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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.

Summary Background Managing oral anticoagulant therapy with vitamin K antagonists remains challenging in paediatric medicine. Aims This study aimed to assess the correlation between time in therapeutic range and quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. Methods Children aged from 2 to 18 years and receiving vitamin K antagonist therapy were eligible for this prospective multicentre study. Clinical and demographic data were collected. Health-related quality of life was assessed using the PedsQL™ 4.0 questionnaire. Correlations between quality of life scores and time in therapeutic range were measured. Results A total of 121 children were included in the study (mean age 9.6 ± 4.9 years). Cardiac conditions were the predominant indication for vitamin K antagonists. The mean time in therapeutic range was 0.78 ± 0.15 overall, and 0.76 ± 0.24 over the 3-month period before quality of life assessment. The mean total quality of life score was 76.2 ± 18 in self reports, 71.4 ± 22 in mother reports and 73.5 ± 19 in father reports. The time in therapeutic range correlated with the total quality of life scores in self reports (r = 0.22; P = 0.04), mother reports (r = 0.23; P = 0.02) and father reports (r = 0.28; P = 0.02). The time in therapeutic range predominantly correlated with school functioning in self reports (r = 0.38; P = 0.002) and mother reports (r = 0.40; P Conclusions Time in therapeutic range correlated with quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. Regular assessment of quality of life in patient education programmes contributes towards understanding the concerns and needs of patients.

Summary Background Because of sports and exercise restrictions, children with inherited cardiac disease are at risk of physical deconditioning. Guidelines on sports participation in cardiovascular disease have become less restrictive over time, but their real-life application and behavioural impact have seldom been evaluated in children. Aims We aimed to evaluate adherence to the 2020 European Society of Cardiology guidelines on sports and exercise in children with inherited cardiac arrhythmia and inherited cardiomyopathy; we also sought to evaluate their aerobic fitness, and the behavioural impact of inherited cardiac diseases on physical activity in children. Methods Children aged 6–18 years with inherited cardiomyopathy or inherited cardiac arrhythmia were eligible for this cross-sectional study. Clinical, demographic and qualitative data were analysed. Results A total of 32 children were included in the study (mean age 12.7 ± 3.5 years). Most children (81.3%) complied with the 2020 European Society of Cardiology guidelines; they were physically active and had good overall aerobic fitness, with a mean peak oxygen uptake (VO2) value of 36.5 ± 8.0 mL/kg/min (84.0 ± 17.2% of theoretical value). As a result of personal or parental behaviour, some children at risk of sudden cardiac death did not comply with the recommended upper limit of physical activity intensity, whereas others at low risk did not comply with the lower limit. Conclusion Most children with inherited cardiac arrhythmia or inherited cardiomyopathy complied with current 2020 European Society of Cardiology guidelines on sports cardiology and exercise in cardiovascular disease.

BackgroundAdvances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial.MethodsThe QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease’s clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%.DiscussionAfter focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population.Trial registrationClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 (https://clinicaltrials.gov/ct2/show/NCT04712136).

Background COVID-19 triggered an unprecedented crisis affecting our society at every level. Clinical research in paediatric and congenital cardiology is currently in full development and may have been disrupted. Aims To determine the impact of COVID-19 in paediatric and congenital cardiology clinical research and to analyse decision-making and adaptation processes, from a panel of on-going academic and industry-sponsored research at the time of the pandemic. Methods This observational study was carried out in April 2020, from a CHD clinical research network involving five tertiary care paediatric and congenital cardiology centres. Investigators and clinical research assistants from each participating research centre filled in an online survey questionnaire, and each principal investigator underwent a one-hour web-based videoconference interview. Results From a total of 34 study questionnaires and 288 enrolled patients at the time of the pandemic, 18 studies were declared as totally suspended. Upon investigator's decision, after discussion on ethical issues and with facilitating support from health authorities, 16 studies were resumed. The rate of study suspension in interventional research (53%) was similar to non-interventional research (56%). Logistical problems were predominantly reported in both continued and suspended trials. Research protocols were adapted, largely thanks to telemedicine, which in some cases even improved the course of the study. Conclusions The impact of the COVID-19 pandemic on clinical research in paediatric and congenital cardiology has been limited by a rapid adaptation of all research structures and an extensive use of telemedicine at all stages of the studies.

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).

BackgroundIn the context of tremendous progress in congenital cardiology, more attention has been given to patient-related outcomes, especially in assessing health-related quality of life (HRQoL) of patients with congenital heart diseases (CHD). However, most studies have mainly focused on teenagers or adults and currently, few HRQoL controlled data is available in young children. This study aimed to evaluate HRQoL of children with CHD aged 5 to 7 y.o., in comparison with contemporary peers recruited in school, as well as the factors associated with HRQoL in this population.MethodsThis multicentre controlled prospective cross-sectional study included 124 children with a CHD (mean age = 6.0 ± 0.8 y, 45% female) during their outpatient visit and 125 controls (mean age = 6.2 ± 0.8 y, 54% female) recruited at school. A generic paediatric HRQoL instrument was used (PedsQL 4.0).ResultsSelf-reported HRQoL in children with CHD was similar to controls, overall (73.5 ± 1.2 vs. 72.8 ± 1.2, P = 0.7, respectively), and for each dimension. Parents-reported HRQoL was significantly lower in the CHD group than in controls. HRQoL was predicted by the disease severity and by repeated invasive cardiac procedures (surgery or catheterization).ConclusionHRQoL in young children with CHD aged 5 to 7 years old was good and similar to controls. This study contributed to the growing body of knowledge on HRQoL in congenital cardiology and emphasized the need for child and family support in the most complex CHD.Trial registrationThis study was approved by the institutional review board of Montpellier University Hospital (2019_IRB-MTP_02-19) on 22 February 2019 and was registered on ClinicalTrials.gov (NCT03931096) on 30 April 2019,https://clinicaltrials.gov/ct2/show/NCT03931096.

