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In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6–18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N > 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and “simple” lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VO2peak, 37.3 ml/min/kg (25th–75th percentile 31.3–43.8)) varied between specific defects; patients with univentricular hearts had lower VO2peak compared with other CHD. All groups had lower VO2peak compared to healthy Dutch children. Males had higher VO2peak, Wpeak and O2pulsepeak than females. Sex- and disease-specific distribution graphs for VO2peak, Wpeak and O2pulsepeak showed increase in variation with increase in height. Conclusion: Disease-specific distribution graphs for exercise capacity in children with CHD from a large multicentre cohort demonstrated varying degrees of reduced VO2peak and Wpeak. The distribution graphs can be used in the structured follow-up of patients with CHD to predict outcome and identify patients at risk. What is Known:• Children with congenital heart disease (COnHD) are at risk to develop heart failure, arrhytmia’s and other complications. Exercise capacity may be an important predictor for outcome in children with ConHD. In children, the interpretation of exercise capacity poses an additional challenge related to physical changes during growth. What is New:• In this report of a multi-center cohort >1300 childrewn with ConHD, we related the changes in exercise capacity to length. We demonstrated that exercise capacity was reduced as compared with healthy children and we observed variation between disease groups. Patients with a univentricular circulation (Fontan) had worse exercise capacity. We constructed disease specific charts of development of exercise capacity throughout childhood, accessible via a web-site. These graphs may help practitioner to guide children with ConHD.

Objectives: The goal of this study was to describe characteristics, cascade screening results, and predictors of adverse outcome in pediatric-onset arrhythmogenic right ventricular cardiomyopathy (ARVC). Background: Although ARVC is increasingly recognized in children, pediatric ARVC cohorts remain underrepresented in the literature. Methods: This study included 12 probands with pediatric-onset ARVC (aged 1-V 3 (P < 0.01), premature ventricular complexes/runs (P ≤ 0.01), and decrease in biventricular ejection fraction (P ≤ 0.01) were associated with VT occurrence. Conclusions: Pediatric ARVC carries high arrhythmic risk, especially in probands. Disease progression is particularly observed on electrocardiogram or Holter monitoring. Arrhythmic events are associated with male sex, T-wave inversions, premature ventricular complexes/runs, and reduced biventricular ejection fraction.

To explore the association between cardiorespiratory fitness and other physical literacy domains in children with cystic fibrosis (CF) or congenital heart disease (CHD).In 28 children with CF (n = 10) or CHD (n = 18), aged 7 to 11 years, cardiorespiratory fitness and the following physical literacy domains were measured: (a) physical competence, (b) motivation and confidence, (c) knowledge and understanding, and (d) daily behavior (ie, self-perceived moderate-to-vigorous physical activity [MVPA]).Cardiorespiratory fitness was significantly associated with motivation and confidence and self-perceived MVPA. There were no other significant associations.Cardiorespiratory fitness is associated with self-perceived MVPA, motivation, and confidence in children with CF or CHD.

In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6-18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and "simple" lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VO

Objective This study focuses on the evolution of treatment techniques for aortic coarctation in children and assesses long-term morbidity. Methods This retrospective cohort study evaluates patients treated for native aortic coarctation, with at least 7 years of follow-up. To assess time-related changes, three time periods were distinguished according to year of primary intervention (era 1, 2 and 3). Operative and long-term follow-up data were collected by patient record reviews. Results The study population consisted of 206 patients (177 surgical and 29 catheter-based interventions), with a median follow-up of 151 months. Anterior approach with simultaneous repair of aortic arch and associated cardiac lesions was more common in the most recent era. Median age at intervention did not change over time. Reintervention was necessary in one third of the cohort with an event-free survival of 74% at 5‑year and 68% at 10-year follow-up. Reintervention rates were significantly higher after catheter-based interventions compared with surgical interventions (hazard ratio [HR] 1.8, 95% confidence interval [CI] 1.04–3.00, p = 0.04) and in patients treated before 3 months of age (HR 2.1, 95% CI 1.27–3.55, p = 0.003). Hypertension was present in one out of five patients. Conclusion Nowadays, complex patients with associated cardiac defects and arch hypoplasia are being treated surgically on bypass, whereas catheter-based intervention is introduced for non-complex patients. Reintervention is common and more frequent after catheter-based intervention and in surgery under 3 months of age. One fifth of the 206 patients remained hypertensive.

Background: Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material. Methods: Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019. Results: A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death Conclusions: In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.

