%, P < .0001) displacements. The decrease of global LV longitudinal strain with age in children with DMD was 0.34% per year more marked than that in control subjects. The LV inferolateral and anterolateral segments were specifically impaired, especially in the basal area. Right ventricular function evaluated using conventional echocardiography and STE analysis was normal and not different between children with DMD and control subjects. CONCLUSIONS: The existence of altered LV strain despite normal LV function in children with DMD represents an important perspective for future pediatric drug trials in DMD-related cardiomyopathy prevention.

International audience; Background: Systemic right ventricle (RV) is a rare and complex congenital heart disease (CHD). Patients with a systemic RV present with a significant decrease of their exercise capacity. We aimed at identifying clinical and paraclinical factors associated with maximum oxygen uptake (VO2max) in adults with a systemic RV.Methods: This multicentre cross-sectional study was performed in 2017 in three French tertiary care CHD centres. Adult patients with a D-transposition of the great artery (d-TGA) or a congenitally corrected TGA (cc-TGA) were included. Demographic, clinical, laboratory and imaging data were collected. Univariate and multivariate analyses were performed to identify predictors of impaired VO2max, as measured by cardiopulmonary exercise test (CPET).Results: A total of 111 patients were included in the study (85% d-TGA, median age 37.2 ± 8.2 years). Most patients presented with impaired physical capacity (mean VO2max of 23.3 ± 6.9 ml/kg/min, representing 68.4 ± 16.6% of predicted values) and ventilatory anaerobic threshold (VAT) impaired (mean VAT of 32.7 ± 10.9% of the predicted values). In univariate analysis, VO2max correlated with professional status, NYHA functional class, BNP level, the type of systemic RV, decreased RV function values in cardiac imaging, the severity of tricuspid regurgitation, the presence of a pacemaker or an implantable defibrillator, the VAT, the maximum load, and the maximal heart rate during exercise. In multivariate analysis, the VO2max remained associated with the NYHA functional class. The final multivariate model explained 49% of the variability of VO2max.Conclusion: NYHA functional class and RV function are predictors of impaired exercise capacity in adult patients with systemic RV.

Background Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to the severity of the ILD. The right ventricular to left ventricular diameter (RV:LV) ratio measured at CT pulmonary angiogram (CTPA) has been shown to provide valuable information in patients with pulmonary arterial hypertension and to predict death or deterioration in acute pulmonary embolism. Methods Demographic characteristics, ILD subtype, echocardiography, and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterization at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored according to CT criteria. The RV:LV ratio at CTPA was evaluated by using three different methods. Cox proportional hazards analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. Results A total of 92 patients were included (64% male; mean age 65 ± 11 years) with an FVC 57 ± 20% predicted, corrected transfer factor of the lung for carbon monoxide 22 ± 8% predicted, and corrected transfer coefficient of the lung for carbon monoxide 51 ± 17% predicted. PH was confirmed at right heart catheterization in 78%. Of all the CTPA-derived measures, an RV:LV ratio ≥ 1.0 strongly predicted mortality or transplantation at univariate analysis (hazard ratio, 3.26; 95% CI, 1.49-7.13; P = .003), whereas invasive hemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (hazard ratio, 3.19; 95% CI, 1.44-7.10; P = .004), adjusting for an ILD diagnosis of idiopathic pulmonary fibrosis and CT imaging-derived ILD severity. Conclusions An increased RV:LV ratio measured at CTPA provides a simple, noninvasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow-up, more aggressive treatment, and consideration of lung transplantation.

Objective Transition education programs dedicated to adolescents and young adults with congenital heart disease (CHD) aim to facilitate transfer to adult cardiology and bring more autonomy to teenagers. This prospective controlled multicentre study analysed the factors influencing the participation in a transition education program. Methods CHD patients aged 13–25 y were offered to participate in the transition program. 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 and 66 non-participants. Both groups showed similar socio-demographic and quality of life characteristics, low level of physical activity with muscular deconditioning and high exposure to risk behaviours (71% patients with ≥1 risk factor). Patients with complex CHD (OR = 4.1, P = 0.03), poor disease knowledge (OR = 0.3, P = 0.02), risk behaviours (body piercing, OR = 5.53, P = 0.01; alcohol, OR = 3.12, P = 0.06), and aged Conclusion Many risk factors influencing the participation of adolescents and young adults with CHD in transition education programs are controllable. Practice implication Further randomized studies are necessary to evaluate the impact of transition education program on quality of life, successful transfer to adult centre and, ultimately, prognosis.