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).

International audience; Background: Asthma is the most common chronic disease in pediatrics. Along with the usual drug therapy using corticosteroids and bronchodilators, some interest has been shown for adjuvant therapies, such as sophrology. However, the level of evidence for non‐pharmaceutical therapies in asthma remains low, especially in children. This study aimed to assess whether in children with asthma, peak expiratory flow (PEF) improved more after a sophrology session alongside standard treatment than after standard treatment alone.Methods: We carried out a prospective randomized controlled clinical trial among 74 children aged 6‐17 years old, hospitalized for an asthma attack. Group 1: conventional treatment (oxygen, corticosteroids, bronchodilators, physiotherapy) added to one session of sophrology. Group 2: conventional treatment alone. The primary outcome was the PEF variation between the initial and final evaluations (PEF2‐PEF1).Results: Demographic and clinical characteristics were similar in both groups at baseline. Measures before and after the sophrology session showed that the PEF increased by mean 30 L/min in the sophrology group versus 20 L/min in the control group (P = 0.02). Oxygen saturation increased by 1% versus 0% (P = 0.02) and the dyspnea score with visual analogue scale improved by two points point (P = 0.01). No differences were observed between the two groups in terms of duration of hospitalization, use and doses of conventional medical treatment (oxygen, corticosteroids, and bronchodilators), and quality of life scores.Conclusions: Sophrology appears as a promising adjuvant therapy to current guideline‐based treatment for asthma in children.

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: The use of coronary computed tomography (CT) angiography in children with coronary artery anomalies is increasing. However, it remains technically demanding and the need to adapt acquisition parameters to a patient’s cardiac characteristics has not yet been addressed. The aim of the study was to prospectively assess the feasibility of personalized multiphasic coronary CT angiography for pediatric patients. Methods: Fifty pediatric patients (mean age 6.1±4.9 years) with coronary artery anomalies underwent a coronary CT angiography on a wide detector single-source CT equipment. Fifteen different acquisition patterns were used to trigger the acquisition at the best theoretical moment within the cardiac cycle. The appropriate pattern was automatically selected based on the patient’s heart rate and heart rate variability, derived from the patient’s ECG. Two independent radiologists qualitatively evaluated images. Results: All acquisitions fully answered the clinical question for a mean effective dose of 0.97±0.34 mSv. Image quality qualified as good or excellent in 94% of cases (47/50). No examination was considered as not assessable but 6% (3/50) were scored as adequate for diagnosis. For these 3 patients, motion artifacts were the main cause of average image quality. No significant visual differences were reported between the different coronary arteries (mean score of 3.6 on a 4-point scale). No correlation between image quality and cardiac parameters were reported ( r =−0.19 and r =0.00, respectively for heart rate and heart rate variability). Conclusions: Personalized multiphasic coronary CT angiography acquisitions could be performed with diagnostic quality for a dose equivalent of Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT03194763.

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.

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 ).

Background In paediatrics, managing oral anticoagulant therapy with vitamin K antagonists (VKA) remains challenging. This study aimed to assess the correlation between time in therapeutic range (TTR) and quality of life (QoL) in children participating in a non-selective INR self-monitoring VKA-educational program. Methods Children aged from 2 to 18 years under VKA therapy were eligible for this prospective multicentre study. Clinical and demographic data were collected. Health-related QoL was assessed using the PedsQL 4.0 questionnaire. Correlations between QoL scores and TTR were measured. Results A total of 121 children were included in the study (mean age 9.6 ± 4.9 years old). Cardiac conditions represented the predominant indication for VKA. The mean TTR was 0.78 ± 0.15, overall, and 0.76 ± 0.24 over the 3-month period before QoL assessment. The mean total QoL score was 76.2 ± 18 in self-reports, 71.4 ± 22 in mother-reports, and 73.5 ± 19 in father-reports. The TTR correlated with the total QoL scores, in self-reports (r = 0.22, P = 0.04), mother-reports (r = 0.23, P = 0.02), and father-reports (r = 0.28, P = 0.02). The TTR predominantly correlated with school functioning in self-reports (r = 0.38, P = 0.002) and mother-reports (r = 0.40, P Conclusion TTR correlated with QoL in children participating in a non-selective INR self-monitoring VKA-educational program. Regular assessment of QoL in patient education programs participates in understanding the concerns and needs of patients.

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 .

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.