Contains fulltext : 229178.pdf (Publisher’s version ) (Closed access) BACKGROUND: The aim of this study was to evaluate early and long-term outcomes (mortality and prosthetic valve replacement) after mitral valve replacement with the 15-mm St Jude Medical prosthesis (St Jude Medical, St Paul, MN). METHODS: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15-mm St Jude Medical Masters prosthesis at 4 congenital cardiac centers in The Netherlands. Operative results were evaluated and echocardiographic data studied at 0.5, 1, 2, 3, 5, and 10 years after surgery. RESULTS: Surgery was performed in 17 infants. Ten patients (59%) were treated in the intensive care unit before surgery; 8 (47%) were on ventilator support. Median age at surgery was 3.2 months (interquartile range [IQR], 1.2-5.6), and median weight was 5.2 kg (IQR 3.9-5.7). There was 1 early cardiac death and 1 late noncardiac death. Median follow-up time was 9.6 years (IQR, 2.4-13.2), including 8 patients with a follow-up more than 10 years. The first prosthetic valve explantation (n = 11) occurred at a median of 2.9 years (IQR, 2.0-5.4). Other reinterventions were permanent pacemaker implantation (n = 3), subaortic stenosis resection (n = 2), and paravalvular leak repair (n = 1). Prosthetic valve gradients increased from a mean of 5.0 mm Hg (at discharge) to a mean of 14.3 mm Hg (at 5-year follow-up). CONCLUSIONS: Mitral valve replacement with the 15-mm prosthesis can safely be performed in infants and even in neonates. Median freedom from prosthesis replacement for outgrowth is 3.5 years. Thromboembolic complications were rare.

PURPOSE To systematically review evidence regarding the severity and prevalence of motor development in children with a critical congenital heart defect (CCHD) without underlying genetic anomalies. SUMMARY OF KEY POINTS Twelve percent of all included studies reported abnormal mean motor developmental scores, and 38% reported below average motor scores. Children with single-ventricle physiology, especially those with hypoplastic left heart syndrome, had the highest severity and prevalence of motor delay, particularly at 0 to 12 months. Most included studies did not differentiate between gross and fine motor development, yet gross motor development was more affected. RECOMMENDATIONS FOR CLINICAL PRACTICE We recommend clinicians differentiate between the type of heart defect, fine and gross motor development, and the presence of genetic anomalies. Furthermore, increased knowledge about severity and prevalence will enable clinicians to tailor their interventions to prevent motor development delays in CCHD.

Background:Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms.Methods:In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland–Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement.Results:The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al.Conclusion:The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.

Background Qualitative features of aortic and mitral valvar pathology have traditionally been used to classify congenital cardiac anomalies for which the left heart structures are unable to sustain adequate systemic cardiac output. We aimed to determine if novel groups of patients with greater clinical relevance could be defined within this population of patients with critical left heart obstruction (CLHO) using a data-driven approach based on both qualitative and quantitative echocardiographic measures. Methods An independent standardized review of recordings from pre-intervention transthoracic echocardiograms for 651 neonates with CLHO was performed. An unsupervised cluster analysis, incorporating 136 echocardiographic measures, was used to group patients with similar characteristics. Key measures differentiating the groups were then identified. Results Based on all measures, cluster analysis linked the 651 neonates into groups of 215 (Group 1), 338 (Group 2), and 98 (Group 3) patients. Aortic valve atresia and left ventricular (LV) end diastolic volume were identified as significant variables differentiating the groups. The median LV end diastolic area was 1.35, 0.69, and 2.47 cm2 in Groups 1, 2, and 3, respectively (p Conclusions Using a data-driven approach, we conceptualized three distinct patient groups, primarily based quantitatively on baseline LV size and qualitatively by the presence of aortic valve atresia. Management strategy and overall mortality differed significantly by group. These groups roughly correspond anatomically and are analogous to multi-level LV hypoplasia, hypoplastic left heart syndrome, and critical aortic stenosis, respectively. Our analysis suggests that quantitative and qualitative assessment of left heart structures, particularly LV size and type of aortic valve pathology, may yield conceptually more internally consistent groups than a simplistic scheme limited to valvar pathology alone.

BackgroundIn response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease. To assess its effectiveness, a stepped-wedge cluster randomised trial was conducted.MethodsAdult patients and caregivers of paediatric patients with congenital aortic and/or pulmonary valve disease and/or tetralogy of Fallot who visited the outpatient clinic at any of the four participating centres in the Netherlands between 1 March 2016–1 July 2017 were prospectively included. The intervention (information portal) was introduced in the outpatient clinic according to a stepped-wedge randomised design. One month after outpatient clinic visit, each participant completed a questionnaire on disease-specific knowledge, anxiety, depression, mental quality of life, involvement and opinion/attitude concerning patient information and involvement.Results343 participants were included (221 control, 122 intervention). Cardiac diagnosis (p=0.873), educational level (p=0.153) and sex (p=0.603) were comparable between the two groups. All outcomes were comparable between groups in the intention-to-treat analyses. However, only 51.6% of subjects in the intervention group (n=63) reported actually visiting the portal. Among these subjects (as-treated), disease-specific knowledge (p=0.041) and mental health (p=0.039) were significantly better than in control subjects, while other baseline and outcome variables were comparable.ConclusionEven after being invited by their cardiologists, only half of the participants actually visited the information portal. Only in those participants that actually visited the portal, knowledge of disease and mental health were significantly better. This underlines the importance of effective implementation of online evidence-based patient information portals in clinical practice.

Background. The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses.Methods. A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated.Results. Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]).Conclusions. Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk. (C) 2020 by The Society of Thoracic Surgeons. Published by Elsevier Inc.

