How should we determine normal echocardiographic right ventricle function reference values in pediatrics?

International audienceSeveral 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

Double gas transfer factors (DLCO-DLNO) at rest in patients with congenital heart diseases correlates with their ventilatory response during maximal exercise

International audienceAimExercise capacity is altered in congenital heart diseases (CHD) with potentially impaired pulmonary perfusion adaptation during exercise, such as in single ventricle or in significant pulmonary regurgitation. This study aimed to evaluate the value of double gas transfer factor analysis, at rest in conjunction with postural manoeuvres, to explore the various components of pulmonary gas transfer and its association with exercise capacity.MethodsA total of 40 subjects (24 CHD, 16 controls) underwent a combined measurement of lung diffusing capacity for carbon monoxide and nitric oxide (DLCO-DLNO) to determine pulmonary membrane diffusion (Dm) and Vcap, in sitting then supine position. CHD patients performed a maximal cardiopulmonary exercise test.ResultsCompared to normal controls, the CHD group's DLNO, DLCO, Vcap, and alveolar volume (AV) at rest, in the sitting position were depressed, whereas the DLCO/AV and DLNO/AV were similar. The magnitude of Dm and Vcap adaptation induced by postural change was similar in both groups, indicating a preserved pulmonary capillary recruitment capacity in CHD. In the CHD group, at rest, for each ml of postural-induced increase in Vcap we observed during exercise a VE/VCO2 slope decrease of 0.46 (95% CI[0.83; 0.098]), indicating a better ventilatory response to exercise.ConclusionCHD patients with impaired pulmonary circulation have a reduced Dm and Vcap mainly due to decreased pulmonary volume but maintain a normal capacity to adapt these parameters through a simple recruitment manoeuver. Vcap adaptation evaluated at rest predicts the level of ventilatory efficiency during exercise, which represents a main limiting factor in these CHD patients

Cardiopulmonary fitness in children with congenital heart diseases versus healthy children.

International audienceOBJECTIVE : We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) with that of age-adjusted and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2max) in this population.METHODS ANS RESULTS : 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. 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±0.3vs 42.6±0.4 mL/kg/min, P<0.0001) and for each group (P<0.05). 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<0.01), for boys (−0.72±0.14vs 0.11±0.19 mL/kg/min/year, P<0.01) and for girls (−1.00±0.13 vs −0.55±0.21 mL/kg/min/year, P=0.05). 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 anomalyCONCLUSIONS : 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

Health-related quality of life in children with congenital heart disease aged 5 to 7 years: a multicentre controlled cross-sectional study

International audienceBackground: In 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. Methods: This 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). Results: Self-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). Conclusion: HRQoL 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 registration This 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 :// clini caltr ials.gov/ct2/show/NCT03 93109 6

Speckle tracking echocardiography in healthy children: comparison between the QLAB by Philips and the EchoPAC by General Electric

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Speckle-Tracking Echocardiography in Children With Duchenne Muscular Dystrophy: A Prospective Multicenter Controlled Cross-Sectional Study.

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

Use of speckle tracking echocardiography to detect late anthracycline-induced cardiotoxicity in childhood cancer: A prospective controlled cross-sectional study

International audienceBackground: This study aimed to detect late sub-clinical patterns of cardiac dysfunction using speckle tracking echocardiography (STE) in children with cancer remission more than 12 months after the end of anthracycline treatment.Methods: This prospective controlled study enrolled 196 children, 98 of which had been treated with anthracyclines (mean age 10.8 ± 3.6 years; 51% female) and 98 were age- and gender-matched healthy subjects in a 1:1 case-control design. Conventional echocardiographic variables were collected for left ventricle (LV) and right ventricle (RV). STE analyses were performed in the LV longitudinal, radial, and circumferential displacements and in the RV free wall longitudinal displacement. The association between LV global longitudinal strain (GLS) and the main clinical and biological parameters was evaluated.Results: After a mean time interval of 5.1 ± 3.2 years since the end of chemotherapy (mean cumulative anthracycline dose of 192 ± 96 mg/m2), conventional echocardiographic measures were normal. GLS was significantly decreased in the anthracycline group (-19.1% vs. -21.5%, P < 0.0001), with a higher proportion of children with abnormal values (Z-score < -2 in 18.6% vs. 1.0%, P < 0.0001). No association was found between GLS and clinical or biological parameters. Circumferential strain was significantly worse in the anthracycline group (-16.8% vs. -19.4%, P < 0.0001), and radial strain significantly better (+51.4% vs. +35.9%, P < 0.0001). RV conventional echocardiography and STE parameters were normal and not different between anthracycline and control groups.Conclusions: The existence of a modified LV strain despite normal LV function in children treated with anthracyclines represents an important perspective for cardiomyopathy surveillance in childhood cancer survivors. Clinical Trial Registration -ClinicalTrials.gov Identifier: NCT02893787

Assessment of left ventricular dyssynchrony by speckle tracking echocardiography in children with duchenne muscular dystrophy

International audiencePrognosis 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 left ventricle (LV) dyssynchrony using 2D-STE analysis. 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. 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 ms vs. 18.3 ± 50.4 ms, P < 0.001, respectively) and in mid inferoseptal to mid anterolateral segments (58.6 ± 35.3 ms vs. 42.4 ± 36.4 ms, P < 0.05, respectively), (2) modified Yu index (33.3 ± 10.1 ms vs. 28.5 ± 8.1 ms, P < 0.05, 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. Detection of LV mechanical dyssynchrony using 2D-STE analysis is an easily and reproducible method in paediatric DMD. 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 paediatric drug trials in the DMD-related cardiomyopathy prevention.Clinical Trial Registration Clinicaltrials.gov NCT02418338. Post-hoc study, registered on April 16, 2015

Impact of a transition education program on health-related quality of life in pediatric patients with congenital heart disease: study design for a randomised controlled trial

International audienceBackground: 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 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. 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 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. Discussion: As 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 registration: This study was approved by the National Ethics Committee (South-Mediterranean IV 2016-A01681-50) and was registered on Clinicaltrials.gov (NCT03005626)