Early hybrid cardiac rehabilitation in congenital heart disease: the QUALIREHAB trial.

BACKGROUND AND AIMS: Cardiopulmonary fitness in congenital heart disease (CHD) decreases faster than in the general population resulting in impaired health-related quality of life (HRQoL). As the standard of care seems insufficient to encourage and maintain fitness, an early hybrid cardiac rehabilitation programme could improve HRQoL in CHD. METHODS: The QUALIREHAB multicentre, randomized, controlled trial evaluated and implemented a 12-week centre- and home-based hybrid cardiac rehabilitation programme, including multidisciplinary care and physical activity sessions. Adolescent and young adult CHD patients with impaired cardiopulmonary fitness were randomly assigned to either the intervention (i.e. cardiac rehabilitation) or the standard of care. The primary outcome was the change in HRQoL from baseline to 12-month follow-up in an intention-to-treat analysis. The secondary outcomes were the change in cardiovascular parameters, cardiopulmonary fitness, and mental health. RESULTS: The expected number of 142 patients was enroled in the study (mean age 17.4 ± 3.4 years, 52% female). Patients assigned to the intervention had a significant positive change in HRQoL total score [mean difference 3.8; 95% confidence interval (CI) 0.2; 7.3; P = .038; effect size 0.34], body mass index [mean difference -0.7 kg/m2 (95% CI -1.3; -0.1); P = .022; effect size 0.41], level of physical activity [mean difference 2.5 (95% CI 0.1; 5); P = .044; effect size 0.39], and disease knowledge [mean difference 2.7 (95% CI 0.8; 4.6); P = .007; effect size 0.51]. The per-protocol analysis confirmed these results with a higher magnitude of differences. Acceptability, safety, and short-time effect of the intervention were good to excellent. CONCLUSIONS: This early hybrid cardiac rehabilitation programme improved HRQoL, body mass index, physical activity, and disease knowledge, in youth with CHD, opening up the possibility for the QUALIREHAB programme to be rolled out to the adult population of CHD and non-congenital cardiac disease.

Aerobic physical capacity and health-related quality of life in children with sickle cell disease.

BACKGROUND: Aerobic fitness is a predictor of cardiovascular health which correlates with health-related quality of life in the general population. The aim is to evaluate the aerobic capacity by cardiopulmonary exercise test (CPET) in children with sickle cell disease in comparison with healthy matched controls. METHODS: Controlled cross-sectional study. RESULTS: A total of 72 children (24 with sickle cell disease and 48 healthy controls), aged 6-17 years old were enrolled. Children with sickle cell disease had a poor aerobic capacity, with median VO2max Z-score values significantly lower than matched controls (-3.55[-4.68; -2.02] vs. 0.25[-0.22; 0.66], P < 0.01, respectively), and a high proportion of 92% children affected by an impaired aerobic capacity (VO2max Z-score < -1.64). The VO2max decrease was associated with the level of anemia, the existence of a homozygote HbS/S mutation, restrictive lung disease and health-related quality of life. CONCLUSION: Aerobic capacity is poor in children with sickle cell disease. VO2max decrease is associated with the level of anemia, the existence of a homozygote HbS/S mutation, lung function, and health-related quality of life. These results represent a signal in favor of early initiation of cardiac rehabilitation in patients with sickle cell disease. CLINICAL TRIALS: NCT05995743. IMPACT: Aerobic fitness is a predictor of cardiovascular health which correlates with health-related quality of life in the general population. Aerobic capacity (VO2max) is poor in children with sickle cell disease, despite the absence of any pattern of heart failure. VO2max decrease was associated with the level of anemia, the existence of a homozygote HbS/S mutation, restrictive lung disease, and health-related quality of life. These results are in favor of early initiation of cardiac rehabilitation in children with sickle cell disease.

ROX (Respiratory rate-OXygenation) index to predict early response to high-flow nasal cannula therapy in infants with viral bronchiolitis.