Background: Anthracycline-induced cardiotoxicity is a major cause of morbidity and mortality in childhood cancer survivors (CCSs). Echocardiographic myocardial strain imaging is recommended in adult patients with cancer, but its role in pediatric CCSs has not been well established. Aims of this study were to determine the prevalence of abnormalities in left ventricular strain in pediatric CCSs, to compare strain with other echocardiographic measurements and blood biomarkers, and to explore risk factors for reduced strain. Methods: CCSs ≥3 years from their last anthracycline treatment were enrolled in this multicenter study and underwent a standardized functional echocardiogram and biomarker collection. Regression analysis was used to identify factors associated with longitudinal strain (LS). Results: Five hundred forty-six pediatric CCSs were compared with 134 healthy controls. Abnormal left ventricular ejection fraction (Z score, Conclusions: Global systolic function and LS are only mildly reduced in pediatric CCSs, and most LS values are within normal range. This makes single LS measurements of limited added value in identifying CCSs at risk for cardiac dysfunction. The utility of strain imaging in the long-term follow-up of CCS remains to be demonstrated.

Introduction: Congenital atrioventricular block (CAVB) is a rare disorder with a significant morbidity and mortality. Consensus regarding the prescription and efficacy of prenatal corticosteroids is lacking. This nationwide study was initiated to evaluate the effects of prenatal treatment with corticosteroids on the outcome of CAVB in The Netherlands.Methods: All fetuses identified with isolated congenital AVB-II degrees or AVB-III degrees in any of the eight academic fetal heart centers of The Netherlands between 2003 and 2013 were included and reviewed.Results: Fifty-six fetuses were included. Fourteen (25%) fetuses were treated with dexamethasone. We found no differences between the steroid-treated and untreated cases regarding in utero progression of the AVB (63% vs 67% respectively), survival to birth (86% vs 84%), pacemaker implantations (74% vs 58%) or long-term dilated cardiomyopathy (13% vs 17%). Steroid treated fetuses demonstrated more in utero growth restriction (38% vs 11%).Conclusion: No benefit from prenatal corticosteroid treatment was demonstrated for fetuses with isolated CAVB in this study. However, we found negative side effects. Our data provide no evidence to support the routine administration of corticosteroids for the treatment of fetal CAVB. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

Contains fulltext : 170596.pdf (Publisher’s version ) (Open Access) Surgical closure of the ventricular septal defect is the most commonly performed procedure in pediatric cardiac surgery. There are conflicting data on weight at operation as risk factor for a complicated course. We performed a retrospective evaluation of mortality and morbidity in all patients undergoing surgical ventricular septal defect closure at our institution between 2004 and 2012 to identify risk factor for a complicated course. Multivariate logistic regression modeling was performed to identify risk factors for a complicated course. 243 patients who underwent surgical ventricular septal defect closure were included. Median age at operation was 168.0 days (range 17-6898), the median weight 6.0 kg (range 2.1-102.0). No deaths occurred. Two patients (0.8%) required a pacemaker for permanent heart block. Five patients (2.1%) underwent reoperation for a hemodynamically important residual ventricular septal defect. No other major adverse events occurred. No risk factors for major adverse events could be established. Multivariate analysis identified a genetic syndrome, long bypass time and low weight at operation as independent risk factors for a prolonged intensive care stay (>1 day) and prolonged ventilation time (>6 h). Contemporary results of surgical VSD closure are excellent with no mortality and low morbidity in this series. Although it is associated with increased ventilation time and a longer hospital stay, low bodyweight at operation is not associated with an increased risk of complications or major adverse events in our series.

OBJECTIVESIn hypoplastic left heart complex patients, biventricular repair is preferred over staged-single ventricle palliation; however, there are too few studies to support either strategy. Therefore, we retrospectively characterized our patient cohort with hypoplastic left heart complex after biventricular repair to measure left-sided heart structures and assess our treatment strategy.METHODSPatients with hypoplastic left heart complex who had biventricular repair between 2004 and 2018 were retrospectively reviewed. Operative results were evaluated and echocardiographic mitral valve (MV) and aortic valve (AoV) dimensions, left ventricular length and left ventricular internal diastolic diameter (LVIDd) were measured preoperatively and during follow-up after 0.5, 1, 3, 5 and 10 years.RESULTSIn 32 patients, the median age at surgery was 10 (interquartile range 5.0) days. The median follow-up was 6.19 (interquartile range 6.04) years. During the 10-year follow-up, the mean Z-scores increased from −2.82 to −1.49 and from −2.29 to 0.62 for MV and AoV, respectively. Analysis of variance results with post hoc paired t-tests showed that growth of left-sided heart structures was accelerated in the first year after repair, but was not equal, with the MV lagging behind the AoV (P = 0.033), resulting in significantly smaller MV Z-scores compared with AoV Z-scores at 10-year follow-up (P CONCLUSIONSThe growth rate of heart structures was most prominent during the first year after biventricular repair with lower growth rate of the MV compared with the AoV.