Background Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods. Methods The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2max) Conclusion After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD. Trial registration: Clinicaltrials.gov ( NCT03690518 ).

The use of hypnosis in children has been described more than two centuries ago, with a more recent research and clinical application. There is currently a good level of evidence for the efficacy of clinical hypnosis in children for minor surgery, medical procedures or pain management. The use of clinical hypnosis, in paediatric cardiology, for invasive procedures such as transesophageal echocardiography, has not been reported.This study evaluated the feasibility of clinical hypnosis in children undergoing transesophageal echocardiography.This prospective, non-randomised, cross-sectional study was carried out over 24 months in a paediatric cardiology referral centre. All children aged 10-18 years requiring a transesophageal echocardiography examination, outside the operating room and the catheterisation laboratory, were eligible for the study. Children and families could choose between transesophageal echocardiography under clinical hypnosis or under general anaesthesia (15 years) or sedation (⩾15 years).We included 16 children aged 11-18 years (seven girls, mean age 14.1±2.5 years). The hypnotic state was achieved for 15 out of the 16 participating children (94%). The transesophageal echocardiography examination could be completely achieved with a full diagnosis for 15 out of 16 children (94%). In all cases, a transesophageal echocardiography examination under clinical hypnosis provided a complete diagnosis.This study demonstrated that hypnosis was feasible and effective for transesophageal echocardiography in adolescents and might be a good alternative to general anaesthesia. Further study with larger numbers of subjects and more diverse congenital cardiac conditions are needed to confirm the results in a more diverse sample.

Introduction Echocardiographic assessment of right ventricle (RV) function has become essential in pediatric cardiology. Objective The aim of our study was to determine the most appropriate mathematical model for RV function reference values in children. Method Normal children aged 0 to 18 years referred to the pediatric cardiology consultation were screened in this prospective cross-sectional study. Each RV parameters (S’, E’, A’ waves, TEI index, TAPSE) was modeled with four explanatory variables: age, size, weight and body surface area. Four different mathematical models were applied: a linear model, a polynomial model of degree 2, a linear regression model by spline with free knot, and a polynomial regression model of degree 2 by spline with a free knot. The choice of the best explanatory variable and modelization method was made on the basis of the model's coefficient of determination (R2). Results We included 314 normal children aged 2 days to 18 years (46% female, 88 infants, 26 neonates, 226 children). The modelization of the RV variables over all ages did not follow a linear model, with the lowest coefficients of determination in the four explanatory variables. The quadratic spline model best adjusted the modelization of all RV variables. No model accurately explained the TEI index in any of the four explanatory variables. The S’ wave was best modelized by the quadratic spline model with the weight. The knot at 6.86 kg corresponded to the curves’ break. The E’ wave was best modelized by the quadratic spline model with the age. The knot at 0.29 years corresponded to the curve's break. The A’ wave was best modelized by the quadratic model with the size. The knot at 81 cm corresponded to the break of the curve. The TAPSE was best modelized by the quadratic spline model with the weight. The knot at 9.04 kg corresponded to the curve's break. Conclusion The quadratic spline model best applies to echocardiographic RV reference values in pediatrics.

International audience; Several models have been used to establish pediatric Z-scores for echocardiographic right ventricle (RV) variables. This study aimed to determine the most appropriate mathematical model for RV function reference values in pediatrics. Prospective cross-sectional study among 314 healthy children ages 2 days to 18 years (46% female, 88 infants, 26 neonates, 226 children). RV parameters (S', E', A' waves, TEI index, TAPSE) were modelized with four explanatory variables: age, height, weight and body surface area. Four mathematical models were applied: a linear model, a polynomial model of degree 2, a linear regression model by spline with free knot, and a polynomial regression model of degree 2 by spline with a free knot. The choice of the best method used the adjusted coefficient of determination (aR2). The modelization of RV variables did not follow a linear model. A single explanatory variable could not determine all Z-scores, as specific and independent variables were required for each parameter. The quadratic spline model best adjusted the modelization of RV variables. The S' wave was best modelized by the spline model with the weight (knot at 6.86 kg). The E' wave was best modelized by the spline model with the age (knot at 0.29 years). The A' wave was best modelized by the model with the height (knot at 81 cm). The TAPSE was best modelized by the spline model with the weight (knot at 9.04 kg). The spline regression models best applies to echocardiographic RV reference values in pediatrics.