INTRODUCTION: High-flow nasal cannula (HFNC) is commonly used as first step respiratory support in infants with moderate-to-severe acute viral bronchiolitis (AVB). This device, however, fails to effectively manage respiratory distress in about a third of patients, and data are limited on determinants of patient response. The respiratory rate-oxygenation (ROX) index is a relevant tool to predict the risk for HFNC failure in adult patients with lower respiratory tract infections. The primary objective of this study was to assess the relationship between ROX indexes collected before and 1 h after HFNC initiation, and HFNC failure occurring in the following 48 h in infants with AVB. METHOD: This is an ancillary study to the multicenter randomized controlled trial TRAMONTANE 2, that included 286 infants of less than 6 months with moderate-to-severe AVB. Collection of physiological variables at baseline (H0), and 1 h after HFNC (H1), included heart rate (HR), respiratory rate (RR), fraction of inspired oxygen (FiO2), respiratory distress score (modified Wood's Clinical Asthma Score [mWCAS]), and pain and discomfort scale (EDIN). ROX and ROX-HR were calculated as SpO 2 FiO 2 RR $\frac{\left(\frac{{\mathrm{SpO}}_{2}}{{\mathrm{FiO}}_{2}}\right)}{\mathrm{RR}}$ and 100 × ROX HR $100\times \frac{\mathrm{ROX}}{\mathrm{HR}}$ , respectively. Predefined HFNC failure criteria included increase in respiratory distress score or RR, increase in discomfort, and severe apnea episodes. The accuracies of ROX, ROX-HR indexes and clinical variable to predict HFNC failure were assessed using receiver operating curve analysis. We analyzed predictive factors of HFNC failure using multivariate logistic regressions. RESULT: HFNC failure occurred in 111 of 286 (39%) infants, and for 56 (50% of the failure) of them within the first 6 h. The area under the curve of ROX indexes at H0 and H1 were, respectively, 0.56 (95% confidence interval [CI] 0.48-0.63, p = 0.14), 0.56 (95% CI 0.49-0.64, p = 0.09). ROX-HR performances were better but remained poorly discriminant. HFNC failure was associated with higher mWCAS score at H1 (p < 0.01) and lower decrease in EDIN scale during the first hour of HFNC delivery (p = 0.02). In the multivariate analyses, age and mWCAS score were were found to be independent factors associated with HFNC failure at H0. At H1, weight and mWCAS were associated factors. CONCLUSION: In this study, neither ROX index, nor physiological variables usually collected in infants with AVB had early discriminatory capacity to predict HFNC failure.

Reference Values of Cardiopulmonary Exercise Test Parameters in the Contemporary Paediatric Population.

BACKGROUND: The evaluation of health status by cardiopulmonary exercise test (CPET) has shown increasing interest in the paediatric population. Our group recently established reference Z-score values for paediatric cycle ergometer VO2max, applicable to normal and extreme weights, from a cohort of 1141 healthy children. There are currently no validated reference values for the other CPET parameters in the paediatric population. This study aimed to establish, from the same cohort, reference Z-score values for the main paediatric cycle ergometer CPET parameters, apart from VO2max. RESULTS: In this cross-sectional study, 909 healthy children aged 5-18 years old underwent a CPET. Linear, quadratic, and polynomial mathematical regression equations were applied to identify the best CPET parameters Z-scores, according to anthropometric parameters (sex, age, height, weight, and BMI). This study provided Z-scores for maximal CPET parameters (heart rate, respiratory exchange ratio, workload, and oxygen pulse), submaximal CPET parameters (ventilatory anaerobic threshold, VE/VCO2 slope, and oxygen uptake efficiency slope), and maximum ventilatory CPET parameters (tidal volume, respiratory rate, breathing reserve, and ventilatory equivalent for CO2 and O2). CONCLUSIONS: This study defined paediatric reference Z-score values for the main cycle ergometer CPET parameters, in addition to the existing reference values for VO2max, applicable to children of normal and extreme weights. Providing Z-scores for CPET parameters in the paediatric population should be useful in the follow-up of children with various chronic diseases. Thus, new paediatric research fields are opening up, such as prognostic studies and clinical trials using cardiopulmonary fitness outcomes. Trial registration NCT04876209-Registered 6 May 2021-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT04876209 .

Think out of the box: association of left congenital diaphragmatic hernia and abnormal origin of the right pulmonary artery : A train can hide another.