A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients. This study sought to assess exercise capacity and blood pressure response in asymptomatic patients long-term after CoA repair in relation to left ventricular and vascular function. Twenty-two CoA patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (n = 10) between 3 months and 16 years of age with a follow-up of > 10 years underwent cardiopulmonary exercise testing at a mean follow-up of 23.9 years. Exercise capacity (peak oxygen uptake; VO 2peak) and blood pressure response were compared to age- and gender-matched reference values. Left ventricular function and volumetric analysis was performed using cardiovascular magnetic resonance imaging. CoA patients showed preserved exercise capacity compared to the healthy reference group, with a VO 2peak of 41.7 ± 12.0 ml/kg/min versus 44.9 ± 6.7 ml/kg/min. VO 2peak/kg showed a significant association with age (p < 0.001) and male gender (p ≤ 0.001). Exercise-induced hypertension occurred in 82% of CoA patients, and was strongly related to left ventricular mass (p = 0.04). Of the 41% of patients who were normotensive at rest, 78% showed exercise-induced hypertension. No significant correlation was found between peak exercise blood pressure and age, BMI, age at time of repair, LVEF, or LV dimensions. Exercise capacity is well preserved in patients long-term after successful repair of coarctation of the aorta. Nevertheless, a high number of patients develop exercise hypertension, which is strongly related to systemic hypertension. Regular follow-up, including cardiopulmonary exercise testing, and aggressive treatment of hypertension after CoA repair is strongly advised.

ObjectiveLong-term prognosis of patients with coarctation of the aorta (CoA) is impaired due to the high prevalence of hypertension and consequent cardiovascular complications. Although stent implantation results in acute anatomical and haemodynamic benefit, limited evidence exists regarding the late clinical outcome. In this meta-analysis, we aimed to evaluate the medium-term effect of stent placement for CoA on systemic blood pressure (BP).MethodsPubMed, EMBASE and Cochrane databases were searched for non-randomised cohort studies addressing systemic BP ≥12 months following CoA stenting. Meta-analysis was performed on the change in BP from baseline to last follow-up using a random-effects model. Subgroup analyses and meta-regression were conducted to identify sources of heterogeneity between studies.ResultsTwenty-six studies with a total of 1157 patients and a median follow-up of 26 months were included for final analysis. Meta-analysis showed a 20.3 mm Hg (95% CI 16.4 to 24.1 mm Hg; pConclusionsStent implantation for CoA is associated with a significant decline in systolic and diastolic BP during medium-term follow-up. The degree of BP reduction appears to be dependent on baseline systolic BP, baseline peak systolic gradient, and whether stenting is performed for native or recurrent CoA.

Arterial vasculopathy and residual aortic obstruction can lead to left ventricular (LV) dysfunction in patients with coarctation of the aorta (CoA) related to adverse ventriculo-arterial coupling. This study aimed to investigate potential differences in LV myocardial deformation indices between repaired CoA patients and healthy controls. Twenty-two CoA patients (age 30 ± 10.6 years) after surgical repair (n = 12) or balloon angioplasty (BA) (n = 10) without residual stenosis, between 3 months and 16 years of age with > 10 years follow-up were compared to 22 healthy age- and gender-matched controls (age 30 ± 3.8 years). Cardiac magnetic resonance feature tracking (CMR-FT) was used for LV longitudinal-, circumferential-, and rotational deformation indices. Global systolic LV function was preserved in CoA patients (LV ejection fraction 58 ± 4.8 vs. 60 ± 6.8%, p = 0.56) when compared to controls, with normal LV dimensions and mass (p > 0.05). Twelve CoA patients (55%) were hypertensive, of whom 4 were on anti-hypertensive medication. LV global longitudinal strain was preserved in the four-chamber (− 18 ± 4.4 vs. − 16 ± 4.7%, p = 0.06) and two-chamber (− 22 ± 5.1 vs. − 20 ± 6.0%, p = 0.22) orientations in CoA patients. Global circumferential strain was preserved at basal (− 29 ± 4.1 vs. − 28 ± 4.8%, p = 0.43), mid-ventricular (− 27 ± 4.2 vs. − 25 ± 3.0%, p = 0.09), and apical levels (− 35 ± 7.8 vs. − 32 ± 34.9%, p = 0.32). No differences were found in global torsion (2.4 ± 1.3° vs. 2.0 ± 1.4°/cm, p = 0.28), twist (14 ± 5.8° vs. 12 ± 6.3°, p = 0.34), and recoil rate (− 17 ± 9.7° vs. − 17 ± 7.1°/cm s, p = 0.97). Analysis of intra-observer variability demonstrated good reproducibility for all CMR deformation indices. Global and rotational myocardial deformation indices are preserved in CoA patients long-term after repair without residual stenosis, despite a high incidence of hypertension.