Nearly four decades ago, the World Health Organization stated that functional capacity explorations best reflected the impact of a chronic disease on quality of life. Today, cardio-pulmonary exercise test (CPET) is recommended in the follow-up of patients with congenital heart diseases (CHDs). Indeed, the maximum oxygen uptake (VO2max) and the ventilatory efficiency (VE/VCO2 slope) correlate with both the prognosis and the quality of life in this population. Atrial septal defects (ASDs) represent the second most frequent CHD and are usually considered as simple CHDs. However, the exercise capacity of ASD patients may be impaired. Therefore, the CPET provides important information in assessment and follow-up of patients with ASDs, for both children and adults. Exercise capacity of patients with unrepaired ASDs depends on the importance of the shunt, the right ventricular (RV) function and volume overload, the level of pulmonary arterial pressure, and the occurrence of arrhythmias. For repaired ASDs, exercise capacity also depends on the delay before closure and the type of procedure (catheter or surgery). In most cases, the exercise capacity is nearly normal and CPET contributes to promote sports participation. In addition, a regular CPET follow-up is necessary to evaluate the occurrence, severity and physiological mechanisms of comorbidities, i.e., heart failure, pulmonary hypertension and arrhythmia. Furthermore, CPET follow-up in patients with ASDs may detect early onset of muscular deconditioning, for which cardiac rehabilitation may be considered.

European Respiratory Society (ERS) guidelines recommend the assessment of patients with interstitial lung disease (ILD) and severe pulmonary hypertension (PH), as defined by a mean pulmonary artery pressure (mPAP) ≥35 mmHg at right heart catheterisation (RHC). We developed and validated a stepwise echocardiographic score to detect severe PH using the tricuspid regurgitant velocity and right atrial pressure (right ventricular systolic pressure (RVSP)) and additional echocardiographic signs. Consecutive ILD patients with suspected PH underwent RHC between 2005 and 2015. Receiver operating curve analysis tested the ability of components of the score to predict mPAP ≥35 mmHg, and a score devised using a stepwise approach. The score was tested in a contemporaneous validation cohort. The score used “additional PH signs” where RVSP was unavailable, using a bootstrapping technique. Within the derivation cohort (n=210), a score ≥7 predicted severe PH with 89% sensitivity, 71% specificity, positive predictive value 68% and negative predictive value 90%, with similar performance in the validation cohort (n=61) (area under the curve (AUC) 84.8% versus 83.1%, p=0.8). Although RVSP could be estimated in 92% of studies, reducing this to 60% maintained a fair accuracy (AUC 74.4%). This simple stepwise echocardiographic PH score can predict severe PH in patients with ILD., A stepwise echocardiographic score to predict severe group 3 pulmonary hypertension http://ow.ly/9cIC30iGSMj

Introduction: Pulmonary hypertension is a malignant prognostic factor in ILD. Prognostic tools are required to help prioritise lung transplant allocation and have not been validated in PH-ILD. We hypothesised that the composite physiological index (CPI) would reliably predict prognosis in PH-ILD, as it reflects both the morphological extent of pulmonary fibrosis and pulmonary vascular disease. Methods: Demographics, ILD subtype, pulmonary function tests, and echocardiograms were collected in consecutive patients undergoing right heart catheterisation (RHC) for suspected PH-ILD. Predictors of prognosis were evaluated in their ability to predict mortality with Cox proportional hazard modelling. Results: 228 ILD patients underwent evaluation between 2005-2015, age 61±11 years, 52% were male, and 79% had PH at RHC. Mean pulmonary pressure (mPAP) in the 168 patients with PH was 37±9.3mmHg, mean pulmonary vascular resistance (PVR) 7.0±3.8 Wood units, mean CPI was 61±13. In the patients with PH-ILD, CPI predicted mortality (Hazard ratio (HR): 1.042, Confidence interval (CI): 1.03-1.06, p Conclusion: The CPI strongly predicts mortality in ILD-PH.

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.

There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD.Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes.28 pregnancies in 20 women (26±6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks' gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred.Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.

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.