BACKGROUND: We report the occurrence of a severe pulmonary hypertension (PH) in a neonate affected by a left congenital diaphragmatic hernia (CDH). PH in this patient was associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery. This malformation, sometimes named hemitruncus arteriosus, has to the best of our knowledge never been reported in association with a CDH. CASE PRESENTATION: A male newborn was hospitalized from birth in the neonatal intensive care unit after prenatal diagnosis of a left CDH. Ultrasound examination at 34 weeks of gestational age evaluated the observed-to-expected lung-to-head ratio at 49%. Birth occurred at 38+ 5 weeks of gestational age. Soon after admission, severe hypoxemia, i.e., preductal pulse oximetry oxygen saturation (SpO2) < 80%, prompted therapeutic escalation including the use of high frequency oscillatory ventilation with fraction of inspired oxygen (FiO2) 100% and inhaled nitric oxide (iNO). Echocardiography assessment revealed signs of severe PH and normal right ventricle function. Despite administration of epoprostenolol, milrinone, norepinephrine, and fluid loadings with albumin and 0.9% saline, hypoxemia remained severe, preductal SpO2 inconsistently greater than or equal to 80-85% and post ductal SpO2 lower on average by 15 points. This clinical status remained unchanged during the first 7 days of life. The infant's clinical instability was incompatible with surgical intervention, while chest X-ray showed a relatively preserved lung volume, especially on the right side. This prompted an additional echocardiography, aimed at searching an explanation of this unusual evolution and found an abnormal origin of the right pulmonary artery, which was confirmed on computed tomography angiography subsequently. A change in the medical strategy was decided, with the suspension of pulmonary vasodilator treatments, the administration of diuretics, and the decrease in norepinephrine dose to decrease the systemic-to-pulmonary shunt. Progressive improvement in the infant respiratory and hemodynamic status enabled to perform CDH surgical repair 2 weeks after birth. CONCLUSIONS: This case recalls the interest of systematic analysis of all potential causes of PH in a neonate with CDH, a condition frequently associated with various congenital malformations.

Use of new paediatric VO2max reference equations to evaluate aerobic fitness in overweight or obese children with congenital heart disease.

AIMS: Overweight and obesity in children with congenital heart disease (CHD) represent an alarming cardiovascular risk. Promotion of physical activity and cardiac rehabilitation in this population requires assessing the level of aerobic fitness (VO2max) by a cardiopulmonary exercise test (CPET). Nevertheless, the interpretation of CPET in overweight/obese children with CHD remains challenging as VO2max is affected by both the cardiac condition and the body mass index (BMI). The new paediatric VO2max  Z-score reference equations, based on a logarithmic function of VO2max, height and BMI, were applied to overweight/obese children with a CHD and compared with overweight/obese children without any other chronic condition. METHODS AND RESULTS: In this cross-sectional controlled study, 344 children with a BMI > 85th percentile underwent a CPET (54% boys; mean age 11.5 ± 3.1 years; 100 CHD; 244 controls). Using the VO2max  Z-score equations, aerobic fitness was significantly lower in obese/overweight CHD children than that in matched obese/overweight control children (-0.43 ± 1.27 vs. -0.01 ± 1.09; P = 0.02, respectively), and the proportion of children with impaired aerobic fitness was significantly more important in obese/overweight CHD children than in matched controls (17% vs.6%, P = 0.02, respectively). The paediatric VO2max  Z-score reference equations also identified specific complex CHD at risk of aerobic fitness impairment (univentricular heart and right outflow tract anomalies). Using Cooper's weight- and height-based linear equations, similar matched-comparisons analyses found no significant group differences. CONCLUSIONS: As opposed to the existing linear models, the new paediatric VO2max  Z-score equations can discriminate the aerobic fitness of obese/overweight children with CHD from that of obese/overweight children without any chronic disease. REGISTRATION: ClinicalTrials.gov NCT04815577.

The new paediatric VO2max  Z-score reference equations found a lower aerobic fitness in obese or overweight children with CHD than that in matched obese or overweight control children and a high proportion of children with impaired aerobic fitness in obese or overweight children with CHD and identified specific complex CHD at risk of aerobic fitness impairment. As opposed to Cooper's weight-based or height-based linear equations, the new paediatric VO2max  Z-score equations can discriminate the aerobic fitness of obese or overweight children with CHD from that of obese or overweight children without any chronic disease.

Context of a neonatal death affects parental perception of end-of-life care, anxiety and depression in the first year of bereavement.