Background Hypertension is common in patients with coarctation of the aorta (CoA), even after successful repair. Increased aortic stiffness has been implicated in the pathology of CoA-associated hypertension. This study aimed to investigate aortic vascular function and its relationship with hypertension in well-repaired CoA-patients at long-term follow-up. Furthermore, we assessed the additive effect of hypertension to adverse arterioventricular coupling associated with increased aortic stiffness. Methods Twenty-two CoA-patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (BA) (n = 10) between 3 months and 16 years of age with a follow-up of >10 years and 22 healthy controls underwent cardiac magnetic resonance imaging (CMR), at mean follow-up of 29.3 years, to study aortic pulse wave velocity (PWV), aortic distensibility, global left ventricular (LV) function, LV dimensions, and LV myocardial deformation. Results CoA-patients had significantly increased aortic arch PWV (5.6 ± 1.9 m/s vs. 4.5 ± 1.0 m/s, P = .02) and decreased distensibility (4.5 ± 1.8 × 10–3 mmHg−1 vs. 5.8 ± 1.8 × 10–3 mmHg−1, P = .04) compared to controls. Significant differences in aortic arch PWV were found between hypertensive patients, normotensive patients and controls (6.1 ± 1.8 m/s vs. 4.9 ± 1.9 m/s and 4.5 ± 1.0 m/s, respectively, P = .03). Aortic arch PWV and distensibility were correlated with systolic blood pressure (R = 0.37 and R = −0.37, respectively, P = .03 for both). Global LV function, LV mass, LV dimensions and myocardial deformation were similar in CoA-patients when compared to controls. Conclusions Central aortic stiffness is significantly increased in well-repaired CoA-patients long-term after repair, and is associated with hypertension. Global LV function, myocardial deformation indices and LV dimensions are however preserved.

Item does not contain fulltext OBJECTIVES: Coarctation of the aorta (CoA) can be treated either surgically or with balloon angioplasty (BA). Long-term follow-up for either treatment has been limited. Our objective was to compare long-term results of BA and surgery for treatment of native CoA in childhood. METHODS: Retrospective cohort study of patients with native CoA treated with BA or surgery between 3 months and 16 years of age. Forty-eight patients filled out questionnaires and approved review of their medical records. Twenty-four patients underwent additional testing, including 24-h ambulatory blood pressure measurement, cardiopulmonary exercise testing and cardiac magnetic resonance imaging. Results were analysed cross-sectionally and longitudinally. RESULTS: Nineteen and 29 patients received BA and surgery, respectively. Prevalence of hypertension and aneurysms was similar in both groups. Fifty percent of patients were hypertensive. Two-thirds of patients demonstrating hypertension were not receiving antihypertensive medication. Aneurysm formation occurred in 1 BA (5%) and 1 surgery (3%) patient. The BA group had a significantly higher risk of recoarctation (47% vs 24%) and reintervention (hazard ratio 2.95, 95% confidence interval 1.04-8.32). Exercise capacity and global left ventricular function were preserved in both groups and not significantly different after correction for age. Quality of life was good to excellent in the majority of the patients. CONCLUSIONS: After CoA repair in childhood, most patients perform well in daily life. However, on the long term, more than half of the patients develop hypertension and many develop re-CoA, especially in those who underwent BA. Therefore, we do not recommend BA for the treatment of native CoA in children.

Background Although consensus guidelines for pretreatment evaluation and monitoring of propranolol therapy in patients with infantile hemangiomas (IH) have been formulated, little is known about the cardiovascular side effects. Objectives We sought to analyze cardiovascular evaluations in patients with IH at baseline and during treatment with an oral beta-blocker. Methods Data from 109 patients with IH were retrospectively analyzed. Patient and family history, pretreatment electrocardiogram (ECG), heart rate, and blood pressure were evaluated before initiation of beta-blocker therapy. Blood pressure and standardized questionnaires addressing side effects were evaluated during treatment. Results Questionnaire analyses (n = 83) identified 3 cases with a family history of cardiovascular disease in first-degree relatives. ECG findings were normal in each case and no serious complication of therapy occurred. ECG abnormalities were found in 6.5% of patients but there were no contraindications to beta-blocker therapy and no major complications. Hypotension in 9 patients did not require therapy adjustment. In all, 88 parents (81%) reported side effects during beta-blocker treatment. Limitations The relatively small patient cohort is a limitation. Conclusion Pretreatment ECG is of limited value for patients with an unremarkable cardiovascular history and a normal heart rate and blood pressure. Hypotension may occur during treatment.

Background Although consensus guidelines for pretreatment evaluation and monitoring of propranolol therapy in patients with infantile hemangiomas (IH) have been formulated, little is known about the cardiovascular side effects. Objectives We sought to analyze cardiovascular evaluations in patients with IH at baseline and during treatment with an oral beta-blocker. Methods Data from 109 patients with IH were retrospectively analyzed. Patient and family history, pretreatment electrocardiogram (ECG), heart rate, and blood pressure were evaluated before initiation of beta-blocker therapy. Blood pressure and standardized questionnaires addressing side effects were evaluated during treatment. Results Questionnaire analyses (n = 83) identified 3 cases with a family history of cardiovascular disease in first-degree relatives. ECG findings were normal in each case and no serious complication of therapy occurred. ECG abnormalities were found in 6.5% of patients but there were no contraindications to beta-blocker therapy and no major complications. Hypotension in 9 patients did not require therapy adjustment. In all, 88 parents (81%) reported side effects during beta-blocker treatment. Limitations The relatively small patient cohort is a limitation. Conclusion Pretreatment ECG is of limited value for patients with an unremarkable cardiovascular history and a normal heart rate and blood pressure. Hypotension may occur during treatment.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P

Although stent implantation for aortic coarctation (CoA) is associated with acute anatomical and hemodynamic benefit, limited evidence exists regarding the late impact on blood pressure and cardiovascular complications. In this meta-analysis we aimed to evaluate the influence of stent placement for

Background Hypoplastic left heart complex (HLHC) is characterized by a mitral valve or an aortic valve annular Z score Z scores of the mitral and aortic valve annuli are major determinants of HLHC. Therefore, the algorithm for Z -score calculation is essential for diagnosis. However, no single universal method of calculation is in use. In the scientific literature addressing HLHC, various Z -score calculation methods have been applied. The aim of this study was to evaluate Z scores derived from two-dimensional echocardiographic dimensions in patients with HLHC. Methods To compare the different published methods using two-dimensional echocardiographic measures for Z -score calculation, a cohort of 18 newborns diagnosed with HLHC was retrospectively evaluated. In addition, the methods to determine body surface area in newborns were evaluated. Results Three Z -score calculation methods were included and compared. Using the method of Daubeney et al . to calculate Z scores in our cohort illustrated a lack of correlation beyond a Z score et al . and Pettersen et al . Z scores calculated using Zilberman et al .'s and Pettersen et al .'s methods were fairly consistent. The equations used by Pettersen et al . are based on the largest population of neonates. Conclusion Although the different methods for calculating Z scores for mitral and aortic valve dimensions correspond fairly well in the normal range, Z scores Z -score calculation algorithms used by Pettersen et al . appear to be the most appropriate for use in an evaluation of HLHC. Because these different methods can yield different values, reporting the method as well as the Z score is essential for an accurate diagnosis. Similarly, the method used to determine body surface area should be reported.

To cite this article: Zomer-Kooijker K, Slieker MG, Kentie PA, van der Ent CK, Meijer Y. A prediction rule for food challenge outcome in children. Pediatric Allergy Immunology 2012: 23: 353359. Abstract Background: In children with food-related symptoms, a food challenge is considered as the gold standard to diagnose allergy. If food allergy could be predicted by patient history and/or diagnostic tests, the number of time-consuming and sometimes risky food challenges could be decreased. We aimed to determine questionnaire and test-based characteristics, to predict the food challenge outcome (FCO) in children referred to a tertiary centre for the evaluation of food-related symptoms. Methods: Pre-challenge standardized questionnaires, skin prick tests (SPT), and specific IgE levels (sIgE) were obtained in patients that underwent a food challenge in our hospital in 2009. Characteristics of patients with positive and negative FCO were compared, and uni- and multivariate associations between predictors and FCO were calculated. Based on the multivariate model, a risk score was developed to predict the FCO. Results: One hundred and twenty-nine challenges were analyzed, 41.9% had a positive outcome. Median age of both groups was 4.9 yrs (range 2.88.3). Patients with a positive FCO reacted faster with symptoms after allergen ingestion and had higher sIgE levels compared to children with negative FCO. A clinical risk score was developed based on the index food, time between allergen ingestion and complaints and sIgE levels (range 010). The prognostic capacity of this model (AUC) was excellent (0.90). The very high- and low-risk groups (24% of patients) are both predicted excellent without misclassification. Conclusion: Positive FCO can be predicted by the index food, time between allergen ingestion and development of symptoms, and the sIgE level.

Pulmonary function and nutritional status are important determinants of exercise capacity in patients with cystic fibrosis (CF). Studies investigating the effects of determinants, such as genotype or infection and inflammation, are scarce and have never been analysed in a multivariate longitudinal model. A prospective longitudinal cohort study was performed to evaluate whether genotype, chronic inflammation and infection were associated with changes in exercise capacity. Furthermore, we investigated whether exercise capacity can predict clinical outcome. 504 exercise tests of 149 adolescents with CF were evaluated. Maximal oxygen uptake corrected for body mass % predicted declined 20% during adolescence, and was associated with immunoglobulin (Ig)G levels and chronic Pseudomonas aeruginosa infection. A lower exercise capacity was associated with a higher mortality, steeper decline in pulmonary function and greater increase in IgG levels. Since a decline in exercise capacity during adolescence was negatively associated with IgG levels and chronic P. aeruginosa infection, these data emphasise the importance of prevention and treatment of chronic inflammation and infections in patients with CF. Furthermore, a lower exercise capacity was associated with a higher mortality rate, steeper decline in pulmonary function and higher increase in IgG levels with increasing age in adolescents with CF. This stresses the value of regular exercise testing for assessing prognosis in adolescents with CF.

Background Siblings with cystic fibrosis (CF) share many genetic and environmental factors but may present different phenotypes. Younger sibs are mostly earlier diagnosed with CF than their older sibs, but might be at risk for an earlier colonization with Pseudomonas aeruginosa (PA) than their older counterparts due to cross-infection within families. Aims To analyze the effects of birth order and age at diagnosis on lung function, PA colonization, nutritional status, and survival during the first two decades of life in siblings with CF. Methods A retrospective cohort study of 52 sibling pairs was performed in two Dutch CF centers. Data were analyzed both cross-sectionally and longitudinally using Kaplan–Meier curves and modified log-rank tests. Results Median age at diagnosis was significantly higher in the older sib compared with the younger sib (3.0 and 0.2 years, respectively, P

Traumatic dissection of the carotid artery is an infrequent but serious complication of blunt craniocervical injury. There is controversy regarding the need for diagnostic screening and management. This report presents a child with delayed neurologic symptoms and multiple cerebral infarcts secondary to bilateral extracranial traumatic carotid artery dissection. The pathophysiology, clinical presentation, and treatment options of blunt carotid artery trauma are discussed.