BACKGROUND: Neonatal death is often preceded by end-of-life medical decisions. This study aimed to determine whether the context of death - after a decision of withholding or withdrawing life-sustaining treatment (WWLST) or despite maximum care - was associated with subsequent risk of parental anxiety or depression. The secondary objective was to assess parents' perceptions of end-of-life care according to death context. METHODS: Prospective single center observational study of all neonatal deaths in a neonatal intensive care unit over a 5-year period. Data were collected during hospitalization and from face-to-face interviews with parents 3 months after the infant's death. Anxiety and depression were assessed using Hospital Anxiety and Depression Scale (HADS) questionnaires, completed by parents 5 and 15 months after death. RESULTS: Of 179 deaths, 115 (64%) occurred after the WWLST decision and 64 (36%) despite maximum care. Parental satisfaction with newborn care and received support by professionals and relatives was higher in the first condition. Sixty-one percent of parents (109/179) attended the 3-month interview, with the distribution between groups very close to that of hospitalization. The completion rates of the HADS questionnaires by the parents who attended the 3-month interview were 75% (82/109) at 5 months and 65% (71/109) at 15 months. HADS scores at 5 months were consistent with anxiety in at least one parent in 73% (60/82) of cases and with depression in 50% (41/82). At 15 months, these rates were, respectively, 63% (45/71) and 28% (20/71). Risk of depression at 5 months was lower after a WWLST decision (OR 0.35 [0.14, 0.88], p = 0.02). Explicit parental agreement with the WWLST decision had an equivocal impact on the risk of anxiety at 5 months, being higher when expressed during hospitalization, but not at the 3-month interview. CONCLUSIONS: Context of death has a significant impact on the emotional experience of parents after neonatal loss, which underlines the importance of systematic follow-up conversations with bereaved parents.

Isolated Ventricular Inversion: A Rare Complex Congenital Heart Disease With Neonatal Cyanosis.

A 1-day-old girl was referred for a cardiology consultation for a mean saturation at 80% without respiratory distress. Echocardiography showed an isolated ventricular inversion. This entity is extremely rare, with fewer than 20 cases reported. This case report describes the clinical evolution and the complex surgical management of this pathology. (Level of Difficulty: Advanced.).

Reference values of aerobic fitness in the contemporary paediatric population.

AIMS: There has been a growing interest in the use of markers of aerobic physical fitness (VO2max assessed by cardiopulmonary exercise test (CPET)) in the follow-up of paediatric chronic diseases. The dissemination of CPET in paediatrics requires valid paediatric VO2max reference values to define the upper and lower normal limits. This study aimed to establish VO2max reference Z-score values, from a large cohort of children representative of the contemporary paediatric population, including those with extreme weights. METHODS AND RESULTS: In this cross-sectional study, 909 children aged 5 to 18 years old from the general French population (development cohort) and 232 children from the general German and US populations (validation cohort) underwent a CPET, following the guidelines on high-quality CPET assessment. Linear, quadratic, and polynomial mathematical regression equations were applied to identify the best VO2max Z-score model. Predicted and observed VO2max values using the VO2max Z-score model, and the existing linear equations were compared, in both development and validation cohorts. For both sexes, the mathematical model using natural logarithms of VO2max, height, and BMI was the best fit for the data. This Z-score model could be applied to normal and extreme weights and was more reliable than the existing linear equations, in both internal and external validity analyses (https://play.google.com/store/apps/details?id=com.d2l.zscore). CONCLUSION: This study established reference Z-score values for paediatric cycloergometer VO2max using a logarithmic function of VO2max, height, and BMI, applicable to normal and extreme weights. Providing Z-scores to assess aerobic fitness in the paediatric population should be useful in the follow-up of children with chronic diseases. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT04876209.

Impaired aerobic capacity in adolescents and young adults after treatment for cancer or non-malignant haematological disease.