To assess outcome of assisted ventilation in cystic fibrosis (CF) patients with acute respiratory failure (ARF), to identify risk factors associated with poor outcome and to compare long-term outcome of CF children who were mechanically ventilated for ARF with unventilated CF controls. Retrospective cohort study. Two large CF centres in the Netherlands. CF patients who required assisted ventilation for ARF and unventilated CF controls. None. Thirty-one CF patients required assisted ventilation for ARF between January 1990 and March 2005. All five children (under 2 years of age) and seven adults (27%) survived. In the total population, age was a statistically significant risk factor for poor outcome (p = 0.02). In adult CF patients who required invasive mechanical ventilation, acute on chronic respiratory failure was associated with poor outcome. In children who required mechanical ventilation for ARF, lung function and CF related complications 5 years later were not significantly different compared with controls matched for age, gender and genotype. CF patients younger than 2 years old, who are ventilated because of ARF, have a good prognosis and their long-term outcome seems identical to unventilated CF controls. ARF in adult CF patients still is associated with high mortality, especially among patients with acute on chronic respiratory failure.

Background Birth prevalence and survival in patients with cystic fibrosis (CF) in the Netherlands were last investigated > 30 years ago. However, since then the birth prevalence may have decreased because of genetic counseling and an increased number of newborns of non-European descent. Although survival of CF patients has increased worldwide, a significantly lower median age at death was recently reported in the Netherlands compared with data from the United States. Objectives To analyze birth prevalence and survival in CF patients in the Netherlands, and to compare this survival data with US CF data. Design Survey of all CF patients living in the Netherlands, and analysis of Dutch CF mortality statistics using data from the Dutch central statistics office, Statistics Netherlands (Voorburg, the Netherlands), and a comparison with Cystic Fibrosis Foundation (Bethesda, MD) patient registry data. Setting All CF centers in the Netherlands and the United States. Participants All CF patients treated in the Netherlands on January 1, 2001, and all persons who died of CF between 1974 and 2000, and an equivalent US population. Measurements Birth prevalence and birth cohort-specific survival. Results The overall birth prevalence of CF for 1974 to 1994 was 1 in 4,750 live births, which is a considerable decrease compared with 1961 to 1965 (1 in 3,600 live births). Estimated survival to 30 years increased from 6% in the 1950-to-1954 cohort, to 36% in the 1970-to-1973 cohort. Exact survival could be calculated from 1974 onwards. Survival to 15 years increased from 72% from the 1974-to-1979 cohort, to 91% in the 1985-to-1989 cohort. Survival in the United States in the 1980-to-1984 cohort was better compared to the Netherlands, but this difference has disappeared over subsequent cohorts. Conclusions The actual birth prevalence of CF in the Netherlands is clearly lower than it was 30 years ago. Survival in CF has dramatically improved. The difference in survival between the Netherlands and the United States, as observed in the cohorts born > 20 years ago, has disappeared.

The variation in cystic fibrosis (CF) lung disease and development of CF related complications correlates poorly with the genotype of the CF transmembrane regulator (CFTR) and with environmental factors. Increasing evidence suggests that phenotypic variation in CF can be attributed to genetic variation in genes other than the CFTR gene, so-called modifier genes.In recent years, multiple candidate modifier genes have been investigated in CF, especially genes that are involved in the control of infection, immunity and inflammation. Some of these genes have been rather conclusively identified as modifiers of the CF phenotype, whereas associations found in other genes have not been confirmed or are conflicting.Identification of genetic variation in modifier genes, obtained by genotype–phenotype studies in well-defined patient populations, may be used as an aid to prognosis and may provide the possibility of new therapeutic interventions.

Item does not contain fulltext BACKGROUND: Aortic arch abnormalities represent 5% to 8% of all congenital heart disease. Measurements of the aortic arch dimensions on two-dimensional echocardiographic images remain of critical importance in the diagnosis of aortic arch pathology. To define aortic hypoplasia or coarctation, measured dimensions must be compared with normal values. Normal values have been described for children of all ages in earlier studies. However, normative data for premature infants are not yet available. Therefore, the aim of this study was to develop normative data in a cohort of premature infants, which could be used in the diagnosis of aortic arch abnormalities. METHODS: A single-center study was conducted in a large population of premature infants with gestational ages of

Background Intraosseous access is used in emergency medicine as an alternative when intravenous access is difficult to obtain. Intraosseous samples can be used for laboratory testing to guide treatment. Many laboratories are reluctant to analyse intraosseous samples, as they frequently block conventional laboratory equipment. We aimed to evaluate the feasibility and accuracy of analysis of intraosseous samples using an i-STAT ® point-of-care analyser. Methods Intravenous and intraosseous samples of twenty children presenting for scheduled diagnostic bone marrow aspiration were analysed using an i-STAT ® point-of-care analyser. Sample types were compared using Bland Altman plots and by calculating intraclass correlation coefficients and coefficients of variance. Results The handheld i-STAT ® point-of-care analyser proved suitable for analysing intraosseous samples without technical difficulties. Differences between venous and intraosseous samples were clinically acceptable for pH, base excess, sodium, ionised calcium and glucose in these haemodynamically stable patients. The intraclass correlation coefficient was excellent (>0.8) for comparison of intraosseous and intravenous base excess, and moderate (around 0.6) for bicarbonate, sodium and glucose. The coefficient of variance of intraosseous samples was smaller than that of venous samples for most variables. Conclusion Analysis of intraosseous samples with a bedside, single-use cartridge-based analyser is feasible and avoids the problem of bone marrow contents damaging conventional laboratory equipment. In an emergency situation point-of-care analysis of intraosseous aspirates may be a useful guide to treatment.

Objective: This study describes the association between the complexity of congenital cardiac and extracardiac malformations, and the parental decision of pregnancy continuation or termination. Methods: Congenital heart defects (CHD) was diagnosed by ultrasound in 251 fetuses before the 24th week (23 + 6 weeks) of gestation during the four year period from 2007 to 2010. All fetuses from the Utrecht region were referred to our center due to a strict referral pattern. The complexity and severity of the cardiac and possible extracardiac malformations were retrospectively categorized by a pediatric cardiologist and a perinatologist who were blinded to the pregnancy outcome. The groups with and without termination of pregnancy were compared using a univariate analysis and multivariate logistic regression. Results: In 119 (47%) of the 251 fetuses, parents opted for termination of pregnancy. In 103 of these cases (87%) there was a high complex or lethal cardiac, or a major or lethal extracardiac malformation. Of the 132 continued pregnancies, 42 fetuses (32%) had a high complex or lethal cardiac, or a major or lethal extracardiac malformation. There were significantly more terminations of pregnancy in case of a high or lethal complex cardiac or extracardiac anomaly (71 vs. 15%, p Conclusion: Parents opted for termination of pregnancy significantly more often in cases with high complex cardiac and extracardiac malformations. It was rare for parents to opt for pregnancy termination in the absence of a severe cardiac or extracardiac malformation.

In children with food-related symptoms, a food challenge is considered as the gold standard to diagnose allergy. If food allergy could be predicted by patient history and/or diagnostic tests, the number of time-consuming and sometimes risky food challenges could be decreased. We aimed to determine questionnaire and test-based characteristics, to predict the food challenge outcome (FCO) in children referred to a tertiary centre for the evaluation of food-related symptoms.Pre-challenge standardized questionnaires, skin prick tests (SPT), and specific IgE levels (sIgE) were obtained in patients that underwent a food challenge in our hospital in 2009. Characteristics of patients with positive and negative FCO were compared, and uni- and multivariate associations between predictors and FCO were calculated. Based on the multivariate model, a risk score was developed to predict the FCO.One hundred and twenty-nine challenges were analyzed, 41.9% had a positive outcome. Median age of both groups was 4.9 yrs (range 2.8-8.3). Patients with a positive FCO reacted faster with symptoms after allergen ingestion and had higher sIgE levels compared to children with negative FCO. A clinical risk score was developed based on the index food, 'time between allergen ingestion and complaints' and sIgE levels (range 0-10). The prognostic capacity of this model (AUC) was excellent (0.90). The very high- and low-risk groups (24% of patients) are both predicted excellent without misclassification.Positive FCO can be predicted by the index food, time between allergen ingestion and development of symptoms, and the sIgE level.

Background: Serological methods to monitor Pseudomonas aeruginosa colonisation in patients with cystic fibrosis (CF) are advocated but the diagnostic value of a commercially available P aeruginosa antibody test to detect early and chronic P aeruginosa colonisation in a non-research setting has not been assessed. Methods: Colonisation with P aeruginosa was estimated by regular culture of sputum or oropharyngeal swabs during three consecutive years in 220 patients with CF aged 0–65 years. Commercially available ELISA tests with three P aeruginosa antigens (elastase, exotoxin A, alkaline protease) were performed at the end of the study period. In a subgroup of 57 patients (aged 4–14 years) serological tests were performed annually. Results: Using culture as the reference standard, the ELISA tests using the advised cut off values had a sensitivity of 79% and a specificity of 89% for chronic colonisation. Receiver-operator characteristic curves were created to optimise cut off values. Applying these new cut off values resulted in a sensitivity of 96% and a specificity of 79%. All three individual serological tests discriminated well between the absence and presence of chronic P aeruginosa colonisation. The sensitivity of the individual antibody test was 87% for elastase, 79% for exotoxin A, and 76% for alkaline protease. First colonisation was preceded by positive serological results in only five of 13 patients (38%). Conclusion: In patients with CF, serological tests using specific antigens are sensitive for diagnosing chronic P aeruginosa colonisation. However, the failure of serological tests to detect early colonisation in young patients emphasises the need for continued reliance on cultures.