PURPOSE: Childhood cancer survivors are at increased risk for cardiovascular disease. Maximal oxygen uptake (VO2max) is a major determinant of cardiovascular morbidity. The aim of this study was to compare aerobic capacity, measured by cardiopulmonary exercise test (CPET), of adolescents and young adults in remission with that of healthy controls and to identify the predictors of aerobic capacity in this population. METHOD: This is a controlled cross-sectional study. RESULTS: A total of 477 subjects (77 in remission and 400 controls), aged from 6 to 25 years, were included, with a mean delay between end of treatment and CPET of 2.9 ± 2.3 years in the remission group. In this group, the mean VO2max was significantly lower than in controls (37.3 ± 7.6 vs. 43.3 ± 13.1 mL/kg/min, P < 0.01, respectively), without any clinical or echocardiographic evidence of heart failure. The VAT was significantly lower in the remission group (26.9 ± 6.0 mL/kg/min vs. 31.0 ± 9.9 mL/kg/min, P < 0.01, respectively). A lower VO2max was associated with female sex, older age, higher BMI, radiotherapy, and hematopoietic stem cell transplantation. CONCLUSION: Impaired aerobic capacity had a higher prevalence in adolescents and young adults in cancer remission. This impairment was primarily related to physical deconditioning and not to heart failure. TRIAL REGISTRY: NCT04815447. IMPACT: In childhood cancer survivors, aerobic capacity is five times more impaired than in healthy subjects. This impairment mostly reflects early onset of physical deconditioning. No evidence of heart failure was observed in this population.

Risk factors for early occurrence of malnutrition in infants with severe congenital heart disease.

To identify the risk factors of early occurrence of malnutrition in infants with severe congenital heart disease (CHD) during their first year of life. Retrospective longitudinal multicenter study carried out from January 2014 to December 2020 in two tertiary care CHD centers. Four CHD hemodynamic groups were identified. Malnutrition was defined by a Waterlow score under 80% and/or underweight under -2 standard deviations. A total of 216 infants with a severe CHD, e.g., requiring cardiac surgery, cardiac catheterization, or hospitalization for heart failure during their first year of life, were included in the study. Malnutrition was observed among 43% of the cohort, with the highest prevalence in infants with increased pulmonary blood flow (71%) compared to the other hemodynamic groups (p < 0.001). In multivariate analysis, low birthweight (OR 0.62, 95% CI 0.44-0.89, p = 0.009), CHD with increased pulmonary blood flow (OR 4.80, 95% CI 1.42-16.20, p = 0.08), heart failure (OR 9.26, 95% CI 4.04-21.25, p < 0.001), and the number of hospitalizations (OR 1.35, 95% CI 1.08 l-1.69, p = 0.009) during the first year of life were associated with malnutrition (AUC 0.85, 95% CI 0.79-0.90).  Conclusions: In infants with a severe CHD, early occurrence of malnutrition during the first year of life affected a high proportion of subjects. CHD with increased pulmonary blood flow, low birthweight, heart failure, and repeated hospitalizations were risk factors for malnutrition. Further studies are required to identify optimal nutritional support in this population. What is Known: • Malnutrition is a known morbidity and mortality factor in children with severe congenital heart disease. What is New: • Early occurrence of malnutrition during the first year of life in infant severe congenital heart disease (CHD) was high (43%). • CHD with increased pulmonary blood flow, low birthweight, heart failure, and repeated hospitalizations during the first year of life were risk factors for malnutrition.

Aerobic fitness change with time in children with congenital heart disease: A retrospective controlled cohort study.

BACKGROUND: To evaluate the change in aerobic fitness (VO2max), measured by cardio-pulmonary exercise test (CPET), in children with congenital heart disease (CHD), compared to matched healthy controls, and identify predictors of VO2max change with time in this specific population. METHOD: This longitudinal retrospective multicentre cohort study was carried out from 2010 to 2020. We included CHD paediatric patients from the cohort of a previous cross-sectional study, who had a second CPET at least 1 year after the first one, during their follow-up. RESULTS: We included 936 children, 296 in the CHD group and 640 controls. Mean time between baseline and final CPET was 4.4 ± 1.7 years. After matching on age and gender and adjustment for age and BMI, the mean VO2max group difference was 10.5% ± 1.0% of percent-predict VO2max at baseline and increased to 19.1% ± 1.3% at final assessment. In the CHD group, the proportion of children with impaired aerobic fitness was significantly higher at final than at baseline CPET assessment (51.4% vs 20.3%; P < 0.01). The mean annual VO2max decrease was significantly worse in the CHD group than in controls (-1.88% ± 0.19% of percent-predict VO2max/year vs. -0.44% ± 0.27% of percent-predict VO2max/year, P < 0.01, respectively). In multivariate analyse, male gender, a high initial VO2max, a high BMI, and the number of cardiac surgical procedures ≥2, were predictors of the VO2max decrease with time. CONCLUSION: The VO2max decrease with time is more pronounced in children with CHD compared to healthy matched controls. This study highlighted the importance of serial CPET assessment in children with CHD. Trial registration NCT04815577.

Birth during on-call period: Impact of care organization on mortality and morbidity of very premature neonates.

Objectives: The evidence that risks of morbidity and mortality are higher when very premature newborns are born during the on-call period is inconsistent. This study aimed to assess the impact of this situation among other determinants of outcomes, particularly newborn characteristics and care organization. Methods: Observational study including all infants born < 30 weeks' gestation in a French tertiary perinatal center between 2007 and 2020. On-call period corresponded to weekdays between 6:30 p.m. and 8:30 a.m., weekends, and public holidays. The primary endpoint was survival without severe morbidity, including grade 3-4 intraventricular hemorrhage (IVH), cystic periventricular leukomalacia, necrotizing enterocolitis, severe bronchopulmonary dysplasia (BPD), and severe retinopathy of prematurity. The relationship between admission and outcome was assessed by an adjusted odds ratio (aOR) on the propensity of being born during on-call period and expressed vs. weekday. Secondary analyses were carried out in extremely preterm newborns (<27 weeks' gestation), in cases of early death (within 7 days), and before (2007-2013, 51.5% of the cohort) vs. after (2014-2020, 48.5% of the cohort) the implementation of a pediatrician-nurse team dedicated to newborn care in the delivery room. Results: A total of 1,064 infants [27.9 (26.3; 28.9) weeks, 947 (760; 1,147) g] were included: 668 during the on-call period (63%) and 396 (37%) on weekdays. For infants born on weekdays, survival without severe morbidity was 54.5% and mortality 19.2%. During on-call, these rates were 57.3% [aOR 1.08 (0.84-1.40)] and 18.4% [aOR 0.93 (0.67-1.29)]. Comparable rates of survival without severe morbidity [aOR 1.42 (0.87-2.34)] or mortality [aOR 0.76 (0.47-1.22)] were observed in extremely preterm infants. The early death rate was 6.4% on weekdays vs. 8.2% during on-call [aOR 1.44 (0.84-2.48)]. Implementation of the dedicated team was associated with decreased rates of mortality [aOR 0.57 (0.38, 0.85)] and grade 3-4 IVH [aOR 0.48 (0.30, 0.75)], and an increased rate of severe BPD [aOR 2.16 (1.37, 3.41)], for infants born during on-call. Conclusion: In this cohort, most births of very premature neonates occurred during the on-call period. A team dedicated to newborn care in the delivery room may have a favorable effect on the outcome of infants born in this situation.

Disseminated Aspergillosis Complicated With a Myocardial Abscess in an Immunodeficient Child.

Aspergillosis infection is a major complication of acquired immunosuppression, and is associated with poor prognosis in its invasive form. Cardiac dissemination of invasive aspergillosis is a rare and usually fatal consequence of systemic mycosis. We describe a pediatric case of myocardial aspergillosis abscess in a 12-year-old girl diagnosed with multimodality cardiac imaging approach. The patient underwent prolonged antimycotic treatment and bone marrow transplant, which improved her immunity, and, for the first time in the literature, resulted in a favorable outcome.

Hemolytic uremic syndrome related to Shiga-like toxin-producing Escherichia coli with encephalitis hiding a human herpesvirus-6 infection: a case report.

BACKGROUND: Cardiac and neurological involvement in hemolytic uremic syndrome are life-threatening complications. The most frequent complications of cardiac involvement in hemolytic uremic syndrome are myocarditis and cardiac dysfunction due to fluid overload. Pericarditis remains very rare in hemolytic uremic syndrome. To our knowledge, only five cases of cardiac tamponade associated with hemolytic uremic syndrome have been described in literature. A 27-month-old Caucasian girl presented with symptoms of nonbloody diarrhea and tonic-clonic seizures. The diagnosis of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome with central nervous system involvement was made, and stool examination revealed infection with a Shiga-like toxin-producing Escherichia coli. She did not need renal replacement therapy but had severe neurological impairment. The patient's course was complicated by pericardial effusion. A pericardiocentesis was performed via an apical approach because the pericardial effusion was predominantly surrounding the left ventricle. Effusion analysis showed an exudate and positivity for human herpesvirus-6B on polymerase chain reaction with viremia. This finding was consistent with primary human herpesvirus-6 infection with encephalitis. CONCLUSION: We report this uncommon case of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome associated with a severe human herpesvirus-6 infection. Secondary isolated pericardial effusion and atypical neurological involvement are uncommon in Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome and should lead the physician to perform additional investigations.

Hemangiol in infantile haemangioma: A paediatric post-marketing surveillance drug study.

AIM: Infantile haemangioma (IH) is the most common benign tumour in children. Since 2014, propranolol has become the first-choice therapy and currently Hemangiol is the only approved drug for complicated haemangioma. This post-marketing study reports the use of Hemangiol for IH in paediatric practice. METHOD AND RESULTS: From January 2014 to November 2018, 94 children (median age 4 [0; 21] months; 75% female) treated with Hemangiol for proliferative IH were enrolled in the study. The systematic paediatric cardiology consultation never contraindicated beta-blockers. Two Hemangiol initiation protocols were used: a conventional ambulatory 3-week titration phase protocol (n = 76, 80.9%), and a rapid initiation protocol with a 48-hour dose escalation in conventional hospitalization for severe proliferative or ulcerated IH (n = 18, 19.1%). In both protocols, the haemodynamic tolerance was good. The mean maintenance dose of Hemangiol was 2.7 ± 0.8 mg/kg/day, with a median treatment duration of 7 [1.5; 19] months. Adverse events (AEs) have been found in 25 (26,6%) patients, including 8 (8.5%) patients with serious AEs (uncontrolled bronchial hyperreactivity, n = 5; serious hypoglycaemia, n = 3). Some patients had one or more AEs, a total of 24 nonserious AEs was reported in 19 patients (sleep disturbances, n = 9; respiratory disorders, n = 5; digestive disorders, n = 6). No cardiac adverse event was reported. CONCLUSION: This post-marketing surveillance drug study supports the good tolerance of Hemangiol in children with IH. A rapid initiation protocol is of interest when treatment is urgent. The pretherapeutic paediatric cardiology consultation should not be systematic but only indicated for specific patients. CLINICALTRIALS.GOV: NCT04105517.

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

BACKGROUND: 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://clinicaltrials.gov/ct2/show/NCT03931096 .

The V̇e/V̇co2 slope: a useful tool to evaluate the physiological status of children with congenital heart disease.

Cardiopulmonary exercise test (CPET) is becoming a key examination to assess physical capacity and disease severity in pediatric cardiology. The V̇e/V̇co2 slope has been increasingly used as a surrogate marker for morbidity and mortality in adult heart failure, pulmonary arterial hypertension, and for adult patients with congenital heart disease (CHD). Nevertheless, the use of the V̇e/V̇co2 slope in children remains limited in the absence of reference values and clearly identified clinical determinants. This study aimed to compare the V̇e/V̇co2 slope in a pediatric cohort with CHD to that of age- and gender-adjusted healthy controls. We also intended to identify the clinical and CPET variables associated with V̇e/V̇co2 slope in this population. This cross-sectional study was carried out between November 2010 and September 2015 in two tertiary care pediatric cardiology reference centers. A total of 700 children were enrolled (399 CHD and 301 healthy controls). The mean V̇e/V̇co2 slope was significantly higher in the CHD subjects than in healthy subjects (31.6 ± 4.8 vs. 29.3 ± 4.8; P < 0.001). The V̇e/V̇co2 slope was higher in children with significant pulmonary regurgitation, tricuspid regurgitation, right ventricular hypertension, and right ventricle outflow tract (RVOT) obstacle. In the CHD group, V̇e/V̇co2 slope increase was associated with body mass index, the presence of a RVOT obstacle, the number of cardiac catheter procedures, as well as low age, forced vital capacity, tidal volume, and [Formula: see text]. Increased V̇e/V̇co2 slope was predominantly in children with single ventricle and/or residual right heart abnormalities, suggesting that maldistribution of pulmonary blood flow during exercise is an important CHD-unique determinant of V̇e/V̇co2 slope.NEW & NOTEWORTHY Using V̇e/V̇co2 slope is useful for children with congenital heart disease. V̇e/V̇co2 slope is sensitive to pulmonary blood flow maldistribution during exercise, this concerns congenital heart disease with pulmonary regurgitation, tricuspid regurgitation, right ventricular hypertension, and right ventricle outflow tract obstacle. V̇e/V̇co2 slope is a good parameter to follow single ventricles and right heart residual lesions (tetralogy of Fallot; pulmonary atresia; truncus arteriosus…).